RSMPD Abstract E Book

RSMPD 2018
Abstracts E-Book
PAEDIATRIC DERMATOLOGY
Content
Case Reports/ Case series
Case Reports/ Case series

(Oral Presentations)
Research Studies
Research Studies
(Oral Presentations)
RSMPD 2018
Paediatric Dermatology
CASE REPORTS/ CASE SERIES
Abstract ID Category Title: Author Links
Other Papulosquamous Disorders
Infantile Generalised Pustular Psoriasis On Acitretin Therapy: Ai Leen Wee

PAP-10047 View Abstract
Case Series In A Single Centre
Intestinal Strongyloides Presenting As Protein Losing

Ai Leen Wee
PAP-10048 Enteropathy In An Immunocompetent Psoriatic Child: A Case View Abstract
Report
PAP-10077 Hydroa Vacciniforme: Presentation And Clinical Course Jade Chee View Abstract
Erythrodermic Psoriasis In Child Treated With Cyclosporine Sarah Diba

PAP-10098 View Abstract
And Methylprednisolone Zulkarnain
Ni Luh Putu Ratih

RSMPD07 A Case Series Of Guttate Childhood Psoriasis View Abstract
Vibriyanti Karna
Harry Potter And The Psorcerer's Skin: A Case Of An 11-Year-

RSMPD22 Kyla Janika Nerva View Abstract
Old Male With Psoriasis
Urticaria And Other Dermal Hypersensitivity Disorders
Systemic Mastocytosis Associated With Hodgkin’s Lymphoma

UDH-10016 Sahana Srinivas View Abstract
In A 4 Year Old Child
Aninda Fitri
UDH-10046 Herpes Associated Erythema Multiforme In A 7-Year-Old Boy View Abstract
Nugrahani
Contact Urticaria To Oatmeal In A Young Child With Atopic

UDH-10108 Eczema: A Cautionary Tale In The Use Of Oatmeal Containing Valencia Long View Abstract
Emollients
Chyntia Giska
UDH-10111 Misdiagnose Of Dermatitis Herpetiformis In Child, Why? View Abstract
Aryunisari
Erythema Multiforme Following Nonspecific Febrile Illness In A Irene Jessica

RSMPD04 View Abstract
Pediatric Patient Soputro
Chronic Idiopathic Urticaria In Paediatric Patients Below 12

Nopriyati Husan
RSMPD08 Years Of Age Treated With Omalizumab: View Abstract
Haluan
A Case Series
I Gusti Agung Ayu

RSMPD10 A Case Series Chronic Bullous Disease Of Childhood View Abstract
Dwi Karmila
A Case Of Chronic Bullous Disease Of Childhood Which Was
RSMPD20 Risty Hafinah View Abstract
Treated With Sulfasalazine
Connective Tissue Disorders
Paediatric Cutaneous Lupus Erythematosus: A 10-year Xinjie Jonathan

CTD-10000
Experience In A Tertiary Dermatological Centre Tang
View Abstract
A 10-Year Retrospective Study Of Morphoea In An Asian

CTD-10001
Paediatric Population
Clare Fern Lim View Abstract
Bullous Systemic Lupus Erythematosus With Systemic

CTD-10045 Nesa Wike Wilanti View Abstract
Corticosteroid
Dhany Prafita
CTD-10054 En Coup De Sabre View Abstract
Ekasari
CTD-10067 Zebra Stripes Like Eruptions In Dermatomyositis Sahana Srinivas View Abstract
Hyper Ige Syndrome In Neonatal Lupus Erythematous : Is It A

CTD-10110 Ari Karmila Sari View Abstract
Complication Or A Comorbid?
I Saw The Signs: A Case Of A 15 Year Old Female With Hazel Lizette
Dermatomyositis Panlilio
Drug Reactions
Stevens Johnson Syndrome Following Measles And Varicella Anggun

DRX-10053 View Abstract
Vaccination PutriYuniaswan
Jusuf Nelva
DRX-10091 Generalized Exfoliative Dermatitis In Children ; A Case Report View Abstract
Karmila
Drug Induced Stevens-Johnson Syndrome In Children : A Rusyati Luh Made

Case Series Mas
Skin Manifestations Of Other Systemic Disorders
Hua-Liang Joel
SSD-10034 An Instructive Case Of Granulomatous Chelitis View Abstract
Lim
Management Of Acrodermatitis Enteropathica With A Normal

SSD-10050 Diane Tantia Sari View Abstract
Serum Zinc Level
A Rare Case Of Circinate Balanitis In A Child With Reiter’s

SSD-10083 Elis Lee View Abstract
Syndrome
Anuradha
SSD-10084 Munchausen Syndrome By Proxy - A Case Report View Abstract
Kakkanatt Babu
Githa
SSD-10089 Cutaneous Langerhans Cell Histiocytosis: Challenge In View Abstract
Rahmayunita
Diagnosis
Acquired Cutaneous Lymphangiectasia Of The Vulva

SSD-10106 Valerie Ho View Abstract
Secondary To Tuberculosis Infection In A Teenage Girl
Rumpel-Leede Phenomenon - A Deceptively Alarming Rash In

SSD-10112 Valerie Ho View Abstract
Otherwise Healthy Infants
A Rare Paediatric Case Of Blastic Plasmacytoid Dendritic Cell

RSMPD06 Jazlyn Read View Abstract
Neoplasm Masquerading As A Chronic Pretibial Abscess
Acute Haemorrhagic Oedema Of Infancy In A 10-Month Old

RSMPD27 Male With Gastroenteritis- A Worrisome Presentation With A Joseph Boustany View Abstract
Benign Course: Case Report And Literature Review
Neonatal Skin Disorders
NSD-10058 Subcutaneous Fat Necrosis Of The Newborn, A Case Report MichaelaTabalon View Abstract
Genodermatoses
GDT-10019 Juvenile Hyaline Fibromatosis: Case Report Of A Rare Nirmala

View Abstract
Inherited Disease Ponnuthurai
Aplasia Cutis Congenita Due To Intrauterine Varicella

GDT-10038 Retno Dwi Utami View Abstract
Infection: A Case Report
Neu-Laxova Syndrome: A Spectrum Of Multiple Congenital

Kathleen Mitch
GDT-10040 Anomalies And Collodion Membrane In A Preterm Newborn View Abstract
Gomez
Filipino Male
Molecular Analysis Of Circumscribed Scalp Leukotrichia In An

GDT-10071 Kenneth Fong View Abstract
Infant Born To A Mother With Oculocutaneous Albinism Type 1
Aplacia Cutis Congenita

GDT-10074 Tantari Sugiman View Abstract
Pachyonychia Congenita With Oral Leukokeratosis In A 2

Michael Hartanto
GDT-10081 Years Old Boy View Abstract
Angriawan
A Case Report
GDT-10086 Ichthyosis Vulgaris Rodinda Hutabarat View Abstract

GDT-10105 Acrokeratosis Verruciformis Of Hopf - A Case Report Charmaine Lim View Abstract
A rare case of ectodermal dysplasia - hypotrichosis with

RSMPD09 Wai Leong Kok View Abstract
juvenile macular dystrophy
Focal facial dermal dysplasia: Two Case Reports with Shi Yun
distinctive features Chia
Nia Srie
RSMPD15 Blau Syndrome: Identical to Juvenile Idiopathic Arthritis or Not? View Abstract
Haryati
"Two Cases of Xeroderma Pigmentosum in One Family:

RSMPD19 Yuri Yogya View Abstract
Different Clinical Severity in Cutaneous Malignancies"
Margaret
A Case Report On A Rare Disease: Dyskeratosis Congenita In
RSMPD28 Stephanie View Abstract
A 28 Year Old Male
Jimenez
Pigmentary Disorders
Combination Of Oral Mini Pulse Dose Corticosteroid With

PMS-10002 Narrow Band Ultraviolet B And Topical Corticosteroid Rini Hastuti View Abstract
In Childhood Vitiligo
Cutaneous Mosaicism: An Interesting Case Of Becker's Nevus

PMS-10018 An Jian Leung View Abstract
Presenting In A Checkerboard Mosaic
PMS-10057 Piebaldism: A Case Report Djohan Kho View Abstract
A Rare Case Of Linear And Whorled Nevoid Hypermelanosis

PMS-10076 With Global Developmental Delay, Scoliosis And Retinal Deo Adiel Wong View Abstract
Degeneration With Dermoscopic Features
PMS-10099 12 Years Old Girl With Erythema Dischromicum Perstans Aryani Inda Astri View Abstract
Viral Infections
Treatment Of Perianal Warts In Children With 5% Imiquimod

VII-10035 Hoi Ling Wong View Abstract
Cream: Case Series
Molluscum Contagiosum In Children On Anogenital Area: A

VII-10059 Ariyati Yosi View Abstract
Serial Case
Bacterial Infections
Staphylococcal Scalded Skin Syndrome : Thigita Aga

BCI-10011 View Abstract
Three Cases Report Pandaleke
Two Case Reports Of Paediatric Leprosy In Institut Pediatrik,
BCI-10068 Sheau SzuHeah View Abstract
Hospital Kuala Lumpur
BCI-10069 Erythema Nodosum Leprosum In Children Mila Darmi View Abstract
Borderline Tuberculoid Leprosy With Type I Leprosy Reaction Ramona Dumasari

In Children Onset Lubis
Staphylococcal Scalded Skin Syndrome In Children And

RSMPD03 IGAA Praharsini View Abstract
Neonates : A Case Series Report
Metastatic Tuberculous Abscess That Difficult To Distinguish

RSMPD14 From Scrofuloderma In A Child With Pulmonary And Skeletal Dia Febrina View Abstract
Tuberculosis
Borderline Tuberculoid Type Of Morbus Hansen With Leprosy

RSMPD17 Angeline Dewi View Abstract
Disability Grade 2 In Children
Reversal Reaction In Pediatric Patients With Morbus Hansen:

RSMPD18 Angeline Dewi View Abstract
A Case Series Report
Fungal Infections
Angie Regina
FUI-10066 Case Report: Black Dot Form Of Tinea Capitis In Children View Abstract
Sutrisno
Parasitic Infections
Agassi Suseno
PAI-10085 Crusted Scabies Presenting As Erythroderma View Abstract
Sutarjo
Cutaneus Larva Migrans In Infant Succesfully Treated With

RSMPD05 Djohan Kho View Abstract
Topical Albendazole
Skin Disorders Of Immunodeficiency (Primary Or Acquired)
Hypersensitivity To Mosquito Bites As A Presentation Of

SDI-10043 Epstein-Barr Virus (EBV) Associated NK-cell Youjia Zhong View Abstract
Lymphoproliferative Disorder
Vascular Tumours and Malformations
Dayang Siti
A Single Centre Experience With Oral Sirollimus For The
VTM-10014 Yuhana Ag Mat View Abstract
Treatment Of Complex Vascular Anomalies.
Yusof
Facial Venolymphatic Malformation With Associated Jenny Yen

VTM-10062 View Abstract
Developmental Venous Anomalies (DVA): Case Report LingTan
Other benign and malignant tumours
Widyaningsih
BMT-10065 Case Report: Giant Congenital Melanocytic Nevus In An Infant View Abstract
Oentari
BMT-10082 Multiple Facial Clyndromas Ferra Olivia Mawu View Abstract
RSMPD 2018
RESEARCH STUDIES
Abstract ID Category Title: Author Links
Atopic Dermatitis and Other Eczematous Conditions
Pharmacists' Counselling Improve Knowledge Of Caregivers Jing Yi Vanessa

ADE-10064 View Abstract
Of Paediatric Eczema Patients Cheong
POEM Is An Ideal Symptom-based Severity Score For

ADE-10102
Childhood Eczema Research
Leung Theresa NH View Abstract
Comparison Of Tewl Value Between Diaper Area And

ADE-10104 Nadia Wirantari View Abstract
Nondiaper Area Of Infant’s Skin
Skin Manifestations Of Other Systemic Disordersenodermatoses
A Characterisation Of Juvenile Acanthosis Nigricans In A

SSD-10072 Liau Mei Qi May View Abstract
Singaporean Tertiary Dermatological Centre
R.M Rendy Ariezal

RSMPD23 Langerhans Cell Histiocytosis: A Case Series View Abstract
Effendi
Neonatal skin disorders
Comparison of the Efficacy between Local Manufactured and

RSMPD11 Imported Moisturizers on The Skin Barrier of Very Low Birth Triana Agustin View Abstract
Weight Premature Neonates
Genodermatoses
Aplasia Cutis Congenita Due To Intrauterine Varicella Infection:
GDT-10094 Srinivas Sahana View Abstract
A Case Report
Pigmentary Disorders
The Combination Therapy Of Excimer Light And Vitamin D3 Oral

RSMPD21 Reiva Farah Dwiyana View Abstract
For Childhood Vitiligo: A Prospective Study In Indonesia
Hair and Nail Disorders
Should We Biopsy Melanonychia Striata In Asian Children? A

HND-10044 Colin Tan Wei Xuan View Abstract
Retrospective Observational Study.
Use Of Diphenylcyclopropenone (DPCP) In The Treatment Of
Wai Mun Sean
HND-10088 Paediatric Alopecia Areata (AA): Experience From A Tertiary View Abstracts
Leong
Healthcare Institution In Singapore
Pattern Of Paediatric Dermatology Inpatient Consultations In

BCI-10061 Hoi Ling Wong View Abstract
A Tertiary Care Hospital In Malaysia
Fungal infections
Retrospective review of paediatric dermatology patients with

RSMPD26 Cheryl JL Lie View Abstract
dermatophytosis
RSMPD 2018
Oral Presenters
Abstract ID Category Title: (Case Reports/ Case series) Author Links
Genodermatoses
Collodion Baby With Vitamin D Deficiency And Oral Yustian Devika

GDT-10052 View Abstract
Candidiasis Rakhmawati
Compound Heterozygosity Of Itgb4 Mutations With A

Splicing Error In Junctional Epidermolysis Bullosa With
GDT-10080 Lynette Wee View Abstract
Pyloric Atresia And Severe Gastrointestional And
Tracheolaryngeal Manifestations.
Identification Of Compound Heterozygous Abca12

GDT-10090 Benny Loo View Abstract
Mutations In A Patient With Harlequin Ichthyosis
Other Papulosquamous Disorders
Congenital And Infantile Erythrodermic Psoriasis: Two

PAP-10097 Sonia Hanifati View Abstract
Case Reports
Vascular Tumours and Malformations
Blue Rubber Bleb Naevus Syndrome Presenting As An In

VTM-10036 Hoi Ling Wong View Abstract
Utero Facial Tumour- A Case Report
Connective Tissue Disorders
A Case Of Juvenile Dermatomyositis Complicating Diane Tantia

CTD-10049 View Abstract
Calcinosis And Hyperimmunoglobulinemia E Syndrome Sari
Management Of Recurrent Erythema Nodosum Leprosum Sheau Szu

In A Child Heah
Abstract ID Category Title: (Research Paper) Author Links
Acne and Acneiform Disorders
Isotretinoin In Young Children. Very Effective And Safe, But Marius

ACN-10005 View Abstract
Relapse Common. Rademaker
Atopic Dermatitis and Other Eczematous Conditions
Nutritional Assesment Of Infants With Severe Atopic Nirmala

ADE-10039 View Abstract
Dermatitis: A Prospective Observational Study Ponnuthurai
Jenny Yen Ling

ADE-10051 Contact Sensitization In Children With Atopic Dermatitis View Abstract
Tan
Longterm Effect Of Methotrexate For Childhood Atopic

ADE-10092 Diana Purvis View Abstract
Dermatitis
Genodermatoses
GDT-10103 Eb In Singapore - Brief Overview Priya Bishnoi View Abstract
Hair and Nail Disorders
Topical Immunotherapy With Diphencyprone In Extensive

Soumya
HND-10096 Alopecia Areata In Children: A 3 Year Experience From A View Abstract
Jagadeesan
Tertiary Centre In South India.
Abstract ID: PAP-10047
Track: Other Papulosquamous Disorders
Contact Author: Ai LeenWee
Country: Malaysia
Organisation: Malacca Hospital
Poster: View Here
Infantile Generalised Pustular Psoriasis On Acitretin Therapy: Case Series In A Single Centre
Background:
Generalised pustular psoriasis, also known as Von Zumbusch, is a rare form of psoriasis. Attacks of pustular psoriasis
are characterised by fever that lasts several days, followed by sudden onset of pustules eruptions. The pustules are
disseminated over the trunk and extremities, including the nail beds, palms and soles.
Case reports:
We report three cases of pustular psoriasis that were diagnosed druing the past 1 year in Malacca Hospital, Malaysia.
All three cases, 2 girls and a boy; presented during the infancy period. The pustules were sterile and the blood
inflammatory markers were normal. Their conditions did not respond to systemic antibiotics. The diagnosis was finally
clinched based on the clinical presentation and confirmed with dermatopathological examinations. Topical treatment did
not manage to control their conditions. Acitretin was commenced at doses ranging from 0.5 - 0.7 mg/ kg/ day and all of
them responded favourably. All improved relatively fast with complete resolution within 2 to 3 weeks after commencing
Acitretin. None of them experienced any unwanted adverse effects from Acitretin so far. Periodic blood monitoring which
included liver function tests and random lipid profiles remained within the normal ranges. They remained well to date..
Discussion:
Infantile generalised pustular psoriasis is uncommon and it may pose as a diagnostic and therapeutic challenge.
Acitretin is used widely as the first line of treatment in generalised pustular psoriasis. Other options of systemic treatment
included Methotrexate and Cyclosporine. Although the long term safety profile of Acitretin has not been well established,
particularly the risk of skeletal toxicity; it still remains a safe and efficacious treatment for this condition till date. Prompt
diagnosis and appropriate treatment is essential to ensure that the disease process does not affect the normal
development of these infants.
Poster PAP-10047
Infantile Generalised Pustular Psoriasis On Acitretin Therapy:

Case Series In A Single Centre
Dr. Wee Ai Leen
Malacca Hospital, Malacca, Malaysia
Introduction:
Generalised pustular psoriasis (GPP), is a rare type of psoriasis, first described in 1910 by
von Zumbusch. It is characterised by development of subcorneal sterile pustules super-
imposed on erythematous bases. It has always been said to be very rare however there
were three new cases of infantile generalised pustular psoriasis seen in my clinic past
one year.
Case series:
We report three cases of pustular psoriasis that were diagnosed past one year Figure 5: Histopathology from biopsy
in Malacca Hospital, Malaysia. All three cases, one boy and two girls; presented during
showing spongiform pustules of Kogoj.
infancy period. Their conditions did not improve and worsen with topical corticosteroids
and systemic antibiotics. There were many similarities in all the three cases. Case 1 Discussion & Conclusion:
(Figure 1-4) is a boy presented at 5 months old with generalized erythematous patches
that quickly became multiple pinhead-sized sterile pustules. The pustules coalesce to Infantile generalised pustular psoriasis (GPP) is rare and it may pose as a
become larger purulent collections. The pustules resolved but new crops recurred again. diagnostic and therapeutic challenge. Infantile psoriasis accounts for only 3.5%-16% of
Child has low grade fever and irritability but consolable. Case 2 and case 3, both girls, childhood psoriasis(1). Although literature shows that it is a rare condition but there
presented with similar clinical features at 7 months old and 6 months old respectively. All were three newly diagnosed infants past one year. All three had the typical clinical
of them had no family history of psoriasis or generalised pustular psoriasis. All three presentation and were diagnoses were confirmed with histopathology.
cases had leucocytosis, thrombocytosis, raised C-reactive proteins, normal renal and liver
functions. All three dermatopathology results were in keeping with pustular psoriasis. Various agents, including infections, irritating topical treatment (Koebner
phenomenon), drugs and stress have been suggested(1). Pustular psoriasis develops
Topical treatment did not manage to control their conditions. Acitretin was independently or in association with pre-existing psoriasis. In a Malaysian retrospective
commenced at doses ranging from 0.5-0.7 mg/kg/day. All of them responded favourably study, 78% of 95 patients with acute GPP had a preceding history of psoriasis(2).
with improvement seen relatively fast with complete resolution of skin rash within 2 to 3 However the study quoted here was among the adult onset GPP. All the cases illustrated
weeks after commencing Acitretin. None of them experienced any unwanted adverse here did not have preceding history of psoriasis. Newer studies suggest that genetic
effects from Acitretin so far. Periodic blood monitoring which included liver function factors are distinct from those associated with chronic plaque psoriasis contributing to
tests and random lipid profiles remained within the normal ranges. At present, their GPP. In particular, mutations in the IL35RN gene that encodes the interleukin-36 receptor
conditions remained well controlled till date and are still receiving treatment and follow antagonist (IL-36Ra), an anti-inflammatory cytokine in the IL-1 family that inhibits
up at Malacca Hospital, Malaysia. proinflammatory signal pathways by preventing the binding of IL-36 to its receptor, have
been detected in some patients with GPP(3). Growing understanding of the molecular
basis of GPP associated with IL36RN mutations may aid in therapeutic decisions for
patients with this type of GPP. Case reports documenting successful treatment of GPP in
patients with IL36RN mutations with anakinra, an IL-1 receptor antagonist, support the
importance of the pathway(3).
All three cases presented during infancy with similar presentations of

generalized erythematous scaly plaques with multiple pin-head sized sterile pustules.
They frequently coalesce forming larger purulent collections often referred to as ‘lakes of
pus’ (Figure 1). The pustules resolve within several days, leaving erythema and extensive
scaling. Erythroderma may occur. Recurrent episodes of pustulation during which new
crops of pustules emerge is a classic feature of acute GPP. These episodes are accompa-
nied by fever, chills and malaise and patients often appears systemically ill. Other
extracutaneous symptoms that may occur include arthralgias, lower extremity oedema,
jaundice and ocular abnormalities. However all three of the illustrated patients did not
experience any extracutaneous manifestations. Diagnosis is based mainly on clinical
Figure 1 Figure 2 features and histology of the skin biopsy. Typical features included psoriasiform changes
in the epidermis (parakeratosis and elongation of rete ridges), numerous neutrophils
migrating from the papillary capillaries into the epidermis, spongiform pustules of Kogoj
(Figure 5), perivascular lymphocyte-predominant infiltrate in the upper dermis. The
spongiform pustule of Kogoj is a characteristic histologic feature of psoriasis that is most
prominent in the pustular variant (Figure 5).
Oral tretinoin ie Acitretin is used widely as the first line of treatment in

generalised pustular psoriasis. Other options of systemic treatment included Methotrex-
ate and Cyclosporine. Although the long term safety profile of Acitretin has not been
established, particularly the risk of skeletal toxicity; it still remains a safe and efficacious
treatment for this condition till date. Prompt diagnosis and appropriate treatment is
essential to ensure that the disease process does not affect the normal development of
these infants.
References:
1. Infantile generalized pustular psoriasis: a case report and review of the literature. Su Y-S,
Chen G-S, Lan C-C E. Dermatologica Sinica 2011: 22-24.
Figure 3 Figure 4
2. Choon SE, Lai NM, Mohammad NA, et al. Clinical profile, morbidity and outcome of adult-
Figure 1: Erythematous, scaly, patches with well defined border. onset generalized pustular psoriasis: analysis of 102 cases seen in a tertia
r y hospital in
Figure 2: Lakes of pustules with erythematous base on the trunk. Widespread similar Johor, Malaysia. Int J Dermatol 2014; 53:676.
lesions seen all over including the palms and soles.
3. Robert EK. Pustular psoriasis: Pathogenesis, clinical manifestations and diagnosis. Up to
Figure 3: Two weeks post treatment: Dry erythematous patches without pustules.
date. Last updated Dec 2016.
Figure 4: Two months post treatment: Clear skin.
Contact Author: Ai LeenWee
Country: Malaysia
Organisation: Malacca Hospital
Poster: View Here
Intestinal Strongyloides Presenting As Protein Losing Enteropathy In An Immunocompetent Psoriatic Child: A

Case Report
Background:
Chronic plaque psoriasis is not uncommonly encountered among the children. Protein losing enteropathy is a common
presentation of inflammatory bowel disease (IBD). There have been increasing reports of association between psoriasis
with IBD since the 1990s. Psoriasis and IBD are related inflammatory diseases with well described epidemiologic and
genetic correlations.
Case presentation:
We report here a ten-year-old girl who has been having chronic plaque psoriasis for many years presented with 2 week
history of abdominal pain and chronic diarrhoea associated with hypoalbuminaemia, oedema and significant weight
loss. Paediatric gastroenterologist was consulted to exclude IBD in view of protein losing enteropathy.
Oesophagogastroduodenoscopy (OGDS) was subsequently performed. Although there were some mild inflammation
noted but biopsy did not reveal any significant findings. Thus she was treated symptomatically and her condition
improved temporarily with albumin transfusion and high protein milk formula. However, approximately two weeks after
discharge, she experienced again gradual worsening of similar symptoms. She was still having intermittent colicky
abdominal pain although diarrhoea was milder. She had severe albuminaemia with generalized oedema and further
worsening of weight loss. Another OGDS was performed and biopsy this time revealed large amount of strongyloides
larvae. She was treated with two courses of oral anti-helminthic medication and symptoms gradually improved and
resolved. Currently she remained well with satisfactory weight gain and growth velocity. Psoriasis is also well controlled.
Discussion:
Strongyloides stercoralis is an intestinal nematode with a complicated life cycle. Several risk factors have been
associated with strongyloides infestation including immunosuppressive therapy, human immunodeficiency virus
infection, diabetes and chronic renal failure. In this patient who is immunocompetent and not on immunosuppressive
therapy, it remains uncertain whether there is a definite correlation of the strongyloides infestation with psoriasis or just
a mere coincident.
Poster PAP-10048
Intestinal Strongyloides Presenting As Protein Losing Enteropathy

In An Immunocompetent Psoriatic Child: A Case Report
Dr. Ai Leen Wee
Malacca Hospital, Malacca, Malaysia
Introduction:
Chronic plaque psoriasis is one of the commonest type of psoriasis. Although more common in adults, it is not unusually seen in the paediatric age group. Protein losing
enteropathy, which is a common presentation of inflammatory bowel disease (IBD) is increasingly reported in patients with pso riasis. Psoriasis and IBD are related inflammatory
diseases with well described epidemiologic and genetic correlations(2). Intestinal strongyloidiasis and other parasitic infes tations can also present as protein losing enteropathy
although it is rarely seen among the immunocompetent individuals.
Case report:
We report here a ten-year-old girl with of chronic plaque psoriasis, on topical corticosteroids and regular phototherapy. She presented with gradual worsening of bilateral
eyes and legs swelling for 2 weeks. It was associated with intermittent colicky epigastric pain past 3 months, with gradual d eterioration. She also experienced abdominal distension,
abdominal bloating, flatulence and altered bowel habit (diarrhoea 3-4 times a day alternating with constipation). There was mucus in stool but no blood. She had significant weight
loss of 4 kg in a month. There were no other significant systemic symptoms. Physical examinations revealed generalised, multi ple erythematous, well demarcated borders, scaly
plaques with marked pedal oedema up to the shin and mild ascites. Vital signs were stable and no other remarkable systemic fi ndings. Blood investigations revealed severe
hypoalbuminaemia of 13g/L, mildly elevated liver enzymes (ALT 136U/L; AST 104U/L), hypokalaemia 2.2mmol/L, hypomagnesemia 0.4 4mmol/L and normal total white count but
had eosinophilia (10%). Inflammatory markers (erythrocyte sedimentation rate and C-reactive protein) were within normal limits and cultures (stool and blood) were negative. She
was referred to the paediatric gastroenterology team to exclude inflammatory bowel disease (IBD). Oesophagogastroduodenoscop y (OGDS) was normal while colonoscopy showed
some inflammation but histopathology showed mild non-specific inflammatory changes. She was started on regular high protein milk formula and albumin transfusion. Her
condition improved, stool became increasingly well-formed and she was discharged.
However she experienced again gradual worsening of similar symptoms about 2 weeks after discharged. She was still having intermittent colicky abdominal pain although
diarrhoea was milder. She had severe hypoalbuminaemia with generalised oedema. Another OGDS was performed by the gastroenterology team and biopsy this time revealed
large amount of strongyloides larvae. Strongyloides stercoralis was detected in stool helminth PCR. She was treated with two courses of oral anti -helminthic medication and
symptoms gradually improved and resolved. Currently she remained asymptomatic with satisfactory weight gain and growth velocity. Psoriasis remained well controlled * Figure
1 +,* Figure 2 +.
[ Figure 3 ] [ Figure 4 ]
Figure 1: Erythematous, scaly well defined plaques over periorbital, chin; well demarcated
hypopigmented patches over forehead.
Figure 2: Multiple erythematous, scaly, well-defined borders plaques over bilateral legs.
Figure 3: Life cycle Strongyloides stercoralis
Figure 4: Strongyloides stercoralis larvae in stool
[ Figure 1 ] [ Figure 2 ]
Discussion:
The case illustrated above is a known case of chronic plaque psoriasis. When she presented with clinical characteristics of protein losing enteropathy associated with
significant weight loss and mucus in the stool, it was essential to exclude the possibility of inflammatory bowel disease (IB D). Strongyloides has been shown to infrequently mimic
IBD or to disseminate when a patient with IBD and unrecognised strongyloides is treated with immunosuppression(2). Strongyloides stercoralis is an endemic intestinal nematode in
tropical and temperate areas around the world. It has a complicated life cycle and infects humans percutaneously and has a d irect, autoinfective and a nonparasitic free living
developmental cycle * Figure 3 +. Several risk factors have been associated with strongyloides infestation including immunosuppressive therapy, human immunodeficiency virus
infection, diabetes and chronic renal failure. Clinically, intestinal strongyloides can present with simple acute gastroenteritis but when it becomes chronic and undiagnosed, hence
untreated, the symptoms can mimic protein losing enteropathy(2, 3).
Diagnosis may not be so easy and straightforward. Gold standard for diagnosis is serial stool examination * Figure 4 +. Howev er traditional stool examinations are
insensitive and require up to seven stool examinations to reach a sensitivity of 100%(3). Although serologic tests are quite sensitive, cross reactions with other parasites decreases
the specificity of the tests(3). Duodenal biopsy is another useful tool, but it can be falsely negative in cases where nemato de has not invaded the mucosa. It may be misdiagnosed as
eosinophilic duodenitis, especially when there are few parasites especially in low prevalence countries and/or in unsuspected cases(1). As illustrated in this case, the stool
examinations were not sent repeatedly and the diagnosis was only clinched with the second duodenal biopsy.
Oral Ivermectin is recommended as the first line therapy for strongyloidiasis by the Centers for Disease Control and Preventi on (CDC). Ivermectin was not prescribed here
as it is not available for human use in Malaysia. However, our patient responded well to the Albendazole (CDC second line the rapy) given. In this patient who is immunocompetent
and not on immunosuppressive therapy, it remains uncertain whether there is a definite association of the intestinal strongyloidiasis with chronic psoriasis or just a mere coincident.
Nonetheless, this case clearly illustrated that high index of suspicion is required to properly investigate similar cases in order to administer treatment promptly.
Conclusion: References:
Although inflammatory bowel disease is a reported association in patients with psoriasis, 1. Intestinal strongyloidiasis in a psoriatic patient following immunosuppressive therapy: Seeing the
unforeseen. Poongodi L.S.K., Revathy C, Nimaladevi P, Nepoleon R. J. of the Scientific Society.
the probable diagnosis of parasitic infestation must not be ignored and should always be
2016: 43; 102-105.
considered, even more among children with psoriasis in endemic countries. High index of
2. IBD or Strongyloidiasis? Marta M.B-W, Andrea M-M, Ester S-N et al. Rev Esp Enferm Dig. 2016:
suspicion is required in order for the clinician to investigate thoroughly and administer 108; 516-520.
treatment promptly. 3. Parasites- Strongyloides. Centres for Disease Control and Prevention. Resources for Health
Professional; 2016: August 19.
Contact Author: Jade Chee
Country: Singapore
Organisation: Singapore General Hospital
Poster : Veiw here
Hydroa Vacciniforme: Presentation And Clinical Course
Hydroa vacciniforme is a photosensitivity disorder of unknown aetiology, characterized by the childhood onset of
necrotic vesiculopapules on exposed areas. Spontaneous improvement usually occurs at puberty or by the late teenage
years. No abnormalities are usually found on investigation.
We present a case of a 10-year-old boy with a 1-year history of papular lesions over his face, described as blisters
which subsequently healed with depressed scarring. Preliminary investigations were non-conclusive. A skin biopsy was
performed on a 3-day-old lesion on his left cheek, which revealed the characteristic histology of Hydroa vacciniforme.
In this report, we will discuss the in-depth histology found in hydroa vacciniforme, the differential diagnoses of
photosensitive dermatoses which will need to be ruled out, as well as the management options in this condition.
Poster PAP-10077
Contact Author: Zulkarnain Sarah Diba
Country: Indonesia
Organisation: Faculty of Medicine Universitas Indonesia/Dr. Cipto

Mangunkusumo National General Hospital
Poster: View Here
ERYTHRODERMIC PSORIASIS IN CHILD TREATED WITH CYCLOSPORINE AND METHYLPREDNISOLONE
Background. Paediatric psoriasis can have a profound long-term impact on the psychological health of affected children.
Treatment of psoriasis in paediatric remains a challenge. Methotrexate remains the first line treatment of psoriasis.
Unfortunately, in Indonesia it was no longer available since 2017. Some patients had abrupt withdrawal of methotrexate
that trigger erythrodermic psoriasis. Cyclosporine and systemic glucocorticoid is using for treated erythrodermic
psoriasis. Cyclosporine is an immunosuppressant drug approved for treatment of psoriasis in adults. However, the
efficacy of cyclosporine in pediatric psoriasis remains unclear.
Case Presentation. A 14-year-old Sumatran boy presented to our hospital with erythrodermic psoriasis after abrupt
withdrawal of methotrexate. He has psoriasis vulgaris since 7-month-old. The histopathology revealed epidermal
spongiosis, parakeratosis, and spongiform micropustules of Kogoj. We treated the patient with methylprednisolone 32
mg daily, and after 1 week we added cyclosporine 50 mg twice daily as a steroid-sparing agent. After 3 weeks of therapy,
there was clinical improvement. This patient is still in remission with minimal dose of methylprednisolone and
cyclosporine.
Discussion. The evidence on efficacy and safety of psoriasis therapy in childhood is still limited. The abrupt withdrawal
of methotrexate has an enormous impact for patients, such as erythroderma. Due to unavailability of methotrexate, we
decided to start cyclosporine and methylprednisolone. Cyclosporine is one of immunosuppressant drug that can be
used for treating erythrodermic psoriasis, either as a single therapy or as a sparing agent. The decision to treat the
paediatric patient with psoriasis with systemic agent should be carefully assessed, as the long-term potential side effect
of many systemic agents is still unknown. Close monitoring of blood pressure and renal function is required.
Keywords: erythrodermic psoriasis, paediatric, cyclosporine, methylprednisolone

Poster PAP-10098
ERYTHRODERMIC PSORIASIS IN CHILD TREATED WITH

CYCLOSPORINE AND METHYLPREDNISOLONE
Sarah Diba Zulkarnain1, Citra Tresna Mur 2
Department of Dermatology and Venereology
Faculty of Medicine Sriwijaya
s University/Dr. Mohammad Hoesin General Hospital Palembang, Indonesia
Email: sarah_diba_dr@yahoo.com
INTRODUCTION
• Paediatric psoriasis can have a profound long-term impact on the psychological health of affected children, and the
treatment remains a challenge.1
• Methotrexate remains the 1st line treatment of psoriasis1, but in Indonesia oral methotrexate was no longer available
since 2017. Some pa ents had abrupt withdrawal of methotrexate that trigger erythrodermic psoriasis.
• Cyclosporine is one of immunosuppressant drug that can be used for trea ng erythrodermic psoriasis, either as a single
therapy or as a sparing agent. Cyclosporine approved for treatment of psoriasis in adults, but the efficacy in pediatric
psoriasis remains unclear.2
CASE HISTOPATHOLOGY
• A 14-year-old Sumatrans boy • Epidermal spongiosis
p r e s e n t e d w i t h d i ffu e • Hyperkeratosis, parakeratosis
erythematous involving 92% skin • Microabscess Munro (black arrow)
surface with psoriasiform scale a er
abrupt withdrawal of methotrexate;
accompanied by chill and swelling
on both knees and feet.
• He has had psoriasis vulgaris since 7- Before treatment

Obj. 40x
month-old, it became more severe
since the last 4 years. Several
physicians and dermatologists have
had treated him with methotrexate
and steroid previously. Obj. 100x
TREATMENT
• He came to our hospital with moon
face, buffalo hump, skin atrophy, • Cyclosporine 50 mg twice daily
and failure to thrive (body weight 40 • Methylprednisolone 32 mg daily
kg and height 141 cm). • Emollient
• Mid-potent topical steroid
A er treatment
DISCUSSION
• The abrupt withdrawal of methotrexate has given enormous impact for pa ent, such as erythroderma.1
• Due to unavailability of oral methotrexate, we decided to start cyclosporine with ini al dose 2 mg/kg daily and
methylprednisolone with ini al dose equal to 1 mg/kg daily of prednisone. We started to taper the steroid off a er 4 weeks
of treatment, then gradually every 2 weeks. A er 5 months therapy the pa ent s ll on remission with 8 mg alternate dose
of methylprednisolone and 50 mg of cyclosporine twice daily .
• Cyclosporine has not been systema cally inves gated in paediatric popula ons with psoriasis, but it has been studied
extensively in paediatric atopic derma s, connec ve ssue disease, and diabetes mellitus and is consistently safe.3
• Pa ent monitoring is important to avoid severe adverse event. In this case, pa ent was monitored for complica on and
drug adverse event by laboratory examina on such as complete blood count, blood sugar level, renal and liver func on,
and electrolyte every 2 months. There was no significant laboratory result indicate complica on and adverse event.
CONCLUSION
The evidence of efficacy and safety of systemic agent for psoriasis therapy in childhood is s ll limited, although its safety and
efficacy for many other diseases has been proven. The decision to treat paediatric psoriasis with systemic agent should be
carefully assessed, and pa ent monitoring for poten al adverse event should be concerned.
REFFERENCES
1. Napolitano M, Megna M, Balato A, Ayala F, Lembo S. Systemic treatment of pediatric psoriasis: a review. Dermatol Ther (Heidelb). 2016;6:125–42.
2. Baskan EB, Yazici S, Tunali S, Saricaoglu H. Clinical experience with systemic cyclosporine: a treatment in severe childhood psoriasis. J Dermatolog Treat.
2015;27(4):328-31.
3. Pereira TM, Vieira AP, Fernandes JC, Sousa Basto A. Cyclosporine a treatment in severe childhood psoriasis. J Eur Acad Dermatol Venereol. 2006.;20:651-6.
Abstract ID RSMPD07
Track Other papulosquamous disorders
Contact author Ni Luh Putu Ratih Vibriyanti Karna
Country Indonesia
Organisation Udayana University/ Sanglah General Hospital
Poster: View Here
A Case Series of Guttate Childhood Psoriasis
Background: Guttate psoriasis is a form of psoriasis, which is commonly found in children. It is affecting 0.5% to 2% of
the pediatric population. Chronicity, inflammation and epidermal hyperproliferation are the cardinal features. Clearance
of disease may be difficult to achieve in most patients and relapse is frequent. Early recognition and management of
psoriasis in children and adolescents is vital in therapy.
Case I: An 8-year-old boy presented with itchy red scaly spots on scalp, trunk, and extremities since 5 years ago. He
frequently got cough and runny nose, the last symptoms was a week ago. There was no family history of psoriasis or
maternal history of any drug intake. Upon dermatological examination, multiple erythematous papules and
hypopigmented macules with white scales on top were noted on almost all over the body, sparing the face, palms, and
soles. Kaarsvlek phenomenon and Auspitz sign were positive. Skin biopsy had already obtained 4 years ago, and the
result suggested psoriasis. The patient was consulted to ENT department and diagnosed with acute rhinopharyngitis,
and treated with oral cefadroxil 500mg bid for 5 days. Other managements included mometasone furoate on the lesions
and moisturizer (urea) on all over the body.
Case II: A 3-year-old boy came with scaly white patches on almost all over the body. The patient was diagnosed with
HIV infection since 3 months of age and since then received antiretroviral therapy. Family history of psoriasis was
denied. There were multiple hypopigmented plaques, well-defined margin, with silvery white scales scattered on all over
the body. Skin biopsy was taken and confirmed psoriasis. Treatments included desoxymethasone on the lesions,
emollient (oleum olivarum), oral antihistamine, and continued ARV therapy.
Discussion: Childhood psoriasis is a well-known clinical entity with rare occurrence. The frequent clinical presentations
were plaque and guttate psoriasis. Guttate psoriasis is usually associated with streptococcal or HIV infections. Diagnosis
can be made clinically and supported with histopathological examination. Topical treatments are the mainstay therapy
of mild-moderate childhood psoriasis.
Keywords: Childhood psoriasis, guttate psoriasis, streptococcal infection, HIV.

Poster: RSMPD07
Karna N L P Vibriyanti, Sudarsa Prima Sanjiwani, Tandyono Venny

Department of Dermatology and Venereology, Udayana University,
Sanglah General Hospital, Denpasar-Bali, Indonesia
BACKGROUND:
Guttate psoriasis is a form of psoriasis, which is commonly found in children. It is affecting 0.5% to 2% of the
pediatric population. Chronicity, inflammation and epidermal hyperproliferation are the cardinal features. Clearance
of disease may be difficult to achieve in most patients and relapse is frequent. Early recognition and management of
psoriasis in children and adolescents is vital in therapy.
CASE I: An 8-year-old boy presented with itchy

red scaly spots on scalp, trunk, and extremities since Initial presentation 2 weeks after therapy
5 years ago. He frequently got cough and runny
nose, the last symptoms was a week ago. Neither
family history of psoriasis nor maternal history of any
drug intake were found. Upon dermatological
examination, multiple erythematous papules and
hypopigmented macules with white scales on top
were noted on almost all over the body, sparing the
face, palms, and soles. Kaarsvlek phenomenon and
Auspitz sign were positive. Skin biopsy had already
obtained 4 years ago, and the result suggested
psoriasis. The patient was consulted to ENT
department and diagnosed with acute
rhinopharingitis, and treated with oral cefadroxil
500mg bid for 5 days. Other managements included
mometasone furoate on the lesions and moisturizer
(urea) on all over the body.
CASE II: A 3-year-old boy came with scaly white Clinical presentation
patches on almost all over the body. The patient was
diagnosed with HIV infection since 3 months of age and
since then received antiretroviral therapy. Family history of
psoriasis was denied. There were multiple hypopigmented
plaques, well-defined margin, with silvery white scales
scattered on all over the body. Skin biopsy was taken and
confirmed psoriasis. Treatments included
desoxymethasone on the lesions, emollient (oleum
olivarum), oral antihistamine, and continued ARV therapy.
Histopathological examination
DISCUSSION: Childhood psoriasis is a well-known
clinical entity with rare occurrence. The frequent clinical
presentations were plaque and guttate psoriasis.
Guttate psoriasis is usually associated with
streptococcal or HIV infections. Diagnosis can be made
clinically and supported with histopathological
examination. Topical treatments are the mainstay
therapy of mild-moderate childhood psoriasis.
References
1. Thomas J, Parimalam K, Sindhu BR. Childhood psoriasis. Expert Rev. Dermatol. 2013; 8(5), 547-563
2. Gudjonsson JE, Elder JT. Psoriasis. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolf K, editors. Fitzpatrick’s Dermatology in General
Medicine. 8thed. New York: McGraw-Hill; 2012. p. 197-231.
3. Mrowietz U, Kragballe K, Reich K, Spuls P, Griffiths CEM, Nast A, et al. Definition of treatment goals for moderate to severe psoriasis: a European
consensus. Arch Dermatol Res. 2011; 303:1-10
Abstract ID RSMPD22
Track Other papulosquamous disorders
Contact author Kyla Janika Nerva
Country Philippines
Philippine Dermatological Society - East Avenue
Organisation
Medical Center Department of Dermatology
Poster View Here
Harry Potter and the PSORcerer's Skin: A Case of an 11-year-old male with Psoriasis
At eleven years old, Harry Potter and AJM found out that scars on their skin could have a profound long-term impact on
their lives. AJM is an 11-year-old previously well and physically active Filipino male who developed Psoriasis in the
absence of a family history. Psoriasis vulgaris is a chronic, non-communicable, disabling and disfiguring inflammatory
disorder. It can occur at any age, commonly between ages 50–69; however, 30–50 % of adults with psoriasis develop
the disease before 20 years of age.
Case:
AJM developed round erythematous plaques on his right knee on a previous site of trauma. In the interim, similar lesions
appeared on his bilateral thighs, knees, buttocks, upper extremities, anterior, posterior trunk, and face. This was
associated with pruritus and appearance of overlying thick white scales. On physical examination, there were
generalized multiple, well-defined, round to irregularly-shaped erythematous plaques topped with thick silvery white
scales and a positive Auspitz sign covering 45% BSA and a PASI score of 15. There were no scalp lesions, nail changes,
joint swellings or limitation of movement. Laboratory diagnostics were unremarkable. Of note ESR was normal.
Histopathology results were consistent with Psoriasis. Patient responded well to Clobetasol ointment + Petroleum Jelly
(1:1) twice daily, antihistamines, mild emollient and cleanser.
Discussion:
Psoriasis affects 2.0 – 3.5% globally and is characterised by complex interplay of alterations in epidermal growth and
differentiation from multiple biochemical, immunologic, and vascular abnormalities. Genetic studies show that HLACw6
and PSORs have been found to be associated with the disease and the former having the strongest association with
early onset psoriasis. Prevalence is increased in females compared with males < 20 years suggesting an interaction
between gender and the development of the psoriasis phenotype in paediatric patients. Geographical variation in
paediatric psoriasis is also observed as it is almost absent in an epidemiological study on childhood dermatoses in Asia
compared to Europe. The WHO Global Report on Psoriasis in 2016 found the same variation reporting 0% prevalence
in Asian children ages 6-11 in China and Taiwan suggesting that environmental factors may play a role in genetically
susceptible subjects.
Poster:RSMPD22
Abstract ID: UDH-10016
Track: Urticaria and Other Dermal Hypersensitivity Disorders
Contact Author: Sahana Srinivas
Country: India
Organisation: Others
Indira Gandhi institute of Child Health, Bangalore, Karnataka,
India
Poster Veiw Here
Systemic Mastocytosis Associated With Hodgkin’s Lymphoma In A 4 Year Old Child
Introduction
Systemic mastocytosis is a myeloproliferative disorder characterized by growth and accumulation of abnormal mast
cells in one or more organs, primarily skin, bone marrow, liver, spleen and lymph nodes. Majority of the associated
neoplasm are of myeloid origin, while lymphoid neoplasms associated with systemic mastocytosis is rare. The
association of systemic mastocytosis with a haematological non-mast cell lineage disease represents a specific subtype
of mastocytosis termed SM-AHNMD and is extremely rare in children. We present a 4 year old child with systemic
mastocytosis associated with Hodgkin’s lymphoma.
Case Report
A-4-year old male child presented with persisting itchy skin lesions on trunk and extremities from 3 years of age. There
was history of slowly progressive, non painful swelling on the left side of the neck from past 6 months. There was no
history of dysphagia or breathing difficulties. Cutaneous examination revealed multiple erythematous plaques with ‘peau
d orange’ surface on the chest, trunk, upper and lower limbs. Darier’s sign was positive. There was a single, large, non
tender, matted cervical lymphnode, rubbery in consistency measuring 10cm x 7cm on the left cervical region. Systemic
examination was normal. Hematology work up and biochemistry profile was normal. Absolute eosinophil count was
increased and peripheral smear showed eosinophilia. Serum tryptase levels was 80ng/ml. Ultrasound abdomen was
normal. Skin biopsy was consistent with cutaneous mastocytoma. Biopsy of the lymph node showed features of classical
Hodgkin’s lymphoma. Immunohistochemical studies of the mononuclear hodgkins and occasional reed-sternberg cells
showed PAX5 (dim) positive, CD 30, CD15, and LMP 1 immunoreactive and CD 20 and EMA negative. Background
showed CD3 positive T cells and CD20 positive lymphoid cells. Bone marrow aspirated smear showed hypercellularity
with > 10% atypical mast cells. Diagnosis of systemic mastocytosis with Hodgkin’s lymphoma was considered. The child
was started on chemotherapy and antihistamines for skin lesions with good results.
Conclusion
Our case met the diagnostic criteria of systemic mastocytosis. Co-existence of systemic mastocytosis and Hodgkin’s
lymphoma is diagnostically challenging as it depends on the rigour with which extracutaneous involvement is
investigated. Multidisciplinary approach with regular follow up and counseling is necessary
Poster: UDH-10016
SYSTEMIC MASTOCYTOSIS ASSOCIATED WITH

HODGKINS LYMPHOMA IN A 4 YEAR OLD CHILD
Sahana M Srinivas, Narendra Babu, Madhavi Naik, Rekha Kumar
Department of Pediatric Dermatology and Pediatric Surgery, Indira Gandhi Institute of Child Health,
Bangalore, Karnataka, India, Department of Pathology, St Theresa’s Hospital, Bangalore, Karnataka, India,
Department of Pathology, Kidwai Memorial Institute of Oncology
INTRODUCTION
Systemic mastocytosis (SM) is a myeloproliferative disorder characterized by growth and accumulation of abnormal mast cells in one
or more organs, primarily skin, bone marrow, liver, spleen and lymphnodes.
Majority of the associated neoplasm are of myeloid origin, while lymphoid neoplasms associated with systemic mastocytosis is rare.
We present a 4 year old child of SM with hodgkins lymphoma (HL) which is an extremely rare association.
REPORT OF A CASE
A-4-year old male child presented with persisting skin lesions on trunk and extremities from past 3 years. There was history of
progressive, non painful swelling on the left side of the neck from 6 months. There was no history of dysphagia or breathing difficulties.
There was no systemic involvement.
Cutaneous examination revealed multiple erythematous plaques and nodules with ‘peau d orange’ surface on the chest, trunk, upper
and lower limbs. Darier’s sign was positive [Fig 1, 2]. Well defined single large, non tender, matted lymph node, rubbery in consistency
measuring 10cms x 7cms seen on the left cervical region [Fig 3]. Systemic examination was normal.
Fig 1: Reddish Fig 2: Erythematous Fig 3: Single

brown, plaque with ‘peau d large, non tender,
smooth orange’ appearance matted , lymph
plaques with present on the node,
positive forearm measuring10cmsx
Darier's sign 7cms on the left
on the chest cervical region
Absolute eosinophil count was increased. Other hematology work up was normal. Peripheral smear showed eosinophilia. Serum
tryptase levels was 80ng/ml. Skin biopsy was consistent with cutaneous mastocytoma [Fig 4]. C-kit positivity was seen in mast cells [Fig
5]. Biopsy of the lymph node showed features of classical HL[Fig 6].
Immunohistochemical studies of the mononuclear hodgkins and occasional reed-sternberg (RS) cells showed PAX5 (dim) positive,
CD 30, CD15, and LMP 1 immunoreactive and CD 20 and EMA negative. CD3 positive T cells and CD20 positive lymphoid cells were
seen [Fig 7,8]. Bone marrow aspirated smear showed hypercellularity with > 10% atypical mast cells. Association of SM with HL was
considered. Child was started on chemotherapy and antihistamines for skin lesions with good results.
Fig 4: Sheets of mast cells Fig 5: c-kit (CD 117) Fig 6: RS cells, mononuclear Fig 7: CD 30 positive Fig 8: LMP 1 positive
(>50/hpf) with few membranous positivity in mast Hodgkins cells against a mononuclear Hodgkin cells Hodgkins cells in lymph
eosinophils in dermis (H & E cells (x40) polymorphous cells (x 40) in lymph node (X 40) node ( x40)
x10)
DISCUSSION
Our case met the diagnostic criteria of SM. SM associated with a non mast cell hematological disease is a specific subtype of SM (SM-
AHNHD) and is rarely seen in children
SM-AHNMD is the second most common type of SM representing 40% cases. It is associated more with hematological neoplasms.
The association with lymphoid neoplasm is unusual.
Bone marrow is affected in nearly 100% of patients with systemic manifestations. Bone marrow biopsy represent the gold standard in
the diagnosis, but often missed as the frequency of mast cells in bone marrow samples is very low.
CONCLUSION REFERENCES
Co-exixtence of SM and HL is diagnostically challenging as it depends on Gesljevic G, Grcar-Kuzmanov B, Grosel A, Sever M, Gazic B, Kloboves-
Prevodnik. Hodgkins Lymphoma is a rare form of clonal haematological non-mast
the rigour with which extracutaneous involvement is investigated. cell disease in systemic mastocytosis. Diagn Pathol 2015:10:5
Srinivas SM, Dhar S, Parikh D. Mastocytosis in children. Indian J Paediatr
Multidisciplinary approach with regular follow up and counseling is Dermatol 2015;16:57-63.
necessary.
Contact Author: Aninda FitriNugrahani
Country: Indonesia
dr. Saiful Anwar Regional General Hospital, Malang, East

Java Indonesia
Poster: View Here
HERPES ASSOCIATED ERYTHEMA MULTIFORME IN A 7-YEAR-OLD BOY
Background: Erythema multiforme (EM) is an acute mucocutaneous syndrome, self-limited disease with the
characteristics of target lesions, especially in young adulthood. Some of the precipitating factors are drugs and Herpes
Simplex Virus (HSV) infection. Herpes Associated Erythema Multiforme (HAEM) occurs with HSV as the precipitator.
Systemic steroids may be given as adjunctive therapy based on their immunosuppressive effects.
Case presentation: A 7-year-old boy weighing 25 kilograms complained about reddish patches and blisters on almost
entire body for five days. Six days before there were 3 small bumps containing water on his lips along with a mild fever.
One day later red patches appeared on the chest and palms, which then widen with blisters. There was no history of
taking medication before it appeared. His father often had herpes on the lips, the last infection occurred about 1 month
earlier. On dermatological examination there were target lesions on almost the entire body, erosion partially covered by
blackish crust on the mucosa of the eyes, lips, genitalia and, anus. Serological examination revealed positive IgG anti
HSV 1. Histopathologic features indicate the presence of vacuolar degeneration, keratinocyte necrosis, erythrocyte
extravasation, and perivascular lymphocyte cells. Therefore the patient was diagnosed with HAEM and given
methylprednisolone 3x8 mg injection a day, observed in the burn care unit and managed jointly with Pediatric, Plastic
Surgery and Ophthalmology Department. Clinical improvement of skin lesions and mucosa were obtained within 2
weeks.
Discussion: Herpes Associated Erythema Multiforme is a delayed type hypersensitivity reaction. Herpes Simplex
Viruses reach the skin through peripheral blood mononuclear cells (PBMC). The HSV antigen induce the CD4 + Th1
cells to produce Interferon-Î3 and initiate an inflammatory cascade. The diagnosis of HAEM is clinically established with
targeted lesions and serologic tests of HSV-1 and HSV-2. Systemic steroids can suppress the formation of cytokines
and chemokines, suppress the function of T cells, and reduce the attachment of inflammatory molecules in vascular
endothelium, thereby reducing the duration and severity of EM.
Keywords: herpes associated erythema multiforme, serologic HSV, methylprednisolone.

Poster: UDH-10046
HERPES-ASSOCIATED ERYTHEMA MULTIFORME

IN A 7-YEAR-OLD BOY
Aninda Fitri Nugrahani, Tantari Sugiman
Department of Dermatology and Venereology, Faculty of Medicine
Universitas Brawijaya/ dr. Saiful Anwar Regional General Hospital
Malang, Indonesia
BACKGROUND
Erythema multiforme (EM) is an acute mucocutaneous syndrome, which is self-limited and characterized by the presence
of target lesions, especially in young adulthood.1 Some of the precipitating factors are drugs and Herpes Simplex Virus
(HSV) infection. Herpes-Associated Erythema Multiforme (HAEM) occurs with HSV as the precipitator.2 Systemic steroids
may be given as an adjunctive therapy based on their immunosuppressive effects.3
CASE
A 7-year-old boy complained about reddish patches and blisters on almost his entire body in the past five days. Six days
before, there were 3 small bumps containing water on his lips accompanied with a mild fever. On the next day, the red
patches appeared on the chest and palms, and spread to all over his body along with the appearance of blisters. There
was no history of taking medication before the lesion appeared. His father often had herpes on the lips, the last infection
occurred about 1 month earlier.
General Examination Dermatological Examination
His general condition was compos

mentis with pulse 80x / minute,
respiratory rate 22x / minute, and
axillary temperature 36,6 oC. His
weight was 25 kg. At the eye
examination, the right and left
conjunctiva were hyperemia. No
edema was obtained in extremities or
enlarged lymph nodes either in the
colli, inguinal, or axilla regions.
Histopathological Figure 1A-B. Errosion on lips and orificium urethrae externa coverred with blackish
Examination crusts ( ) C-D. Target lesion on almost entire body ( ) E. Bullae in the centre of
target lesion ( ) F. Nikolsky sign positive ( )
Laboratory Examination Follow Up
Serological examination of IgG anti

HSV 1 >200 U/mL
( Normal : 20-25 U/mL)
Treatment
• Isolate in the burn care

Figure 2 A-B Vacuolar degeneration • Methylprednisolone 3x8 mg
( ), keratinocyte necrosis ( ), injection a day
erythrocyte extravasation ( ) in • Levocin ed 6x1 ODS
epidermal.(HE,100X) Lymphocyte • Fluorometholon ed 6x1 ODS Figure 3 A-E. Recovery of skin lesions in 2 weeks
infiltrate ( ) in dermal.(HE,400X) • Artificial tears 1 gtt/hour ODS showed hypo- and hyperpigmented patches
• Wound care moist dressing
DISCUSSION
Herpes-associated Erythema Multiforme is a delayed type hypersensitivity reaction.Herpes Simplex Viruses infects the
skin through peripheral blood mononuclear cells (PBMC). 4 The HSV antigen stimulates CD4 + Th1 cells to produce
Interferon-γ and to initiate an inflammatory cascade. The diagnosis of HAEM is clinically established by the presence of
targeted lesions and serologic tests of HSV-1 and HSV-2.5 Systemic steroids can suppress the formation of cytokines and
chemokines, the function of T cells, and the attachment of inflammatory molecules in The vascular endothelium, thereby
reducing the duration and severity of EM.3
REFERENCES
1. Roujeau JC. Erythema Multiforme, in Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K. editors. Fitzpatrick’s Dermatology in General Medicine, 8 th ed. New York: McGraw-Hill,
2012; p . 431-9
2. Sokumbi, O. and Wetter, D.A. Clinical features, diagnosis, and treatment of erythema multiforme: a review for the practicing dermato logist. Int j dermatol, 2012; 51(8): 889-902
3. Michaels, B. The role of systemic corticosteroid therapy in erythema multiforme major and Stevens-Johnson syndrome: a review of past and current opinions. Journal Clin Aesthet dermatol,
2009; 2(3): 51
4. Aurelian, L., Ono, F. and Burnett, J. Herpes simplex virus (HSV)-associated erythema multiforme (HAEM): a viral disease with an autoimmune component. Dermatol online J , 2003; 9(1):1
5. Sun, Y., Chan, R.K.W., Tan, S.H. and Ng, P.P.L. Detection and genotyping of human herpes simplex viruses in cutaneous lesi ons of erythema multiforme by nested PCR, J med virol, 2003; 71(3):
423-28
Contact Author: Valencia Long

Country: Singapore
Organization: Tan Tock Seng Hospital
Poster View here
Contact Urticaria To Oatmeal In A Young Child With Atopic Eczema: A Cautionary Tale In The Use Of Oatmeal
Containing Emollients
Background
Colloidal oatmeal has been used in dermatology for many decades for its soothing and calming effect on the skin in
conditions such as atopic dermatitis and irritant diaper dermatitis. Although oatmeal tends to have low allergenicity,
there is still a rare risk of contact sensitization.
Case presentation
We present an 18 month old Chinese female girl, with a history of atopic eczema from infancy, who developed acute
generalized contact urticarial following the topical use of an oatmeal containing emollient. The child had a strong family
history of atopy, and had been diagnosed with atopic eczema since 1 month old. Her eczema was controlled with the
use of topical steroids and emollients containing ceramide, glycerin and paraffin. Initially limited to the cheeks and limbs,
the eczema progressed to involve both periorbital areas when she was 9 months old. She was unable to tolerate the
use of topical calcineurin inhibitors, and an oatmeal containing emollient was used as a steroid sparing measure. She
responded well with improvement of periorbital eczema and the oatmeal containing emollient was used daily over her
whole body, with initial improvement of her eczema and reduced requirements for topical steroids.
However, after about 6 months of using the oatmeal containing moisturiser, there was gradual worsening of eczema
with no identifiable trigger. Cleansers were changed to the same brand containing oatmeal.
At 18 months of age, she presented with a generalized urticarial reaction with blistering immediately following the use
of the oatmeal containing cleanser and moisturiser. There was no upper airway obstruction. The moisturiser was
immediately washed off with another cleanser that did not contain oatmeal and she was administered syrup
fenofexadine and cetirizine orally. There was complete resolution of the urticarial wheals within 30 minutes.
On follow up at 2.5 years of age, the child continues to have atopic eczema but has not had any recurrence of acute
contact urticaria.
Discussion
We wish to highlight the rare but important risk of developing contact sensitization and urticaria in a young child
exposed to oatmeal containing emollients given the increasing use of such products in childhood atopic eczema.
Poster: UDH-10108
Contact urticaria to oatmeal in a young child

with atopic eczema: A cautionary tale in the
use of oatmeal containing emollients
V Long1, M.W.T Liang2
1Tan Tock Seng Hospital, Singapore 2 National Skin Centre, Singapore
INTRODUCTION
Colloidal oatmeal has been used in
dermatology for many decades for its
soothing and calming effect on the skin in
conditions such as atopic dermatitis and
irritant diaper dermatitis. Although oatmeal
tends to have low allergenicity, there is still a
rare risk of contact sensitization
CASE REPORT
We present an 18 month old Chinese female
girl, with a history of atopic eczema from
infancy, who developed acute generalized
contact urticarial following the topical use of
an oatmeal containing emollient.
Figure 1: Atopic eczema affecting cheeks and
The child had a strong atopic family history, periorbital regions
and had been diagnosed with atopic eczema
since 1 month old. Her eczema was
controlled with the use of topical steroids
and emollients containing ceramide, glycerin
and paraffin. Initially limited to the cheeks
and limbs, the eczema progressed to involve
both periorbital areas when she was 9
months old (Figure 1). She was unable to
tolerate the use of topical calcineurin
inhibitors, and an oatmeal containing
emollient was used as a steroid sparing
measure. She responded well with
improvement of periorbital eczema and the
oatmeal containing emollient was used daily
over her whole body, with initial
improvement of her eczema and reduced
requirements for topical steroids.
Six months after using the oatmeal

containing moisturiser, there was gradual Figure 2: Generalised urticaria with blistering seen
worsening of eczema with no identifiable DISCUSSION
trigger. Cleansers were changed to the same We wish to highlight the rare but important risk of
brand containing oatmeal. At 18 months of developing contact sensitization and urticaria in a
age, she presented with a generalized young child exposed to oatmeal containing emollients
urticarial reaction with blistering (Figure 2) given the increasing use of such products in childhood
immediately following the use of the atopic eczema. In a 2007 prospective study by
oatmeal containing cleanser and moisturiser. Boussalt et al1, 302 atopic children were subjected to
There was no upper airway obstruction. The atopy patch testing and skin prick testing to oat
moisturiser was immediately washed off proteins (1,3,5%) as well as the European standard
with another cleanser that did not contain test series, Subsequently an oral food challenge test
oatmeal and she was administered syrup and repeated open application test (ROAT) was
fenofexadine and cetirizine orally. There was conducted in the oat-sensitized group. This study
complete resolution of the urticarial wheals demonstrated that children under 2 years of age were
within 30 minutes. more likely to have a positive APT. Thirty-two percent
of oat cream users had oat-positive APT vs 0% in the
On follow up at 2.5 years of age, the child non-user group. Oat sensitization in children,
continues to have atopic eczema but has not particularly those younger than 2 years old, may be
had any recurrence of acute contact due to repeated applications of oat creams on a
urticaria. predisposed impaired epidermal barrier. This case
1) Boussault, P., Léauté-Labrèze, C., Saubusse, E., Maurice-Tison et further illustrates the potential dangers of using
al. Oat sensitization in children with atopic dermatitis: prevalence,
risks and associated factors. Allergy, 2007; 62: 1251–1256. colloidal oatmeal in infants and young children
Abstract ID UDH-10111
Track Urticaria and Other Dermal Hypersensitivity Disorders
Contact author Chyntia Giska Aryunisari
Country Indonesia
Organisation Universitas sumatera utara general hospital
Poster View Here
MISDIAGNOSE OF DERMATITIS HERPETIFORMIS IN CHILD, WHY?
Background: Dermatitis herpetiformis (DH) is a chronic pruritic cutaneous eruption associated with gluten-sensitive
enteropathy (celiac disease [CD]) and immunoglobulin A (IgA) deposition in the skin. While the disease is not uncommon
among adolescents, DH is rarely seen in prepubertal patients. Children with DH present similarly to adults; however,
uncommon skin findings have been reported. Because of an increased risk for autoimmune diseases and lymphoma,
accurate diagnosis and treatment are imperative.
Case: A 7 years old child, presented with itchy erosion in her back foot since 2 weeks before visit. Two weeks before,
there were group of vesicles on the back of her foot. The itchy vesicle always recurred symmetrically on her upper and
lower extremity also on her buttocks for 2 years. She was previously diagnosed with atopic dermatitis and psoriasis.
Because the lesions are intensely pruritic she always scratching the vesicles until all of them becomes erosion. The
examination of dermatopathology show cleft between dermis and epidermis which support diagnose of DH. This patient
given an education of free-gluten diet and planned to get dapsone.
Discussion: Childhood DH is rare, with an uncertain incidence and prevalence. Most children receive the diagnosis
between the ages of 2 and 7 years. Among childhood cases, there is a female predominance. The clinical presentation
of DH is characterized by symmetrically distributed papulovesicular lesions and urticarial plaques, often favouring the
back, buttocks, and extensor surfaces of the extremities. Because the lesions are intensely pruritic, intact vesicles are
rarely observed by the clinician. Thus, childhood DH often misdiagnosed as atopic dermatitis, papular urticaria, scabies,
linear IgA dermatosis, or chronic urticaria. Rare case of childhood DH may contribute to misdiagnose of DH in child.
Keywords : Dermatitis Herpetiformis, Child, gluten sensitive

Poster UDH-10111
Abstract ID RSMPD04
Track Urticaria and other dermal hypersensitivity disorders
Contact author Irene Jessica Soputro
Country Indonesia
Udayana University, Sanglah General Hospital

Organisation
Indonesia
Poster View here
Erythema Multiforme Following Nonspecific Febrile Illness in a Paediatric Patient
Introduction
Erythema multiforme (EM) is an acute, inflammatory eruption affecting skin and mucous. The condition mostly affects
young adolescent, with rare portion in children under 3 and adult over 50 years old. EM is classified as minor and major,
depending on whether there is a mucosal involvement. Commonly known to be associated with Herpes Simplex Virus
(HSV) or Mycoplasma pneumoniae infection, some EM presented without specific pathogens. This case reports an EM
major following nonspecific febrile illness.
Case
A 3-year-old Asian girl was brought to seek medical advice with complaint of multiple red patches on face, trunk, and
extremities for 2 days. Patient experienced acute fever occurred 5 days prior to the development of skin lesions. Skin
eruption shows typical lesions with circular, wheal-like erythematous halo on periphery, infiltrated pale ring towards the
center, and violaceous central disk at the innermost. Some lesions display only two rings, giving the appearance of
raised atypical targets. Ocular mucous was involved with conjunctival injection manifestation besides minimal erosions
on oral mucous. There were no clinical signs and symptoms of systemic condition other than the fever per se. There
was no history of drug exposure within the plausible period. Supportive examination revealed no abnormalities other
than high erythrocyte sedimentation rate. Diagnosis of EM major was achieved from history taking and clinical
examination. We managed the patient with systemic corticosteroid and improvement was seen within a week of
hospitalization.
Discussion
The exact ethology of EM remains unclear, notwithstanding studies had shown relationship between EM and existed
HSV or M.pneumoniae infection, of which our patient did not meet the criteria. In state where other risk factors had been
excluded and infection seems to be the most possible causative agent, it is essential to understand that EM in children
might not presented with specific pathogens as the potential triggers. Thorough anamnesis and examination should be
conducted to minimize the chance of pediatric EM misdiagnosis.
Poster: RSMPD04
BACKGROUND
Erythema multiforme (EM) is an acute, inflammatory eruption affecting skin and mucous. The
condition mostly affects young adolescent, with rare portion in children under 3 and adult over 50
years old. EM is classified as minor and major, depending on whether there is a mucosal
involvement. Commonly known to be associated with H erpes Sim plex V irus (HSV) or M ycoplasm a
pneum oniae infection, some EM presented without specific pathogens. This case reports an EM
major following nonspecific febrile illness.
CASE
A 3-year-old Asian girl was brought to seek medical advice
with complaint of multiple red patches on face, trunk, and
extremities for 2 days. Patient experienced acute fever
occurred 5 days prior to the development of skin lesions.
Skin eruption shows typical lesions with circular, wheal-
like erythematous halo on periphery, infiltrated pale ring B
towards the center, and violaceous central disk at the
innermost. Some lesions display only two rings, giving the
A
appearance of raised atypical targets. Ocular mucous was
involved with conjunctival injection manifestation besides
minimal erosions on oral mucous. There were no clinical
sign and symptoms of systemic condition other than the
fever per se. History of drug exposure within plausible
period was denied. Supportive examination revealed no
abnormalities other than high erythrocyte sedimentation C D
rate. Diagnosis of EM major was achieved from history A, Lesions on trunk and extremities; B,
taking and clinical examination. We managed the patient Typical target lesion with three concentric
with systemic corticosteroid and improvement was seen rings; C, Atypical target lesion showing
only two rings; D, Improvement on the
within a week of hospitalization. lesions after steroid administration
DISCUSSION
The exact etiology of EM remains unclear, notwithstanding studies had shown relationship
between EM and existed HSV or M . pneum oniae infection, of which our patient did not meet the
criteria. In state where other risk factors had been excluded and infection seems to be the most
possible causative agent, it is essential to understand that EM in children might not presented
with specific pathogens as the potential triggers. Thorough anamnesis and examination should be
conducted to minimize the chance of pediatric EM misdiagnosis
REFERENCES
1. Roujeau, J. Erythema Multiforme. In: Goldsmith LA, Katz SI, editors. Fitzpatrick’s Dermatology in
General Medicine. 8th ed. New York: McGraw Hill; 2012; 39: 431-8.
2. Samim F, Auluck A, Zed C, Williams PM. Erythema multiforme: a review of the epidemiology,
pathogenesis, clinical features, and treatment. D en t C lin N A m . 2013;57:583-96.
3. Sokumbi O, Wetter DA. Clinical features, diagnosis, and treatment of erythema multiforme: a review
for the practicing dermatologist. In t J D erm a to l. 2012;51:889-902.
Abstract ID RSMPD08
Contact author Nopriyati Husan Haluan

Country Indonesia
Organisation Mohammad Hoesin General Hospital Palembang
Poster View Here
CHRONIC IDIOPATHIC URTICARIA IN PAEDIATRIC PATIENTS BELOW 12 YEARS OF AGE TREATED WITH
OMALIZUMAB:
A CASE SERIES
Abstract
Background. Generally, 15-25% of general population experience urticaria during their life. The prevalence of chronic
urticaria is about 0.1-0.3% in children and most often occurs between ages of 6-11 years. There are several causes for
development of chronic urticaria. Known etiologies of chronic urticaria in children vary from 21% to 83%. The rest is
idiopathic. Similar to adults, antihistamines are the first line of treatment. Omalizumab as a biological engineering
molecule is a recombinant humanized monoclonal antibody, which targets the CH3 domain of the ε chain of the free
IgE. Omalizumab has been used in adults and adolescents patients (12 years of age and above) with H1-antihistamine-
refractory chronic idiopathic urticaria (CIU). Safety and efficacy of Omalizumab in pediatric patients with CIU below 12
years of age have not been established.
Case Presentation. This is a case series of 3 paediatric patients who suffered from CIU and treated by Omalizumab.
The 1st patient had a history of acute urticaria and angioedema induced by food allergy. The 2nd patient had a history
of acute urticaria treated with dexamethasone injection. And the 3rd patient had a history of chronic urticaria and
bronchial asthma. All patients received antihistamines as the 1st line therapy, and tapered up to 4 folds as the 2nd line
therapy, but the result that evaluated by counting the weekly urticarial activity score (UAS) was not really satisfying. All
patients injected with Omalizumab 75 mg 3 times and show a good clinical response, with weekly UAS decreased to 0.
The adverse effect founded was only ascariasis in the 1st patient.
Discussion. The evidence of efficacy and safety of Omalizumab therapy for CIU in paediatric patients below 12 years
of age is still limited. But there were several cases reported showed complete response to Omalizumab treatment and
was symptom free with no serious adverse effect. Omalizumab is a new promising therapeutic modality. However,
further study of efficacy and safety for children below 12 years of age still needed.
Keywords: idiopathic chronic urticaria, omalizumab, paediatric

Poster: RSMPD08
CHRONIC IDIOPATHIC URTICARIA IN PAEDIATRIC PATIENTS

BELOW 12 YEARS OF AGE TREATED WITH OMALIZUMAB:
A CASE SERIES
Nopriya 1, Citra Tresna Mur 2
Faculty of Medicine Sriwijaya University/Dr. Mohammad Hoesin General Hospital Palembang, Indonesia
Email: nopriya husan@gmail.com
INTRODUCTION
• The prevalence of chronic ur caria is about 0.1-0.3% in children and most o en occurs between ages of 6-11 years.1
• Known e ologies of chronic ur caria in children vary from 21% to 83%. The rest is idiopathic.1
• Omalizumab has been used in adults and adolescents pa ents (12 years of age and above) with H1-an histamine-
refractory chronic idiopathic ur caria (CIU).2
• Safety and efficacy of Omalizumab in pediatric pa ents with CIU below 12 years of age have not been established.2
CASE 1 CASE 2 CASE 3
Before A er Before A er Before A er

treatment treatment treatment treatment treatment treatment
The 1st pa ent had a history of acute The 2nd pa ent had a history of acute The 3rd pa ent had a history of chronic
ur caria and angioedema induced by ur caria treated with dexamethasone ur caria and bronchial asthma.
food allergy. injec on.
TREATMENT
• Omalizumab 75 mg 3 mes (every 4 weeks)
DISCUSSION
• Omalizumab is a recombinant humanized monoclonal an body that reduces levels of circula ng immunoglobulin E (IgE)
and expression of IgE high-afinity receptors on mast cells and basophils, interrup ng the subsequent allergic inflammatory
cascade.1
• Current indica ons for treatment with Omalizumab in paediatric pa ents are clearly defined and are confined to
moderate-to-severe uncontrolled allergic asthma and CIU.1
• In one study, 8-year old boy with refractory CIU with associated angioedema who had no response to cyclosporine and
frequent steroid therapy, showed complete response to Omalizumab treatment and was symptom free.2
• In our case, 3 children with a history of chronic ur caria received an histamines as the 1st line therapy, and tapered up to
4 folds as the 2nd line therapy, but the result evaluated by coun ng the weekly UAS was not really sa sfying. All pa ents
injected with Omalizumab and showed a good clinical response, with weekly UAS decreased to 0. The adverse effect
founded was only ascariasis in the 1st pa ent.
CONCLUSION
The evidence of efficacy and safety of Omalizumab therapy for CIU in paediatric pa ents below 12 years of age is s ll limited.
But there were several case reports showed complete response to Omalizumab treatment and was symptom free with no
serious adverse effect. Omalizumab is a new promising therapeu c modality. However, further study of efficacy and safety
for children below 12 years of age s ll needed.
REFFERENCES
1. Licari A, Marseglia A, Caimmi S, Castagnoli R, Foiadelli T, Barberi S, et al. Omalizumab in children. Pediatr Drugs. 2014;16:491-502
2. Ghaffari J, Shahmohammadi S, Ashrafi H, Ranjbar AR, Ghaffari N. Omalizumab (Xolair) in children above 12 years with chronic ur caria: a review of
literature. J Pediatr Rev. 2015;3(1):e152
Abstract ID RSMPD10
Contact author I Gusti Agung Ayu Dwi Karmila

Country Indonesia
Organisation Udayana University/ Sanglah Hospital

Poster View Here
A CASE SERIES CHRONIC BULLOUS DISEASE OF CHILDHOOD
Background:
Chronic bullous disease of childhood (CBDC) is a rare blistering disease that occurs predominantly in children younger
than 5 years of age. The clinical presentations are characterized by the development of tense bullae and may occur in
cluster, giving a “cluster of jewels” appearance. CBDC is a self-limited disease, and remission usually occurs in 2 years.
Case I: A 1,5 year-old-boy presented with generalized pruritic bullous lesions for 15 days. He had widespread, tense
blisters of various size and annular lesions so-called the “cluster of jewels” sign. No involvement is found on oral mucosa.
Histologic examination of the bullous lesion showed subepidermal blister containing some neutrophils and eosinophils.
The skin lesions responded well to systemic corticosteroid (prednisolone and mycophenolate mofetil).
Case II : A 1 year-old-boy came with complaint of itchy blistering eruption confined to the skin since 1 month ago.
Dermatologic examination showed multiple vesicles and tense blister arising from erythematous skin on extremities,
forearms, trunk and face. The vesicles and tense blisters were seen in annular lesion, and some of them were grouped,
described as the “rosette pattern” or “cluster of jewels” sign. The oral mucosa was not involved. Histopathology revealed
subepidermal blister containing some neutrophils and eosinophils. Treatment with systemic steroid was indicated due
to widespread disease. The patient was treated with prednisolone. There was a clinical improvement after 20 days of
therapy.
Discussion:
Chronic bullous disease of childhood (CBDC) is a nonhereditary, autoimmune subepidermal bullous disease, and is
seen more common in males. A direct immunofluorescence examination from perilesional skin is the gold standard,
where linear IgA depositions along the basement membrane were typically found. The target of treatment is to achieve
a long lasting remission with minimum side effect. Adjuvant therapy can be given if treatment is unresponsive.
Key words: chronic bullous disease of children, cluster of jewels, therapy

Poster: RSMPD10
A CASE SERIES CHRONIC BULLOUS DISEASE OF

CHILDHOOD
Karmila IGAA Dwi, Rusyati Luh Made Mas, Praharsini IGAA
Pramita Nyoman Yoga Maya
BACKGROUND
Chronic bullous disease of childhood (CBDC) is a rare blistering disease that occurs predominantly in
children younger than 5 years of age. The clinical presentations is characterized by the development of
tense bullae and may occur in cluster, giving a “cluster of jewels” appearance. CBDC is a self limited
disease, and remission usually occurs in 2 years.
CASE I : A 1,5 year-old-boy presented with generalized pruritic bullous

lesions for 15 days. He had widespread, tense blisters of various size
and annular lesions so-called the “cluster of jewels” sign. No
involvement is found on oral mucosa. Histologic examination of the
bullous lesion showed subepidermal blister containing some
neutrophils and eosinophils. The skin lesions responded well to
systemic corticosteroid (prednisolone and mycophenolate mofetil).
INITIAL PRESENTATION 20 DAYS AFTER THERAPY
CASE II : A 1 year-old-boy came with complaint of itchy blistering eruption confined to the
skin since 1 month ago. Dermatologic examination showed multiple vesicles and tense
blister arising from erythematous skin on extremities, forearms, trunk and face. The
vesicles and tense blisters were seen in annular lesion, and some of them were grouped,
described as the “rosette pattern” or “cluster of jewels” sign. The oral mucosa was not
involved. Histopathology revealed subepidermal blister containing some neutrophils and
eosinophils. Treatment with systemic steroid was indicated due to widespread disease.
The patient was treated with prednisolone. There was a clinical improvement after 20
days of therapy.
INITIAL PRESENTATION 20 DAYS AFTER THERAPY DISCUSSION : Chronic bullous disease of

childhood (CBDC) is a nonhereditary,
autoimmune subepidermal bullous
disease, and is seen more common in
males. A direct immunofluorescence
examination from perilesional skin is the
gold standard, where linear IgA
References depositions along the basement
1. Rao CL, Hall RP III. Linear Immunoglobulin A Dermatosis and
Chronic Bullous Disease of Childhood. In: Wolff K, Goldsmith LA,
membrane were typically found. The
Katz SI, Gilchrest BA, Paller AS, Leffell DJ. Eds. Fitzpatrick’s target of treatment is to achieve a long
Dermatology In General Medicine. 7th ed. New York: McGraw Hill. lasting remission with minimum side
2008. p.485-528. effect. Adjuvant therapy can be given if
2. Patsatsi A. Chronic Bullous Disease or Linear IgA Dermatosis of treatment is unresponsive.
Childhood – Revisited. J Genet Syndr Gene Ther. 2013; 4(6). p.1-5.
Abstract ID RSMPD20
Contact author Risty Hafinah
Country Indonesia
Organisation Universitas Padjadjaran Bandung Indonesia
Poster View here
A case of chronic bullous disease of childhood which was treated with sulfasalazine
Abstract
Background:
Chronic bullous disease of childhood (CBDC) or linear IgA bullous dermatosis of childhood is a rare, chronic, and
nonhereditary bullous autoimmune disease. It affects often during the first decade of life. CBDC is characterized by
tense bullae and vesicles on normal or erythematous skin, forming annular or polycyclic plaques, named “cluster of
jewels”. The diagnosis of CBDC is based on clinical, histopathological, and immunofluorescence test. The treatment of
CBDC is dapsone and sulfasalazine as well as sulfapyridine as second line treatment.
Case:
We reported a case of 17-year-old boy, diagnosed as CBDC that presented as multiple pruritic tense blisters and
erythematous plaques over the limbs and arms for the last six months. There was a history of upper respiratory tract
infection. Physical examination showed tense bullae, vesicles, forming annular plaques, and cluster of jewels on limbs
and arms. Histopathology from the skin of vesicle revealed subepidermal blister consisted of neutrophils and
lymphocytes. DIF test of perilesional skin showed no IgA, IgG, IgM deposits. He was diagnosed as CBDC and treated
with sulfasalazine 60 mg per body weight daily divided into two doses. The clinical improvement was achieved one
month of starting treatment without any side effects. The dose of sulfasalazine was gradually decreased over one month
and planned to continue until three years.
Discussion:
The first line treatment of CBDC is dapsone. If the patient cannot tolerate dapsone or unavailable, sulfapyridine or
sulfasalazine as second line therapy. Sulfasalazine undergoes azo-reductive cleavage by bacteria into its two major
metabolites: 5-ASA and sulfapyridine. Like dapsone, sulfasalazine inhibit synthesis of the neutrophil chemotactic factor,
leukotriene B4, and granulocyte-mediated histamine release from mast cells. There have been rare reports regarding
CBDC treated with sulfasalazine. The treatment of sulfasalazine showed excellent response in this patient and effective
as well as safe treatment for CBDC.
Key words: chronic bullous disease of childhood, cluster of jewels, sulfasalazine

Poster: RSMPD20
CHRONIC BULLOUS DISEASE OF CHILDHOOD WHICH WAS TREATED WITH

SULFASALAZINE
Risty Hafinah, R.M. Rendy Ariezal Effendi, Reiva Farah Dwiyana, Inne Arline Diana
Faculty of Medicine Universitas Padjadjaran/ Hasan Sadikin Hospital, Bandung
Email: ristyha@gmail.com
BACKGROUND
Chronic bullous disease of childhood (CBDC) or linear IgA bullous dermatosis of childhood is a rare,
chronic, and nonhereditary bullous autoimmune disease.1,2 It affects often during the first decade of life.3
CBDC is characterized by tense bullae and vesicles on normal or erythematous skin, forming annular or
polycyclic plaques, named “cluster of jewels”.3 The diagnosis of CBDC is based on clinical,
histopathological, and immunofluorescence test. The treatment of CBDC is dapsone and sulfasalazine as
well as sulfapyridine as second line treatment.4
Before treatment
CASE REPORT
We reported a case of 17-year-old boy,
diagnosed as CBDC that presented as multiple
pruritic tense blisters and erythematous plaques over
the limbs and arms for the last six months. There
were history of upper respiratory tract infection.
Physical examination showed tense bullae,
vesicles, forming annular plaques, and cluster of Cluster of
jewels on limbs and arms. Histopathology from the jewels
skin of vesicle revealed subepidermal blister
consisted of neutrophils and lymphocytes. DIF test of
perilesional skin showed no IgA, IgG, IgM deposits. After treatment
He was diagnosed as CBDC and treated with
sulfasalazine 60 mg per body weight daily divided
into two doses. The clinical improvement was
achieved one month of starting treatment without any
side effects. The dose of sulfasalazine was gradually
decreased over one month and planned to continue
until three years
DISCUSSION
The first line treatment of CBDC is dapsone. If the
patient can not tolerate dapsone or unavailable,
sulfapyridine or sulfasalazine as second line therapy.
Sulfasalazine undergoes azo-reductive cleavage by
bacteria into its two major metabolites: 5-ASA and
sulfapyridine. Like dapsone, sulfasalazine inhibit synthesis
of the neutrophil chemotactic factor, leukotriene B4, and 4A
granulocyte-mediated histamine release from mast cells.4,5
There have been rare reports regarding CBDC treated
with sulfasalazine. The treatment of sulfasalazine showed
excellent response in this patient and effective as well as
safe treatment for CBDC Figure. Histopathology: Subepidermal blister consisted of
neutrophlis and lymphocytes
REFERENCES
1. Rao CL, Hall RP. Linear immunoglobulin A dermatosis and chronic bullous disease of childhood. Dalam: Goldsmith LA, Katz SI, Gilchrest A, Paller AS, Laflell DJ, Wolf Klaus,
penyunting. Fitzpatrick’s dermatology in general medicine. Edisi ke-8. New York: McGraw-Hill Incorporation; 2012. hlm.623-9.
2. Kenani N, Mebazana A, Denguezli M, Ghariani N, Sriha B, Belajouva C. Childhood linear IgA bullous dermatosis in Tunisia. Pediatr Dermatol. 2009;26:28-33.
3. Patsatsi A. Chronic bullous disease or linear IgA dermatosis of childhood-revisited. J Genet Syndr Dene Ther. 2013;4(6):1-5.
4. Fortuna G, Marinkovich MP. Linear immunoglobulin IgA bullous dermatosis. Clin Dermatol. 2012;30:38-50.
5. Mintz EM, Morel KD. Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin. 2011;29:459-62.
Abstract ID: CTD-10000
Track: Connective Tissue Disorders
Contact Author: Xinjie Jonathan Tang
Country: Singapore
Yong Loo Lin School of Medicine
Poster View Here
BULLOUS SYSTEMIC LUPUS ERYTHEMATOSUS WITH SYSTEMIC CORTICOSTEROID
Abstract
Background: Bullous Systemic Lupus Erythematosus (BSLE) is an autoantibody-mediated disease characterised by

sub-epidermal blisters, a rare clinical subtype of Systemic Lupus Erythematosus (SLE). This condition predominantly
occurs in women in the second to fourth decade of life, while very rare in children, affecting only 1% of total pediatric
patients with SLE in the past 30 years. Dapsone is the primary choice of therapy, followed by corticosteroid and
immunosuppressant as the alternative therapies.
Case Presentation: An 11-year-old girl complained of clear fluid-filled blisters on her arms and legs since four days
before admission. No itching and no history of previous blisters. The patient also complained of joint pain, fever,
weakness, and decreased of appetite. The patient has been diagnosed with SLE by Pediatric Department. General
examination results was within normal limits, body weight was 27 kgs and body height was 135 cms. Dermatological
examination of the left arm revealed multiple vesicles and tense bullae with erythematous base filled with clear fluid,
well-defined borders, irregular shapes, and vary in size. The Nikolsky sign and Asboe-Hansen sign were negative.
Dermatological examination of the right arm and both legs revealed multiple vesicles with erythematous base filled with
clear fluid, well-defined borders, irregular shapes, and variable in size. Laboratory examination showed anemia,
lymphocytopenia, and positive Anti-Nuclear Antibodies (ANA) titers. Histopathology features showed vacuolar
degeneration of basal cells, dermoepidermal cleavage, and perivascular infiltration of lymphocytes and neutrophils in
papilla dermis. Patient was treated with intravenous methylprednisolone 30 mg/day for 2 weeks before being tapered,
chloroquine 150 mg/day, gentamicin cream and wet dressing. Significant clinical improvement appeared within one
week.
Discussion: In the treatment of BSLE, dapsone is the effective basic therapy. Hydroxychloroquine, though commonly
used to control the cutaneous lesions and disease activity of SLE, does not effective for BSLE eruptions. Corticosteroid
is one of the alternative drug for patients who have contraindication to dapsone. Corticosteroid gives slow progress for
BSLE, but can control the other systemic manifestations. In this patient, systemic corticosteroid therapy, 1-2 mg/kg/day
for one week gave a rapid positive response.
Keywords: bullous systemic lupus erythematosus, systemic corticosteroid, children.

Poster: CTD-10000
Paediatric cutaneous lupus erythematosus: A 10-

year experience in a tertiary dermatological centre
TANG Xinjie Jonathan1, KER Khor Jia2, HO Sheun Ling Madeline2, GAN Emily Yiping2
Yong Loo Lin School of Medicine, Singapore1; National Skin Centre, Singapore2
Introduction Methods Results Discussion Introduction Methods Results Discussion

Lupus erythematosus (LE) is an autoimmune disease due to Majority (86.7%, n=13) required combination therapy with
autoantibody production and immune complex formation. systemic as well as topical agents. Tables 3 and 4
Many organ systems may be involved, including the skin. summarise the systemic and topical agents used in
combination therapy.
LE is uncommon among children. However, compared to
adult onset SLE, it has been reported that paediatric At time of last follow-up, disease remission was achieved in
systemic LE has been associated with an increased only 2 patients (13.3%), both of whom required combination
frequency of malar rash and mucocutaneous ulcers. therapy. Two other patients (13.3%) experienced remission
Paediatric LE is also associated with more active disease at on combination therapy, but subsequently relapsed.
diagnosis, with patients developing damage at an increased Systemic medications prescribed, by regime Prescribed (%)
rate. Oral prednisolone alone 15.4
Hydroxychloroquine alone 15.4

We describe the clinical features and treatment responses of
Methotrexate alone 7.7
paediatric LE patients seen at our tertiary dermatological
centre. Both oral prednisolone and hydroxychloroquine only 23.1
Azathioprine, oral prednisolone and hydroxychloroquine 23.1

Introduction Methods Results Discussion Methotrexate, oral prednisolone and hydroxychloroquine 7.7
Mycophenolate, oral prednisolone and hydroxychloroquine 7.7

Patients diagnosed with cutaneous LE between 2007 and
Table 3. Systemic agents in combination therapy
2016 at the National Skin Centre were identified from our
clinical and histological databases. Patients were included if Topical medications prescribed Prescribed (%)
they were diagnosed before 16 years of age and excluded if Corticosteroids alone 84.6
they were cases of neonatal lupus. Calcineurin inhibitor alone 7.7
Corticosteroid and calcineurin inhibitor 7.7

Analysis of the patients’ clinical, laboratory and histological
data was performed. Table 4. Topical agents in combination therapy
Introduction Methods Results Discussion

15 children (13 females (86.7%) and 2 males (13.3%)) were
diagnosed with LE. Five patients (33.3%) were also
diagnosed with systemic LE. The mean age of diagnosis of
13.0 years (range 9-16). The mean duration of follow up
23.6 months (range 1-98).
The most common subtype of LE in this series was Discoid

LE (DLE). All patients presented with erythematous
plaques/patches, but only 7 (46.7%) complained of
photosensitivity. Other presenting features are summarised Fig 5. Clinical photographs of a 14 year old child with DLE, showing
multiple erythematous plaques over the forehead, with some lesions
in Table 1. associated with scaling (a); and an erythematous plaque on the
triangular fossa of the left ear, associated with follicular plugging (b).
Serological results of those evaluated are summarised in
Table 2, with ANA as the most common positive serological Introduction Methods Results Discussion
test (60.0%).
This study summarises the experience with paediatric LE at
a dermatological centre over a 10-year period. The findings
6.7% Presenting Present Serological Positive
6.7% features (%) test (%)
of our study are in keeping with studies done in other
Erythematous dermatological centres.
6.7% plaques/patches 100% ANA 60%
Photosensitivity 46.7% Anti ds-DNA 50%

Less than half of the patients complained of photosensitivity.
73.3% Antinuclear antibody was positive in approximately two-
Scaly lesions 33.3% Anti-RNP 40%
thirds of patients tested. Hence, the clinician would have to
Scarring alopecia 26.7% Anti-Sm 30% rely on a high degree of suspicion and clinic-pathological
correlation to diagnose and treat these patients.
DLE LE tumidus Scarring of skin 13.3% Anti-Ro 20%
References:
Subacute LE Acute LE Anti-La 0% 1. Dickey BZ et al. Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric
Oral ulcers 6.7% dermatology referral centre. Br J Dermatol. 2013;169(2):428-433. doi:10.1111/bjd.12383
2. Moises-Alfaro C et al. Discoid lupus erythematosus in children: clinical, histopathologic, and follow-up features in 27
Fig 1. Lupus subtypes Table 1. Presenting features Table 2. Positive serology cases. Pediatr Dermatol. 20(2):103-107.
3. Joo YB et al. Differences in Clinical Features and Mortality between Childhood-onset and Adult-onset Systemic Lupus
Erythematosus: A Prospective Single-center Study. J Rheumatol. 2016;43(8):1490-1497. doi:10.3899/jrheum.151129.
Contact Author: Clare Fern Lim
Country: Singapore
Yong Loo Lin School of Medicine
Poster View Here
A 10-Year Retrospective Study Of Morphoea In An Asian Paediatric Population
Introduction and Objectives:

Morphoea is a rare but potentially disabling fibrosing disorder of the skin and underlying tissues. We studied the clinico-
epidemiologic features and treatment of morphoea in a Southeast Asian paediatric cohort.
Materials and Methods:

A retrospective, cross-sectional study was conducted at the National Skin Centre in Singapore. Clinical, laboratory and
histological data was collected from patients aged 16 or below diagnosed with morphoea between 1 January 2007 to
31 December 2016.
Results:
Results from 37 patients revealed a male: female ratio of 1:3.6. Plaque (n=18,48.6%) subtype was most common
followed by linear (n=11,29.8%), generalized (n=6,16.2%) and mixed (n=2,5.4%). Lesions were found on the head
(n=6,16.2%), trunk (n=9,24.3%), extremities (n=12,32.4%) or were generalized (n=7,19.0%). Biopsy was done for 29
patients and 25 (86.2%) had results consistent with morphoea. The mean time from disease onset to diagnosis was
15.6 months (0-7 years). Most patients (n=31, 83.8%) were treated with only topical medications and 6 (13.5%) patients
received topicals and phototherapy or systemic immunosuppressants. The mean duration of follow-up was 2.4 years
(2.5 months to 7 years). Treatment type and response between plaque and linear morphoea revealed no significant
difference.
Conclusion:
Plaque morphoea was most common and more likely to be biopsied than linear morphoea in our study. This is an
interesting observation, as linear morphoea is known to occur more commonly in childhood. Therefore, these results
should ideally be corroborated in a larger Asian paediatric population.
Poster: CTD-10001
CHRONIC IDIOPATHIC URTICARIA IN PAEDIATRIC PATIENTS

BELOW 12 YEARS OF AGE TREATED WITH OMALIZUMAB:
A CASE SERIES
Nopriya 1, Citra Tresna Mur 2
Faculty of Medicine Sriwijaya University/Dr. Mohammad Hoesin General Hospital Palembang, Indonesia
Email: nopriya husan@gmail.com
INTRODUCTION
• The prevalence of chronic ur caria is about 0.1-0.3% in children and most o en occurs between ages of 6-11 years.1
• Known e ologies of chronic ur caria in children vary from 21% to 83%. The rest is idiopathic.1
• Omalizumab has been used in adults and adolescents pa ents (12 years of age and above) with H1-an histamine-
refractory chronic idiopathic ur caria (CIU).2
• Safety and efficacy of Omalizumab in pediatric pa ents with CIU below 12 years of age have not been established.2
CASE 1 CASE 2 CASE 3
Before A er Before A er Before A er

treatment treatment treatment treatment treatment treatment
The 1st pa ent had a history of acute The 2nd pa ent had a history of acute The 3rd pa ent had a history of chronic
ur caria and angioedema induced by ur caria treated with dexamethasone ur caria and bronchial asthma.
food allergy. injec on.
TREATMENT
• Omalizumab 75 mg 3 mes (every 4 weeks)
DISCUSSION
• Omalizumab is a recombinant humanized monoclonal an body that reduces levels of circula ng immunoglobulin E (IgE)
and expression of IgE high-afinity receptors on mast cells and basophils, interrup ng the subsequent allergic inflammatory
cascade.1
• Current indica ons for treatment with Omalizumab in paediatric pa ents are clearly defined and are confined to
moderate-to-severe uncontrolled allergic asthma and CIU.1
• In one study, 8-year old boy with refractory CIU with associated angioedema who had no response to cyclosporine and
frequent steroid therapy, showed complete response to Omalizumab treatment and was symptom free.2
• In our case, 3 children with a history of chronic ur caria received an histamines as the 1st line therapy, and tapered up to
4 folds as the 2nd line therapy, but the result evaluated by coun ng the weekly UAS was not really sa sfying. All pa ents
injected with Omalizumab and showed a good clinical response, with weekly UAS decreased to 0. The adverse effect
founded was only ascariasis in the 1st pa ent.
CONCLUSION
The evidence of efficacy and safety of Omalizumab therapy for CIU in paediatric pa ents below 12 years of age is s ll limited.
But there were several case reports showed complete response to Omalizumab treatment and was symptom free with no
serious adverse effect. Omalizumab is a new promising therapeu c modality. However, further study of efficacy and safety
for children below 12 years of age s ll needed.
REFFERENCES
1. Licari A, Marseglia A, Caimmi S, Castagnoli R, Foiadelli T, Barberi S, et al. Omalizumab in children. Pediatr Drugs. 2014;16:491-502
2. Ghaffari J, Shahmohammadi S, Ashrafi H, Ranjbar AR, Ghaffari N. Omalizumab (Xolair) in children above 12 years with chronic ur caria: a review of
literature. J Pediatr Rev. 2015;3(1):e152
Contact Author: Nesa Wike Wilanti
Country: Indonesia
Java Indonesia
Poster Veiw Here
BULLOUS SYSTEMIC LUPUS ERYTHEMATOSUS WITH SYSTEMIC CORTICOSTEROID
Abstract
Background: Bullous Systemic Lupus Erythematosus (BSLE) is an autoantibody-mediated disease characterised by

sub-epidermal blisters, a rare clinical subtype of Systemic Lupus Erythematosus (SLE). This condition predominantly
occurs in women in the second to fourth decade of life, while very rare in children, affecting only 1% of total pediatric
patients with SLE in the past 30 years. Dapsone is the primary choice of therapy, followed by corticosteroid and
immunosuppressant as the alternative therapies.
Case Presentation: An 11-year-old girl complained of clear fluid-filled blisters on her arms and legs since four days
before admission. No itching and no history of previous blisters. The patient also complained of joint pain, fever,
weakness, and decreased of appetite. The patient has been diagnosed with SLE by Pediatric Department. General
examination results was within normal limits, body weight was 27 kgs and body height was 135 cms. Dermatological
examination of the left arm revealed multiple vesicles and tense bullae with erythematous base filled with clear fluid,
well-defined borders, irregular shapes, and vary in size. The Nikolsky sign and Asboe-Hansen sign were negative.
Dermatological examination of the right arm and both legs revealed multiple vesicles with erythematous base filled with
clear fluid, well-defined borders, irregular shapes, and variable in size. Laboratory examination showed anemia,
lymphocytopenia, and positive Anti-Nuclear Antibodies (ANA) titers. Histopathology features showed vacuolar
degeneration of basal cells, dermoepidermal cleavage, and perivascular infiltration of lymphocytes and neutrophils in
papilla dermis. Patient was treated with intravenous methylprednisolone 30 mg/day for 2 weeks before being tapered,
chloroquine 150 mg/day, gentamicin cream and wet dressing. Significant clinical improvement appeared within one
week.
Discussion: In the treatment of BSLE, dapsone is the effective basic therapy. Hydroxychloroquine, though commonly
used to control the cutaneous lesions and disease activity of SLE, does not effective for BSLE eruptions. Corticosteroid
is one of the alternative drug for patients who have contraindication to dapsone. Corticosteroid gives slow progress for
BSLE, but can control the other systemic manifestations. In this patient, systemic corticosteroid therapy, 1-2 mg/kg/day
for one week gave a rapid positive response.
Keywords: bullous systemic lupus erythematosus, systemic corticosteroid, children.

Poster: CTD-10045
BULLOUS SYSTEMIC LUPUS ERYTHEMATOSUS

WITH SYSTEMIC CORTICOSTEROID
Nesa Wike Wilanti*, Lita Setyowatie*, Diah Prabawati Retnani**
*Department of Dermatology and Venereology
**Department of Pathology Anatomy
Faculty of Medicine, Universitas Brawijaya/ dr. Saiful Anwar Regional General Hospital, Malang, Indonesia
BACKGROUND
Bullous Systemic Lupus Erythematosus (BSLE) is an autoantibody-mediated disease characterised by sub-epidermal blisters,
a rare clinical subtype of SLE.1 This condition predominantly occurs in women in the second to fourth decade of life, while
very rare in children, affecting only 1% of total pediatric patients with SLE in the past 30 years.2 Corticosteroid can also be an
alternative therapy for dapsone, which is the first line choice in treating BSLE.3
CASE
An 11-year-old girl complained of clear fluid-filled blisters on her arms and legs since four days before admission. No itching
and no history of previous blisters. The patient also complained of joint pain, fever, weakness, and decreased of appetite.
The patient has been diagnosed with SLE by Pediatric Department. General examination results was within normal limits,
body weight was 27 kg and body height was 135 cm. Dermatological examination facial region showed erythematous
patches, ill-defined borders, irregular shapes, vary in sizes on both cheeks. Left arm revealed multiple vesicles and tense
bullae with erythematous base filled with clear fluid, well-defined borders, irregular shapes, and vary in size. Right arm and
both legs revealed multiple vesicles with erythematous base filled with clear fluid, well-defined borders, irregular shapes,
and vary in size. The Nikolsky sign and Asboe Hansen sign were negative. Histopathological examination supported BSLE
feature. Laboratory examination showed anemia,lymphocytopenia and positive ANA titers. Patient was treated with
intravenous methylprednisolone 30 mg/day for 2 weeks before being tappered, chloroquine 150 mg/day, gentamicin cream
and wet dressing.
Dermatological Examination Histopathological Examination
Figure 1: (A) Facial region showed erythematous patches, (B) Multiple

vesicles and tense bullae on the left arm, (C) Multiple vesicles on the right
arm, (D) Multiple vesicles on the both legs.
Follow Up
FOLLOW UP
Figure 2: (A) Dermo-epidermal junction splitting ( ) &

vacuolar degeneration (H&E staining x200) (B) Dermis
contained lymphocyte ( ) & neutrophils ( ) (H&E
Figure 3: Follow-up after 2 weeks showed hyperpigmentation patches staining x400)
DISCUSSION
In the treatment of BSLE, dapsone is the effective basic therapy. Hydroxychloroquine, though commonly used to control the
cutaneous lesions and disease activity of SLE, does not effective for BSLE eruptions.3,4 Corticosteroid is one of the alternative
drug for patients who have contraindication to dapsone. Corticosteroid gives slow progress for BSLE, but can control the
other systemic manifestations.5 In this patient, systemic corticosteroid therapy, 1-2 mg/kg/day for one week gave a rapid
positive response.
REFERENCES
1. Miziara ID, Mahmoud A, Chagury AA, Alves RD. Bullous Systemic Lupus Erythematosus: Case Report. Int Arch Otorhinolaryngol. 2013;17(3):344-6.
2. Lourenço D, Gomes R, Aikawa NE, Campos L, Romiti R, Silva C. Childhood-Onset Bullous Systemic Lupus Erythematosus. Pediatr Rheumatol Online J.
2014;12(Suppl1):335
3. Duan L, Chen L, Zhong S, Wang Y, Huang Y, He Y, et al. Treatment of Bullous Systemic Lupus Erythematosus. J Immunol Res. 2015;1-6.
4. Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PT, et al. Derivation and validation of the Systemic Lupus International Collaborating
Clinics Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheum. 2012;64(8):2677-86.
5. Yogarajah M, Sivasambu B, Jaffe EA. Bullous Systemic Lupus Erythematosus Associated with Esophagitis Dissecans Superficialis. Case Rep
Rheumatol. 2015;2015:1-4.

Contact Author: Dhany Prafita Ekasari
Country: Indonesia
Dr. Saiful Anwar Regional General Hospital,
Malang, East Java Indonesia
Poster: View Here
En Coup De Sabre
Background: Scleroderma can be diffuse (systemic sclerosis) or localized (localized scleroderma). Linear scleroderma
is a variant of localized scleroderma which is characterized by thickening grooves and linear induration. When it appears
on the forehead and front of the scalp, it is known as en coup de sabre. It is a rare craniofacial subtype of localized
scleroderma, commonly found in children and adolescents. Its clinical presentation is commonly accompanied by
seizure, muscular dysfunction or dental abnormality. This report presents a case of en coup de sabre affecting only the
skin and subcutaneous tissue without any accompanying disorders.
Case Presentation : A 5-year-old boy presented with brown patches on his left forehead since 9 months earlier, began
as a small brown patch, then slowly became larger and extended to the front of the scalp and upper nose. For the last
5 months, the patch felt stiff and dented. Dermatological examination revealed hyper and hypopigmented patches on
the forehead, scalp, nose, and left cheek, accompanied by linear atrophy on the forehead paramedian following the
Blaschko's lines. There was alopecia on the frontal scalp on hyperpigmented patch area. In palpation, the lesion was
firm and stiff. Investigations of serum autoantibodies obtained antinuclear antibody (+) and rheumatoid factor (-). There
were no abnormalities on skull X-ray and laboratory (complete blood count, liver function, kidney function) examination.
Histopathologically there was fibrous connective tissue on dermis and subcutaneous fat, supporting the diagnosis of
morphea.
Discussion : En coup de sabre is a rare connective tissue disorder, identified by its location on the scalp and
frontoparietal forehead and usually a self-limiting disease in children. It may soften or regress, but complete resolution
is rare and re-activation is always possible. In most cases, localized scleroderma becomes spontaneously inactivated
and in more severe cases can cause irreversible fibrosis/sclerosis of the skin and subcutaneous tissue. There is no
specific therapy for the disease. The goal of therapy is to reduce disease progression, preventing functional
complications and cosmetics purposes. Therapeutic options should be made with consideration of risks and benefits of
therapeutic options.
Keyword : en coup de sabre, linear scleroderma

Poster: CTD-10054
Contact Author: Sahana Srinivas
Country: India
Indira Gandhi institute of Child Health, Bangalore, Karnataka,
India
Poster: View Here
Zebra Stripes Like Eruptions In Dermatomyositis
Background
Zebra-like striped erythema also described as “flagellate erythema” or centripetal flagellate erythema is a rare and
distinct type of cutaneous feature seen in dermatomyositis. It is seen in 5% of patients with dermatomyositis and
represents active disease state. Literature review has shown only one case of juvenile dermatomyositis reported till
date.
Case Presentation
An 8-year-old girl child presented with asymptomatic skin lesions on the face, neck, trunk and extremities from past 1
month. There was no history of fever, oral ulcers, arthritis, photosensitivity or any topical application. However, there
was history suggestive of proximal muscle weakness. Cutaneous examination revealed erythematous to
hyperpigmented linear flagellate macules on face, neck, trunk and upper limbs. Multiple erythematous to violaceous
papules were present on the metacarpal-phalangeal joints (Gottron’s papules). There was no dermatographism. Oral
mucosa, nails, palms and soles were normal. Proximal muscle strength was grossly reduced. Other systemic
examinations were normal. Laboratory investigations showed antinuclear antibodies weakly positive in a speckled
pattern. Anti Mi-2, anti Jo-1 antibodies were strongly positive. Creatinine phosphokinase and lactic dehydrogenase were
increased. Other autoantibodies like anti DsDNA, SCL70, anti Ro, and anti nuclear cytoplasmic antibodies were normal.
Rheumatoid factor, C-reactive protein, thyroid function tests, renal function tests and serum electrolytes were normal.
Erythrocyte sedimentation rate, hepatic transaminase levels were raised. Other routine blood counts and ultrasound
abdomen were normal. Based on the clinical and laboratory findings diagnosis of dermatomyositis was considered. Skin
and muscle biopsy findings supported the diagnosis.
Discussion
Zebra stripe like erythema may be the initial presentation in dermatomyositis. The pathogenesis of flagellate erythema
is not unknown. Probable causes include minor trauma, physical injury and sun exposure. Classical features of
dermatomyositis like heliotrope erythema and Gottron’s papules have been described in dermatomyositis associated
flagellate erythema. Interstitial lung disease is seen in 20% cases and concurrent malignancy was seen in 40% cases.
Bleomycin induced linear streaks are more pigmented as compared with flagellate erythema associated
dermatomyositis which is more red indicating strong inflammation. Flagellate erythema responds well to treatment.
Our case is presented for its rare cutaneous asymptomatic eruption along with oligo-systemic juvenile
dermatomyositis.
Poster: CTD-10067
ZEBRA STRIPES LIKE ERUPTIONS IN DERMATOMYOSITIS

Sandipan Dhar, Sahana M Srinivas
Department of Pediatric dermatology, Institute of Child Health, Kolkata, West Bengal, India, Department of
Pediatric dermatology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
INTRODUCTION HISTOPATHOLOGY
Zebra-like striped erythema also described as ‘flagellate
Moderate vacuolar
erythema’ or centripetal flagellate erythema is a rare and distinct
type of cutaneous feature seen in dermatomyositis (DM)
degeneration of basal
keratinocytes with
It is seen in 5% of patients with DM and represents active perivascular lymphocytic
disease state. Literature review has shown only one case of infiltrate in the dermis.
juvenile DM reported till date. Pigmented melanophages
were present in the upper
We present a 8-year-old girl with zebra stripes like skin dermis (H & E x 40)
eruption diagnosed with DM
CASE HISTORY LABORATORY INVESTIGATIONS
An-8-year-old girl presented with asymptomatic skin lesions on ANA –weakly positive (speckled pattern)
face, neck, trunk and extremities from past 1 month. Two months
prior she had developed few asymptomatic skin rashes on both Anti Mi-2, anti Jo-1 antibodies – strongly positive
hands
Creatinine phosphokinase , Lactic dehydrogenase – increased
No history of fever, oral ulcers, arthritis , photosensitivity or any
topical application ESR, Hepatic transaminase levels – increased
History suggestive of proximal muscle weakness Other antibodies/RA factor/CRP/thyroid function tests, serum
electrolytes - normal
EXAMINATION
Physical examination - Proximal muscle strength grossly Ultrasound abdomen/chest X ray – normal
reduced
Muscle biopsy and electromyogram supported the diagnosis
Cutaneous examination
Erythematous to hyperpigmented linear flagellate macules or
DISCUSSION
‘zebra stripes like macules’ on face, neck, trunk and upper limbs Cutaneous lesions of juvenile DM are mainly inflammatory and
[fig 1,2 &3]. telangiectatic eruptions. Characteristic features include heliotrope
Gottrons papules present on metacarpal-phalangeal joints rash, nail fold capillary changes, vasculitis and calcinosis cutis
No dermatographism
Rarely linear streaks called ‘Zebra stripes like erythema’ or
Oral mucosa, nails, palms and soles – normal flagellate erythema can be the initial presentation in DM
Systemic examination normal
Exact pathogenesis of flagellate erythema is not known. Probable
mechanical stimuli that includes minor trauma, physical injury, sun
exposure may play a role
Flagellate erythema is associated with amyopathic DM (20%),

interstitial lung disease (20%) and malignancy (40%)
Flagellate erythema has been associated with chemotherapeutic

agents like bleomycin, adult-onset stills disease, toxicoderma, child
abuse and chikungunya fever. Flagellate erythema in DM is more
red indicating inflammation
Fig 1
CONCLUSIONS
Any child with extensive flagellate erythema should be
investigated thoroughly to know the cause and its association.
Flagellate erythema of DM responds well to treatment.
Our case is presented for its rare cutaneous asymptomatic

Fig 3 eruption along with oligo-systemic juvenile DM
References
Fig 1, 2 & 3 showing multiple
1. Eichenfield DZ, Paravar T. Zebra stripes in dermatomyositis: case
erythematous to hyperpigmented
report and review of flagellate erythema- associated dermatomyositis. J
flagellate macules on face, trunk
Eur Acad Dermatol Venereol 2017;31:e7-e9.
and upper limbs
Fig 2 2. Dupre A, Viraben R, Banafe JL, Touran P, Lamon P. Zebra-like
dermatomyositis. Arch Dermatol 1981;117:4.
TEMPLATE D ESIGN © 2008
www.PosterPresentations.com
Abstract ID CTD-10110
Track Connective Tissue Disorders
Contact author Ari Karmila Sari
Country Indonesia
Resident of Dermatology and Venereology Universitas
Organisation
Sumatera Utara
Poster: View Here
Hyper IgE Syndrome In Neonatal Lupus Erythematous: Is It A Complication Or A Comorbid?
Background: Lupus erythematosus (LE) is an autoimmune disease affecting the connective and vascular tissues.
Neonatal lupus erythematosus (NLE) is a common manifestation of lupus erythematosus in childhood period. The
disease is caused by transplacental transmission of maternal autoantibodies, especially Ro/SSA or La/SSB. The
pathogenic underlying mechanism of LE has been poorly understood. The disease rate of LE is 1 out of 12,500 to
20,000 births. Hyper IgE syndrome is characterized by a high IgE blood count at > 2000 IU. Erythematous rashes may
also present during birth or the subsequent weeks after.
Case report: A 13 month-old male baby came with erythematous rashes, scabs, and soft-flaky scales on his facial,
hands, and genital areas since one year ago. The rashes manifested first on his left cheek when he was 10 days old.
The lesion on the facial, neck, buttocks, and feet areas had been preceded by wet abrasive lesions that eventually
spread out and became erythematous rashes. The lesion worsened and became painful after sunbathing. Underweight
was observed during the physical examination. Erythematous plaque on the neck, right and left scapular areas, and
genital areas, also erythematous plaque with diffuse soft squames on the gluteal areas and right and left femoral areas
were observed during the dermatological examination. Laboratory findings include elevations of the thrombocyte, RDW
(red blood cells distribution width), dsDNA, and IgE levels. The patient was diagnosed with neonatal lupus
erythematosus with hyper IgE syndrome. The patient was treated with cetirizine, moisturizer and hydrocortisone cream.
Discussion : Clinical manifestation of NLE and hyper IgE may be present during childhood. Hyper IgE is not a
complication of LE. High IgE levels may aggravate the disease and thus affecting the disease severity. The initial
diagnosis of the patient proved to be challenging considering both SLE and hyper IgE syndrome manifested as an
eczematous lesion in early stages. Though elevation of IgE levels may be observed in NLE, the elevation is usually not
as significant as the hyper IgE syndrome.
Keywords : Neonatal Lupus Erythematous, Hyper IgE Syndrome

Poster: CTD-10110
Abstract ID RSMPD24
Track Connective Tissue Disorders
Contact author Hazel Lizette Panlilio
Country Philippines
Organisation East Avenue Medical Center
Poster View here
I SAW THE SIGNS: A CASE OF A 15 YEAR OLD FEMALE WITH DERMATOMYOSITIS
This is a case of AF, a 15-year-old, female, who consulted due to a 10 year history of erythematous patches over her
upper eyelids and erythematous papules over her hand joints which worsen upon sun exposure. She was assessed by
a pediatric rheumatologist as a case of juvenile dermatomyositis and was on Prednisone and Hydroxychloroquine for 4
years which caused resolution of lesions. However, in 2015, there was reappearance of her previous lesions and now
involving the shoulder, scalp, nape and chest which still worsen upon sun exposure. Histopathology result revealed
Interface Dermatitis suggestive of a connective tissue disease while Direct Immunofluorescence revealed consistent
with Dermatomyositis or Lupus Erythematosus. Normal Lactate dehydrogenase, Creatinine Kinase MM, Anti-nuclear
antibody, C3 and Anti-dsDNA ruled out lupus erythematosus. The patient was given Mometasone cream applied once
daily over face and scalp, Clobetasol + Vaseline Blanca applied twice daily over other affected parts of the body,
Sunblock SPF 30 and above applied 30 minutes prior sun exposure and mild lotion applied liberally. She was referred
to Ophthalmology for baseline eye screening and to Rheumatology for co-management. Rheumatology service started
the patient on Hydroxychloroquine 200mg/tab 1 tablet once daily, Calcium + Vitamin D tablet 1 tablet once daily and
Prednisone 22.5mg/day tapered by 2.5mg every 2 weeks for 2 months with marked improvement after. Dermatomyositis
is an idiopathic inflammatory myopathy characterized by an inflammatory infiltrate affecting the skeletal muscle and
skin. It occurs in children 2–17 years of age with girls more affected. In managing dermatomyositis, photoprotection is
important as sunlight can exacerbate skin lesions. Emollients can be given to control pruritus and burning. Topical
steroids are given to control cutaneous inflammation and pruritus. Topical calcineurin inhibitors, which act as steroid-
sparing agents, may be given for cutaneous inflammation. Oral corticosteroids remain the mainstay of treatment.
Antimalarial may be given for cutaneous inflammation and as steroid-sparing agents. Juvenile Dermatomyositis has
favourable functional outcomes with patients having a good health-related quality of life. The therapeutic goal’s to
provide patient relief using topical and/or systemic medications.
Poster: RSMPD24
I SAW THE SIGNS

A case of a 15 year old female with Dermatomyositis
Hazel Lizette M. Panlilio, MD; Alexis Paula D. Ibanez, MD;
Deejay B. Arcega, MD, FPDS; Elizabeth P. Prieto, MD, FPDS
Department of Dermatology, East Avenue Medical Center, Quezon City, Philippines
INTRODUCTION
Dermatomyositis is an idiopathic inflammatory
myopathy affecting the skeletal muscle and skin. Its
incidence is 3.2 cases per million children per year. It occurs
in children 2 to 17 years of age with an average age of
1, 2, 3
onset at 7 years old. There are several medications
Figure 4. Histopathology shows flattening of the epidermis with areas of epidermal
which may be given to control cutaneous inflammation, atrophy and thickened basement membrane and basal layer vacuolization. There is
prominent dermal edema with mucin deposits in the papillary and reticular dermis,
pruritus and myositis as well to prevent calcinosis cutis and telangiectasia, and a sparse superficial perivascular and slightly interstitial
lung disease which are complications of dermatomyositis. lymphocytic infiltrate.
CASE SUMMARY
We report a case of a 15 year old, Filipino, female, who
presented with a 10 year history of initially erythematous
patches over her upper eyelids and erythematous papules
over her hand joints which worsen upon sun exposure. Figure 5. IgG, IgM and C3 intracellularly in the epidermis
Previously assessed as Juvenile Dermatomyositis by a

Normal lactate dehydrogenase, creatinine kinase MM,
pediatric rheumatologist, she was on oral medications for 4 and C3 levels and negative ANA and anti-dsDNA ruled out
years which caused resolution of lesions. However, in 2015, lupus erythematosus.
there was reappearance of her previous lesions on the face
and now involving the shoulder, scalp, nape and chest. CONCLUSION
There were areas of hypo-hyperpigmentation over the Juvenile Dermatomyositis has favorable functional
arms, chest and back. Thickening of previously flattened outcomes with patients having a good health-related
erythematous papules over her hand joints were also quality of life. Patients usually start with behavioral
noted. The lesions were still exacerbated by sun exposure. modification by photoprotection and topical therapy such
as steroids and calcineurin inhibitors. However, these
usually serve as an adjunct as most patients will require
some form of systemic medication such as oral
corticosteroids and anti-malarials to control their skin
4, 5
disease and prevent complications.
REFERENCES
Figure 1. A-D. Multiple, ill to well-defined, irregularly-shaped, pink to 1. Goldsmith L, Fitzpatrick T. Fitzpatrick's dermatology in general medicine.
erythematous patches and plaques over the face, shoulder, scalp, nape and
New York: McGraw-Hill Medical; 2012.
chest. A. Few, ill-defined, irregularly-shaped, hyperpigmented patches over
2. Robinson A, Reed A. Clinical features, pathogenesis and treatment of juvenile
both upper eyelids. B-D. Several, well-defined, irregularly-shaped, hypo-
hyperpigmented patches over the arms, chest and back. and adult dermatomyositis. Nature Reviews Rheumatology. 2011;7(11):664-
675.
3. Iaccarino L, Ghirardello A, Bettio S, Zen M, Gatto M, Punzi L et al. The clinical
features, diagnosis and classification of dermatomyositis. Journal of
Autoimmunity. 2014;48-49:122-127.
4. Femia A, Vleugels R, Callen J. Cutaneous Dermatomyositis: An Updated
Review of Treatment Options and Internal Associations. American Journal of
Clinical Dermatology. 2013;14(4):291-313.
Figure 2. Several, well-defined, Figure 3. Solitary, well-defined, round to 5. Lam C, Vleugels R. Management of cutaneous dermatomyositis.
round to irregularly-shaped, pink irregularly-shaped, skin-colored to Dermatologic Therapy. 2012;25(2):112-134.
papules over the joints of both yellowish nodule over the right medial side
hands. of the flexor wrist and right knee.
Abstract ID: DRX-10053
Track: Drug Reactions
Contact Author: Anggun PutriYuniaswan
Country: Indonesia

Java Indonesia
Poster: View Here
STEVENS JOHNSON SYNDROME FOLLOWING MEASLES And VARICELLA VACCINATION
Abstract
Background: Stevens Johnson Syndrome (SJS) majority caused by drugs. However,it also has been linked with other
etiologies such as viral infection and vaccination. The vaccine includes components like active immunizing antigens and
other conjugating agents. All of these components might contribute to a hypersensitivity reaction.
Case Presentation: 11 years old boy presented with generalized red rash and blisters. Erythematous rash appeared on
his face and progresively spread to the trunk, limbs, palms and soles within hours. Some lesions evolved to flaccid
blisters which ruptured easily. He also had painful ulceration on lips and oral cavity leading to difficulty in opening mouth
and swallowing. The patient had his Measles and Rubella vaccination 3 days preceeding the initial lession. He was
febrile several hours after the vaccination and to relieve it, the mother gave paracetamol syrup. Dermatological
examination showed well defined erythematous-purpuric patches. Flaccid blisters were observed on his face, neck and
Nikolsky sign was positive. He also had conjunctivitis and oral ulcers covered with hemorrhagic crusts. Vital signs were
stable and his SCORTEN was 2, including his increased heart rate and urea serum. Diagnosis of SJS was made with
the presumptive culprit thought to be paracetamol. However, measles-varicella vaccination as causative agent still
cannot be eliminated. Suspected drug was discontinued and methylprednisolone 25mg (2mg/KgBW/day) was given
intravenously, three times daily. During observation, denuded areas increased but otherwise his condition remained
stable with no complications. Skin and mucosal lesions gradually started to heal within two weeks.
Discussion: The patient developed SJS immediately after vaccination and ingestion of Paracetamol. Despite being
considered relatively safe, hypersensitivity reactions have been reported with Paracetamol. Similar to other drugs,
vaccines have potential to cause drug hypersensitivity. Type IV hypersensitivity reactions generally begin 48 hours after
vaccination and peak between 72- 96 hours. These reactions are typically observed following vaccines containing
thimerosal, aluminum and anti-microbial agents. Even after hypersensitivity reaction following vaccination, it is difficult
to ascertain whether the reaction was caused by the vaccine itself or other factors.
Keywords: stevens johnson syndrome, paracetamol, vaccination

Poster: DRX-10053
Abstract ID: DRX-10091
Track: Drug Reactions
Contact Author: Jusuf Nelva Karmila
Country: Indonesia
Organisation: Faculty of Medicine Universitas Sumatera Utara
Poster: View Here
Generalized Exfoliative Dermatitis In Children ; A Case Report
Background
Exfoliative dermatitis (ED) is defined as diffuse erythema and scaling of the skin involving more than 90% of the total
body skin surface area. Common underlying etiologies are psoriasis, atopic dermatitis, and other spongiotic dermatoses,
drug hypersensitivity reactions, and Cutaneous T-Cell Lymphoma (CTCL). The cause of ED is unknown (idiopathic) in
approximately 20% of cases. Management of ED involves combining symptomatic relief with addressing the underlying
etiology and potential systemic complications. Inpatient hospitalization is required in acute cases.
Case presentation
A 10 years-old girl was consulted with erythematous macules and scaling of the skin surface more than 90% of the total
body with itchy since 1 week ago. Patient have a history of fever which was present two days before the appearance of
the lesions. The skin lesions occured after the patient consumed oral cefadroxil for 2 days, which had been prescribed
by a midwife due to the fever. A history of drug allergy was negative. This was the first time the patient showed such
reaction. Dermatological examination showed erythematous macules with scaling more than 90% of the total body skin
surface. The diagnosis of patient was generalized exfoliative dermatitis based on history and clinical appearance. The
patient was informed to stop consumed the suspected drug. The patient was treated with metilprednisolone tablet,
cetirizine tablet and urea 20% cream. This patient showed improvement after two weeks of therapy.
Discussions
Exfoliative dermatitis may embrace or be caused by any pre-existing dermatosis, drug-induced reaction, malignancy
and miscellaneous or idiopathic disorder. Topical and systemic medications may precipitating exfoliative dermatitis it is
therefore pertinent to provide an inventory of drugs frequently responsible for causing such an episode. In this case may
be drug induced ED due to cefadroxil. Drug-induced cases of ED recover completely if initial medical management is
promptly undertaken, and has the best prognosis.
Poster DRX-10091
Abstract ID RSMPD12
Track Drug reactions
Contact author Rusyati Luh Made Mas
Country Indonesia
Organisation Udayana University/ Sanglah General Hospital
Poster: View here
DRUG INDUCED STEVENS-JOHNSON SYNDROME IN CHILDREN : A CASE SERIES
ABSTRACT
Background:
Stevens - Johnson syndrome (SJS) is an acute life-threatening hypersensitivity syndrome, rarely in children. Most
frequent offending agents are antibiotics, aromatic anti-convulsants and anti-retroviral drugs (ARV). Problems arise
when these drugs are required for long-term use and necessary for several health conditions. The aim of this case
series is to describe SJS in children and provide replacement therapy in patient with epilepsy or HIV infection.
Case I : A 19 month-old-boy, was admitted with fever, erythema on both conjunctivas, erosions on lips, erythematous
rashes all over his body. He had history of seizures and was prescribed Carbamazepine. After 3 months of medications,
symptoms of Steven-Johnson syndrome (SJS) appeared. Carbamazepine was replaced with diazepam. The patient
was treated with dexamethasone 1.7 mg every 8 hour intravenously, borax glycerine for lips after open dressing with
normal saline, xytrol 1 drops / 8 hours, polyvinyl pypolidone 1 drop every 4 hours on both eyes, and the patient recovered
within 7 days.
Case II : A 3 year-old-boy, came with chief complaint blisters and red macules all over his body. He was diagnosed with
HIV/ AIDS since he was 2 months old and received Nevirapine, Cotrimoxazole, Lamivudine, Zidovudine. On physical
examination, secretion was found on both eyes, blisters and erosions covered most parts of the body, and erosions
covered with haemorrhagic crust on lips. The treatment for this patient was to stop Nevirapine, the suspected causing
drugs. Nevirapine was replaced with Tenofovir, Emtricitabine, and Efavirenz. Methylprednisolone injection 31.75 mg
every 8 hours, triamcinolone actinide in orabase for lips after open dressing with normal saline, eye toilet, lyters 1 drop
every 4 hours, and xytrol 1 drop every 4 hours on both eyes, were administered.
Discussion : SJS is an uncommon, but life-threatening condition in children. It is a challenge to determine the causative
agent in treating SJS syndrome, when there are varieties of suspected drugs. The management of SJS was done by
excluding and replacing the suspected drugs, while providing anti-inflammatory agents.
Key words : Stevens-Johnson syndrome, children

Rusyati Luh Made Mas
Poster RSMPD12
DRUG INDUCED STEVENS-JOHNSON SYNDROME

IN CHILDREN : A CASE SERIES
Rusyati Luh Made Mas, Karmila IGAA Dwi
Sudarsa Prima Sanjiwani, Pramita Nyoman Yoga Maya
BACKGROUND
Stevens-Johnson Syndrome (SJS) is an acute life-threatening hypersensitivity syndrome, rarely in
children. Most frequent offending agents are antibiotics, aromatic anti-convulsants and anti-retroviral
drugs (ARV). Problems arise when these drugs are required for long-term use and necessary for several
health conditions. The aim of this case series is to describe SJS in children and provide replacement
therapy in patient with epilepsy or HIV infection.
CASE I : A 19 month-old-boy, was admitted INITIAL PRESENTATION

with fever, erythema on both conjuntiva,
erosions on lips, erythematous rashes all over his 7 DAYS AFTER THERAPY
body. He had history of seizures and was
prescribed Carbamazepin. After 3 months of
medications, symptoms of Steven-johnsons
syndrome (SJS) appeared. Carbamazepine was
replaced with diazepam. The patient was treated
with dexamethasone 1.7 mg every 8 hour
intravenously, borax gliserin for lips after open
dressing with normal saline, xytrol 1 drops / 8
hours, polivinyl pypolidone 1 drop every 4 hours
on both eyes, and the patient recovered within 7
days.
CASE II: : A 3 year-old-boy, came with chief complaint

INITIAL PRESENTATION blisters and red macules all over his body. Patient were
diagnosed with HIV/ AIDS since 2 month ago and received
Nevirapine, Cotrimoxazole, Lamivudine, Zidovudine. On
physical examination, secretion was found on both eyes,
blisters and erosions covered most parts of the body, and
erosions covered with haemorragic crust on lips. The
treatment for this patient was to stop Nevirapine, the
8 DAYS AFTER THERAPY suspected causing drugs. Nevirapine was replaced with
Tenofovir, Emtricitabine, Efavirenz. Metylprednisolone
injection 31.75 mg every 8 hours, triamcinolone acetonide in
ora base for lips after open dressing with normal saline, eye
toilet, lyters 1 drop every 4 hours, and xytrol 1 drop every 4
hours on both eyes, were administered.
DISCUSSION : SJS is an uncommon, but life-threatening condition in children. It is a challenge to

determine the causative agent in treating SJS syndrome, when there are varieties of suspected drugs. The
management of SJS was done by excluding and replacing the suspected drugs, while providing anti-
inflammatory agents .
References
1. Allanore LV, Rojeau JC. Epidermal Necrolysis (Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis). In: Goldsmith LA, Katz SI, Gilchrest BA,
Paller AS, Leffel DJ, Wolff K, eds. Fitzpatrick’s Dermatology In General Medicine. 8th ed. USA: McGraw-Hill 2012. p.439-448
2. Kasprzyk JC, Krzeminska AP, Zabolska IO. Toxic Epidermal Necrolysis. Anaesthesiology Intensive Therapy. 2015;47(3): 257-262.
Abstract ID: SSD-10034
Track: Skin Manifestations of Other Systemic Disorders
Contact Author: Hua-Liang Joel Lim
Country: Singapore
Organisation: National Skin Centre
Poster View Here
An Instructive Case of Granulomatous Chelitis
CASE PRESENTATION
A healthy 15 year old Indian male presented with a 6-month history of asymptomatic swelling of his upper lip with
progressive involvement of the lower lip. Systemic review revealed intermittent haematochezia starting 2 months prior
and remote bouts of self-limiting oral ulcers. Family history was non-contributory. Examination revealed a homogenously
rubbery induration of upper and lower labia without any facial dermatoses, lingual plication or facial paralysis. Biopsy of
the cutaneous lip revealed tuberculoid granulomas. Subsequent evaluation by a gastroenterologist revealed iron-
deficiency anaemia along with elevated erythrocyte sedimentation rate and stool calprotectin levels. A colonoscopy
demonstrated terminal ileitis with patchy colonic erythema. Endoscopic intestinal biopsies revealed active chronic
inflammatory change with focal non-caseating granulomas and crypt architectural effacement. A diagnosis of
granulomatous chelitis (GC) secondary to Crohn’s disease (CD) was made.
DISCUSSION
GC belongs to the spectrum of orofacial granulomatosis and is regarded as a monomorphic presentation of

Melkerson-Rosenthal syndrome. Clinically, GC presents abruptly as swelling, with a predilection to involve the upper
lip before affecting the lower lip. Painful labial fissures may occur which subsequently heal and relapse, culminating in
the lip taking on a rubbery or nodular consistency. Early recognition and treatment is vital to prevent irreversible
textural change and persistence of labial swelling. It is imperative to recognise the secondary aetiologies that present
with GC. Systemic diseases include CD, sarcoidosis, granulomatous polyangiitis and lymphoma. Infections such as
leprosy and histoplasmosis may present with GC. Exogenous causes such as filler injections and exposure to gold,
cinnamates or benzoates can cause foreign body granulomatous reaction and contact dermatitis, respectively.
Studies have shown linkages with HLA-A2/A11 in primary GC. Therapy in secondary GC involves treatment of the
underlying aetiology. First-line treatment for GC involves moderate potency topical corticosteroids or intra-lesional
steroids. Outcomes for cases that fail this treatment remain unsatisfactory. Alternative options including tetracyclines,
non-steroidal anti-inflammatory drugs, ketotifen and clofazamine. Surgical debulking may be the best option in such a
setting. The patient reported improvement in lip swelling with topical mometasone furoate 0.1% ointment. He was also
treated with systemic prednisolone and oral mesalazine with control of his CD.
Poster: SSD-10034
Contact Author: Elis Lee
Country: Singapore
Organisation: KK Women's and Children's Hospital
Poster View Here
A Rare Case Of Circinate Balanitis In A Child With Reiter’s Syndrome
Background:
Reiter’s syndrome, also known as reactive arthritis, is characterized by the classical triad of conjunctivitis, arthritis, and
urethritis. Circinate balanitis is a known mucocutaneous manifestation, but is less commonly reported in children.
Case presentation:
A nine-year-old boy presented with two weeks of fever, oligoarthritis involving the right elbow, left knee and left elbow,
bilateral conjunctivitis, and a non-pruritic rash on his glans penis for 3 days. Investigations also showed sterile pyuria,
suggestive of urethritis. He has a history of typhoid fever, which was treated a few years ago and resolved. On
examination, there were psoriasiform, annular plaques on the glans penis. A diagnosis of Reiter’s syndrome with
circinate balanitis was made in view of his clinical and investigation findings.
Discussion:
Reiter’s syndrome is commonly associated with either a preceding gastrointestinal or genitourinary infection. The former
is more common in children. HLA B27 haplotype is believed to be a contributing factor as well. The full triad does not
occur frequently in childhood, and circinate balanitis is rarely reported in the paediatric population. Other differential
diagnoses to be considered include infective causes, psoriasis and fixed drug eruptions involving the penis. The clinical
context and relevant investigation findings are critical in diagnosis. Various treatment modalities have been used for
mucosal lesions in adults with reactive arthritis, including topical steroids, tacrolimus and keratolytic agents. However,
these treatments are not well studied in children. Further studies should be done to evaluate the prevalence and
treatment of circinate balanitis and other mucocutaneous manifestations in juvenile Reiter’s syndrome.
Poster: SSD-10083
A Rare Case of Circinate Balani s in a Child with

Reiter’s Syndrome
Lee E 1 , Koh M 2,, Foong A 2 , Teh KL 1 , Thaschawee A 3
1 Department of Paediatric Medicine, KK Women's and Children's Hospital, Singapore
2 Department of Dermatology, KK Women's and Children's Hospital, Singapore
3 Department of Paediatric Subspecial es, Rheumatology and Immunology Service, KK Women’s and Children’s Hospital
He was diagnosed with Reiter’s syndrome in view of the combina on

Introduc on of clinical features of circinate balani s, arthri s, conjunc vi s and
Reiter’s syndrome, also known as reac ve arthri s, is characterized sterile urethri s. Extensive biochemical, microbiological and imaging
by the classical triad of conjunc vi s, arthri s, and urethri s. studies returned nega ve. Notably, there was no evidence of
Circinate balani s is a known mucocutaneous manifesta on, but is venereal infec on – Chlamydia and gonorrhea PCR were nega ve,
less commonly reported in children. and syphilis and Human Immunodeficiency Virus (HIV) serologies
were nega ve. Penile swab culture and fungal scraping were
Clinical Case unremarkable. HLA-B27 was posi ve. Autoimmune markers, such as
A 9-year-old boy presented with two weeks of fever, oligoarthri s rheumatoid factor and an -nuclear an bodies (ANA), were nega ve.
involving the right elbow, le knee and le elbow, and bilateral The child was treated with oral indomethacin and prednisolone, and
conjunc vi s. He had a history of typhoid fever a few years ago, was empirically covered with systemic an bio cs (intravenous
which was treated in another country, and resolved. Inves ga ons cloxacillin and ce riaxone, then oral cephalexin). The penile rash was
showed sterile pyuria, with a white blood cell count of 25 cells per treated with betamethasone (valerate) 0.025% clioquinol 3% cream.
high power field (hpf) with nega ve urine culture, sugges ve of The rash resolved within a week, but there was persistent
urethri s. During the admission, he developed painless, oligoarthri s at 3-week review.
psoariasiform plaques with surrounding erythematous borders and
scaling on the glans penis (Figure 1). There was no associated urethral
Discussion
discharge. There were no other mucocutaneous lesions, and the Reiter’s syndrome can occur with mucocutaeous manifesta ons, such as
palms and soles were uninvolved. circinate balani s, ulcera ve vulvi s, keratoderma blennorrhagicum,
and other oral and nail lesions. Reiter’s syndrome is commonly
associated with either a preceding gastrointes nal or genitourinary
infec on. The former is more common in children. HLA B27 haplotype
is believed to be a contribu ng factor to disease development as well.
Reiter’s syndrome presen ng with circinate balani s is uncommon in

children. The full triad of conjunc vi s, arthri s and urethri s does not
occur frequently in childhood. Circinate balani s is a known
mucocutaneous manifesta on, especially in adults, but is rarely
reported in the paediatric popula on. Cases of boys with circinate
balani s have been described in two case series studies involving
children with reac ve arthri s in China. Otherwise, there is very limited
literature available.
Differen al diagnoses to be considered in a child with erythematous

penile plaques and superficial erosions include infec ve causes such as
venereal diseases (especially if there is sugges on of sexual contact)
and candidiasis, as well as non-infec ve dermatological causes like
psoriasis and atopic or contact derma s. A fixed drug erup on is
another important considera on, especially if there is a sugges ve
medica on history. Correla on of clinical findings and and relevant
inves ga on findings are cri cal in arriving at a diagnosis of Reiter’s
syndrome in children.
Various treatment modali es have been used for mucosal lesions in

adults with reac ve arthri s, including topical steroids, combina on
keratoly c agents and topical calcineurin inhibitors (e.g. tacrolimus).
However, these are not well studied in children. Further studies should
be done to evaluate the prevalence and treatment of circinate balani s
and other mucocutaneous manifesta ons in juvenile Reiter’s syndrome.
Contact Author: Diane TantiaSari
Country: Indonesia
Java Indonesia
Poster: View Here
MANAGEMENT OF ACRODERMATITIS ENTEROPATHICA WITH A NORMAL SERUM ZINC LEVEL
Abstract
Background: Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder due to zinc deficiency that occurs
in 1: 500,000 individuals. Typical clinical manifestations include alopecia, diarrhoea, periorificial and acral dermatitis.
Low serum zinc levels is amongst the diagnosis indicator, yet 30% reported cases presented with normal serum zinc
levels. Zinc supplementation as the primary treatment should commence following the establishment of AE diagnosis.
Case Presentation: A 21-month-old boy brought to Emergency Room with recurrent wounds and crusted lesions
throughout his body since three months postnatal. He had persistently low body mass, itchiness, fever, and recurrent
diarrhoea with mucus. General physical examination within normal limit, except bodyweight and height: 7 kilograms in
mass and 71 centimetres in height. Dermatological examination revealed total alopecia; multiple erythematous-based
erosions with sharply demarcated peripheral crusted borders on periorificial and acral regions. Gram stains of the
erosion revealed polymorphonuclear (PMN) cells and Gram-positive cocci. Culture grew positive for Staphylococcus
aureus and Staphylococcus haemolyticus. Stool analysis revealed fungal elements. Histopathologic examination was
non-specific for AE. Serum zinc was normal (128,75µg/dL). Due to marasmic condition, we involved Paediatric
Department in managing accordingly. We prescribed zinc supplements 120 mg/day, resomal, saline dressings,
mupirocin ointment, and 2% ketoconazole cream along with the management of marasmus. Clinical improvement was
achieved within 2 weeks.
Discussion: Diagnosis of AE is commonly established based on typical clinical manifestations confirmed by low serum
zinc levels <70 µg/dL. However, normal serum zinc levels shall not necessarily exclude AE diagnosis if a patient
presented with typical clinical manifestations. The rapid clinical response achieved following zinc supplementation, in
this case, confirmed the diagnosis of AE. Zinc supplementation whose doses range between 0.5-3 mg/kg/day, ought
not to be delayed despite normal serum zinc levels.
Keywords : acrodermatitis enteropathica, zinc deficiency, normal serum zinc level, zinc supplementation
Poster: SSD-10050
MANAGEMENT OF
ACRODERMATITIS ENTEROPATHICA
WITH A NORMAL SERUM ZINC LEVEL
Diane Tantia Sari*, Lita Setyowatie*, Tantari Sugiman*, Diah Prabawati Retnani**
*Department of Dermatology and Venereology
**Department of Pathology Anatomy
Faculty of Medicine, Universitas Brawijaya/ dr. Saiful Anwar Regional General Hospital, Malang, Indonesia
BACKGROUND
Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder due to zinc deficiency that occurs in 1:500,000
individuals.1,2 Typical clinical manifestations include alopecia, diarrhoea, periorificial and acral dermatitis.1 Low serum zinc
levels is amongst the diagnosis indicator, yet 30% reported cases presented with normal serum zinc levels.3,4 Zinc
supplementation as the primary treatment should commence following the establishment of AE diagnosis.4
CASE
A 21-month-old boy brought to Emergency Room with recurrent wounds and crusted lesions throughout his body since
three months postnatal. He had persistently low bodyweight, itchiness, fever, and recurrent mucus diarrhoea. General
examination within normal limit, except bodyweight 7 kg and height 71 cm (marasmic condition).
Pedigree Microscopic Examination Histopathological Examination
Gram stains from the perinasal (A), perioral (B), and

genital (C) erosion revealed polymorphonuclear A: Lichenoid reaction, neutrophil and lymphocytic
(PMN) cells and Gram-positive cocci (Gram, x1000) debris ( ). Epidermal pallor, acanthosis, spongiotic
( ). B: Dermal vasodilatation ( ). This was non-
specific for AE (H&E, x400)
Laboratory Examination
Serum zinc was normal (128,75 µg/dL). Culture of erosion grew positive for
S.aureus & S.haemolyticus. Stool analysis revealed fungal elements.
Dermatological Examination Treatment Follow Up
•Life-long zinc
supplementation
(20 mg/day)
•Saline dressings
•Mupirocin
ointment
•Management of
marasmus
Total alopecia; multiple erythematous-based erosions with
sharply demarcated peripheral crusted borders on periorificial
and acral regions Clinical improvement was achieved within 2 weeks
DISCUSSION
Normal serum zinc levels shall not necessarily exclude AE diagnosis if a patient presented with typical clinical
manifestations.3 The rapid clinical response achieved following zinc supplementation, in this case, confirmed the diagnosis
of AE.3 Zinc supplementation whose doses range between 0.5-3 mg/kgBW/day, ought not to be delayed despite normal
serum zinc levels.1,5
REFERENCES
1. Jen M, Yan AC. Cutaneous changes in nutritional disease. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell D, editors. Fitzpatrick's Dermatology in General Medicine, 8th Ed. New York:
McGraw-Hill, 2012; p.1499-525.
2. Geiser J, Venken KJ, De Lisle RC, Andrews GK. a Mouse model of acrodermatitis enteropathica: loss of intestine zinc transporter ZIP4 (SLC39A4) disrupts the stem cell niche and intestine integrity. PLoS
Genet. 2012;8(6):1-16.
3. Jung AG, Mathony UA, Behre B, Küry S, Schmitt S, Zouboulis CC, et al. Acrodermatitis enteropathica: an uncommon differential diagnosis in childhood–first description of a new sequence variant. J Dtsch
Dermatol Ges. 2011;9(12):999-1002.
4. Lee SY, Jung YJ, Oh TH, Choi EH. a Case of acrodermatitis enteropathica localized on the hands and feet with a normal serum zinc level. Ann Dermatol. 2011;23(Suppl1):S88-90.
5. Wessells KR, King JC, Brown KH. Development of a plasma zinc concentration cutoff to identify individuals with severe zinc deficiency based on results from adults undergoing experimental severe
dietary zinc restriction and individuals with acrodermatitis enteropathica. J Nutr. 2014;144(8):1204-10.
Contact Author: Anuradha Kakkanatt Babu
Country: India
Aster Medcity,Kochi ,Kerala
Poster: View Here
Munchausen Syndrome By Proxy - A Case Report
Introduction:
Munchausen syndrome by proxy (MSBP) is a form of child abuse in which a parent or caretaker simulates or produces
illness in a child. Case reports of Munchausen syndrome by proxy document a wide and confusing spectrum of
symptoms. The children who are the victims of this syndrome suffer morbidity linked to numerous unnecessary hospital
admissions and diagnostic procedures, severe psychological morbidity, and a significant risk of mortality. Because of
these extreme consequences, all health-care professionals must be able to recognize its warning signals.
Case Presentation:
A 5 year old girl was admitted in our hospital for evaluation of drowsiness of 1 month duration .On admission there were
a few fluid filled and crusted lesions on trunk & limbs .The parents claimed that these lesions started appearing 2 days
back. While admitted in the hospital, the number of lesions increased day by day.
On examination, the child was drowsy .Multiple hyperpigmented crusted plaques , some with vesicles were seen on
face , trunk & limbs .Most of the lesions showed a peculiarly similar morphology which could be matched to have been
made using a cigarette lighter. Blood sample of the child which was sent for toxicology screening showed significant
levels of alcohol. On further interrogation the parents admitted to have been intoxicating the child with alcohol to make
her drowsy and fabricated the skin lesions using a cigarette lighter.
A diagnosis of Munchausen syndrome by proxy was made.
Child welfare society was informed about this case who did the needful for the protection of the child. Parents were
referred to psychiatry department for expert management.
Discussion:
MSBP was named after Baron von Munchausen, an 18th century German dignitary known for making up stories about
his travels and experiences in order to get attention
"By proxy" indicates that a parent or other adult is fabricating or exaggerating symptoms in a child, not in himself or
herself
In a review of 451 victims of MSBP, majority of perpetrators were biological mothers (76.5%) or fathers (6.7%).
Munchausen syndrome by proxy is a mental illness and requires treatment
Poster: SSD-10084
MUNCHAUSEN SYNDROME BY PROXY – A CASE REPORT
Dr Anuradha Kakkanatt Babu,Consultant Dermatologist,Aster Medcity,Kochi,Kerala,India

dranuradhaprasad@yahoo.co.in
INTRODUCTION:
Munchausen syndrome by proxy (MSBP) is a form of child abuse in which a parent or caretaker simulates or produces
illness in a child. Case reports of MSBP document a wide and confusing spectrum of symptoms. The children who are
the victims of this syndrome suffer morbidity linked to numerous unnecessary hospital admissions and diagnostic
procedures, severe psychological morbidity, and a significant risk of mortality. Because of these extreme
consequences, all health-care professionals must be able to recognize its warning signals.
CASE PRESENTATION:
A 5 year old girl was admitted in our hospital for evaluation of drowsiness of 1 month duration .On admission there were
a few fluid filled and crusted lesions on trunk & limbs .The parents claimed that these lesions started appearing 2 days
back. While admitted in the hospital, the number of lesions increased day by day.
On examination, the child was drowsy .Multiple hyperpigmented crusted plaques ,some with vesicles were seen on
face,trunk & limbs (Figure 1).Most of the lesions showed a peculiarly similar morphology which could be matched to
have been made using a cigarette lighter(Figure 2). Blood sample of the child which was sent for toxicology screening
showed significant levels of alcohol. On further interrogation the parents admitted to have been intoxicating the child
with alcohol to make her drowsy and fabricated the skin lesions using a cigarette lighter.
A diagnosis of MSBP was made.
Child welfare society was informed about this case who did the needful for the protection of the child. Parents were
referred to psychiatry department for expert management.
DISCUSSION:
MSBP was named after baron Von Munchausen, an 18 th century German dignitary known for making up stories about
his travels and experiences in order to get attention.“By proxy" indicates that a parent or other adult is fabricating or
exaggerating symptoms in a child, not in himself or herself.In a review of 451 victims of MSBP, majority of perpetrators
were biological mothers (76.5%) or fathers (6.7%).MSBP is a mental illness and requires psychiatric care.
Figure 1 Figure 2
Contact Author: Githa Rahmayunita
Country: Indonesia
Dept of Dermatology Venereology, Fac of Medicine

of Universitas Indonesia, Cipto Mangunkusumo
Hosp
Poster: View Here
CUTANEOUS LANGERHANS CELL HISTIOCYTOSIS: CHALLENGE IN DIAGNOSIS
BACKGROUND
Langerhans cell histiocytosis (LCH) is a disorder characterized by infiltration of Langerhans cell into
various organs of the body. It is a rare disease, with incidence 4-10 per million population and peak in
children aged 1-3 years. In Pediatric Dermatology Division of Cipto Mangunkusumo Hospital, from
2012-2017 there were only 7 new cases of LCH. Usually the diagnosis is based on classic
manifestations such as erythematous to brownish, crusted and purpuric papules on seborrheic areas,
lymph nodes enlargement, hepatosplenomegaly, and icterus. LCH can simulate several disease such
as seborrheic dermatitis, scabies, varicella, etc. If those classic manifestations were not present, clinical
diagnosis of LCH can be challenging.
CASE PRESENTATION
A 9 month-old boy with greasy scaly erythematous papules and plaque on his scalp, forehead, axilla,
both lateral abdomen, inguinal, palmar and plantar since 6 months ago. First it appeared on his head.
There were no purpuric lesion, lymph nodes enlargement, hepatosplenomegaly or icterus. The patient
was diagnosed as seborrheic dermatitis with LCH as differential diagnosis. Skin scrapping for fungal
examination with 20% potassium hydroxide examination revealed negative results. Peripheral blood
test results was within normal limit. Histopathology examination result supported the diagnosis of LCH.
On the second visit, developed purpuric striae on his finger and toe nails. The patient was treated with
emollient and consulted to Child Health Department.
DISCUSSION
In this case, the classical manifestation of LCH was absent. The lesions were located on seborrheic
areas so that at first he was diagnosed as seborrheic dermatitis; however, lesions on lateral abdomen,
palms and soles raised the suspicion cutaneous LCH. It was later confirmed by histopathological
examination. LCH can affect several organs such as lymph nodes, liver, spleen, lung, bones, and other
organ. In this case, such diffuse organ involvement was not detected by screening laboratory tests.
Skin lesion is often the presenting sign and our case could be at an early stage of systemic LCH.
Keywords: Langerhans cell histiocytosis, cutaneous manifestation

Poster: SSD-10089
Skin Manifestations of Other Systemic
Track:
Disorders
Contact Author: Valerie Ho
Country: Singapore
Organization: KK Women's and Children's Hospital
Poster: View Here
Acquired Cutaneous Lymphangiectasia Of The Vulva Secondary To Tuberculosis Infection In A

Teenage Girl
Background: Lymphangiomas are benign malformations of the lymphatic system, which can occur on
the skin and mucous membranes, and can be categorized as superficial or deep, and congenital or
acquired. Acquired cutaneous lymphangioma or lymphangiectasia of the vulva is a rare condition and
results secondary to pelvic lymphatic obstruction. It has been associated with chronic abdominal-pelvic
inflammatory disease, infections and malignancies.
Case Presentation: A previously well 14-year old teenage girl was treated for miliary tuberculosis and
tuberculosis of the gastrointestinal tract, which was complicated by protein-losing enteropathy and ileal
perforation. She underwent resection of the terminal ileum and an ileostomy creation. Intraoperative
findings showed extensive peritoneal contamination with pus and serous fluid. Her post-operative
course was further complicated by enterococcus faecalis and candida tropicalis intra-abdominal sepsis
and disseminated intravascular coagulopathy. During her inpatient stay, she developed a crop of
transparent vesicles over the vulva. A punch biopsy performed showed increased numbers of dilated
thin-walled lymphatic channels that stained positively for D2-40 immunohistochemical stain. This was
consistent with lymphangiectasia. Over the course of her 3-month day, the lesions increased over the
vulva region, then spontaneously resolved as anti-tuberculosis medications were continued.
Discussion: Acquired cutaneous lymphangiectasia of the vulva is a rare but relatively benign cutaneous
condition affecting the dermal lymphatic channels. They arise because of pelvic lymphatic obstruction
and have been reported in adults after radiotherapy for cervical and vulva cancer, Crohnâ_™s disease,
tuberculosis of the gastrointestinal tract and filariasis. The lymphatics of the vulva drain into the
superficial and deep inguinal nodes, and any obstruction to the deep lymphatic vessels will lead to back-
flow and pressure, leading to dilatation of the dermal lymphatics. It can be asymptomatic, pruritic, or
even painful. Differential diagnoses include genital herpes and viral warts. The diagnosis can be
confirmed by histology. Subsequently, an evaluation for a secondary cause should be carried out. While
more commonly associated with malignancy in adults, chronic infections like tuberculosis of the gut and
chronic inflammatory bowel disease like Crohnâ_™s disease is more common in the Paediatric
population. Treatment of the underlying cause leads to complete resolution
Poster: SSD-10106
Acquired Cutaneous Lymphangiectasia of the Vulva

Secondary to Gut Tuberculosis Infection in a Teenager
Valerie PY Ho, Mark JA Koh
KK Women’s and Children’s Hospital, Dermatology Service, Singapore
Background
Lymphatic malformations are benign malformations of the
lymphatic system which can occur on the skin and mucous
membranes. They can be categorized as superficial or deep,
and congenital or acquired. Acquired cutaneous
lymphangiectasia (ACL) of the vulva is a rare condition and
results secondary to pelvic lymphatic obstruction which leads to
dilated dermal and subcutaneous lymphatic channels. It has
been associated with chronic abdominal-pelvic inflammatory
diseases, infections and malignancies.
Case Presentation Histology of the lesions shows dilated lymphatic channels (black arrows).
A previously well 14-year old teenage girl was treated for

military tuberculosis and tuberculosis of the gastrointestinal
tract, which was complicated by protein-losing enteropathy and
ileal perforation. She underwent resection of the terminal ileum
and an ileostomy creation. Intraoperative findings showed
extensive peritoneal contamination with pus and serous fluid.
Her post-operative course was further complicated by
enterococcus faecalis and candida tropicalis intra-abdominal
sepsis and disseminated intravascular coagulopathy. During her
inpatient stay, she developed a crop of translucent vesicles over
the vulva.
The lymphatic channels stained positively for D2-40 immunohistochemical

stain (black arrows).
Discussion
Acquired cutaneous lymphangiectasia of the vulva is a rare and
relatively benign cutaneous condition affecting the dermal
lymphatic channels. They arise because of pelvic lymphatic
obstruction and have been reported in adults after radiotherapy
for cervical and vulva cancer, Crohn’s disease, tuberculosis of
the gastrointestinal tract and filariasis. The lymphatics of the
vulva drain into the superficial and deep inguinal nodes, and
any obstruction to the deep lymphatic vessels will lead to back-
flow and pressure, leading to dilatation of the dermal
lymphatics. The lesions can be asymptomatic, pruritic, or even
painful. Differential diagnoses include genital herpes, viral warts
and molluscum contagiosum. The diagnosis can be confirmed
Acquired lymphangiectasia on the vulva – crops of translucent vesicles
coalescing over the labia majora with a “frog spawn – like” appearance (red by histology, and the lesions will stain positive with D2-40,
arrows) which is a monoclonal antibody directed against human
podoplanin, a protein that is expressed in lymphatic endothelial
cells. In cases of localized vulva lymphangiectasia, an
A punch biopsy performed showed increased numbers of evaluation for a secondary cause should be carried out. While
dilated thin-walled lymphatic channels that stained positively for more commonly associated with malignancy in adults, chronic
D2-40 immunohistochemical stain, consistent with infections like tuberculosis of the gut and chronic inflammatory
lymphangiectasia. Over the course of her 3-month stay, the disease like Crohn’s disease are more common in the
lesions increased over the vulva region, then spontaneously Paediatric population. Treatment of the underlying cause leads
resolved as anti-tuberculosis medications were continued. to complete resolution of the lesions.
References
1. Miceli, A. and K.M. Stewart, Lymphangioma, in StatPearls. 2018, StatPearls Publishing LLC.: Treasure Island FL.
2. Chang, M.B., et al., Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: Clinicopathologic study of 11 patients from a single institution and 67 from the literature. Int J Dermatol, 2016. 55(9): p. e482-7.
3. Tan, S.K. and Y.K. Tay, Images in clinical medicine. Lymphangioma circumscriptum. N Engl J Med, 2012. 366(18): p. 1724.
4. Kalof, A.N. and K. Cooper, D2-40 immunohistochemistry--so far! Adv Anat Pathol, 2009. 16(1): p. 62-4.
Abstract ID SSD-10112
Track Skin Manifestations of Other Systemic Disorders
Contact author Valerie Ho
Country Singapore
Organisation KK Women's and Children's Hospital
Poster: View Here
Rumpel-Leede Phenomenon - A Deceptively Alarming Rash in Otherwise Healthy Infants
Background:
The Rumpel-Leede phenomenon is characterised by acute dermal capillary rupture caused by
tourniquet-like forces leading to distal petechial and purpura on release of the pressure. This
eponymous sign was first reported in 1909 by Theodor Rumpel, then in 1911 by Carl Stockbridge
Leede. It has been associated with conditions that predispose patients to capillary fragility, like scarlet
fever, diabetic microangiopathy, thrombocytopenia, and after blood pressure monitoring. In children, it
has been associated with haematological malignancies like leukaemia, bleeding diatheses, liver
disease, and infantile scurvy. Most recently, it has been reported to be brought on by tourniquet-like
forces of baby carriers in healthy infants.
Case Presentation:
We present a case series of 3 healthy infants with self-limiting Rumpel-Leede phenomenon of the lower
extremities. Two infants developed the phenomenon after being carried in “legs-out, trunk against
parent’s chest” baby carriers with their lower limbs circumferentially constricted prior to the onset over
their distal extremities. One infant also developed a similar presentation after being held down
circumferentially around both upper thigh and legs for vaccinations. Blood investigations of the infants
did not show any bleeding diatheses, and all of them remained well with spontaneous resolution of the
rash.
Discussion:
The acute onset of a petechial and purpuric rash in infants can be alarming and may result in emergency
room consults. Underlying bleeding diatheses or serious conditions such as meningococcemia should
be ruled out. These 3 cases demonstrate the association between excessively tight baby carriers and
the use of constrictive grips to immobilize infants during vaccinations with the development of this self-
limiting condition. Healthcare professionals should be cognizant of this relatively benign condition as a
differential diagnosis to avoid extensive investigations or hospital admissions. Caregivers and
healthcare workers can be advised to avoid prolonged restrictive mechanical forces when carrying their
infants in carriers or handling them.
Poster: SSD-10112
Rumpel-Leede Phenomenon – A Deceptively

Alarming Rash
Valerie PY Ho1, Jin Ho Chong2, Priya Bishnoi1, Sharon MY Wong1
1KK Women’s and Children’s Hospital, Dermatology Service, Singapore
2Raffles Hospital, Children’s Centre, Singapore
Background
The Rumpel-Leede phenomenon is characterized by an
acute dermal capillary rupture caused by tourniquet-like
forces leading to distal petechial and purpura on release of
the pressure. This eponymous sign was first reported in
1909 by Theodor Rumpel, then in 1911 by Carl Stockbridge
Leede. It has been associated with conditions that
predispose patients to capillary fragility, like scarlet fever,
Epstein-Barr virus infection1, diabetic microangiopathy,
thrombocytopenia, and after blood pressure monitoring. In The third infant developed the Rumpel-Leede phenomenon after
children, it has been associated with hematological vaccinations to both mid-thighs. The infant was held
circumferentially around the thighs to immobilize the limbs.
malignancies like leukemia, inherited bleeding diatheses,
liver disease, and infantile scurvy. Most recently, it has been Blood investigations of the infants (Full Blood Count,
reported to be brought on by tourniquet-like forces of baby Prothrombin Time, Activated Partial Thromboplastin Time)
carriers in healthy infants2. did not show any bleeding diatheses, and all of them
remained well with spontaneous resolution of the rash within
Case Presentation days to weeks.
We present a case series of 3 healthy infants with self-
limiting Rumpel-Leede phenomenon of the lower Discussion
extremities. Two infants developed the phenomenon after The acute onset of a petechial and purpuric rash in infants
being carried in “legs-out, trunk against parent’s chest” baby can be worrisome and may result in emergency room
carriers with their lower limbs circumferentially constricted consults. Underlying bleeding diatheses or serious
prior to the onset of petechiae and purpura over their distal conditions such as meningococcemia are some of the
extremities. differential diagnoses to consider3,4. These 3 cases
demonstrate the association between excessively tight baby
carriers and the use of constrictive grips to immobilize
infants during vaccinations with the development of this self-
limiting condition. Healthcare professionals should be
cognizant of this relatively benign condition as a differential
diagnosis to avoid extensive investigations or hospital
admissions. Infants who are otherwise well with no febrile
symptoms, and with a positive history of prior constrictive
The first infant developed the Rumpel-Leede phenomenon after mechanical trauma prior to the rash, with supportive
being carried in a baby carrier. Notice the abrupt demarcation at the physical examination findings, could be evaluated with a
thigh region, which was where the constrictive force was applied screen for underlying bleeding diatheses, and briefly
(dashed line, right picture). observed before allowing discharge3. Caregivers and
healthcare workers should also be advised to avoid
prolonged restrictive mechanical forces when carrying their
infants in carriers or handling them.
References
1. Dubach, P., G. Mantokoudis, and B. Lammle, Rumpel-Leede sign in
thrombocytopenia due to Epstein-Barr virus-induced
mononucleosis. Br J Haematol, 2010. 148(1): p. 2.
In this second infant, the acute Rumpel-Leede phenomenon 2. Nguyen, T.A., et al., Rumpel-Leede Phenomenon Associated With
showed signs of resolution 12 to 18 hours from exposure to the Tourniquet-like Forces of Baby Carriers in Otherwise Healthy
baby carrier. Infants: Baby Carrier Purpura. JAMA Dermatol, 2016. 152(6): p.
728-30.
3. Lee, M.H. and P.L. Barnett, Petechiae/purpura in well-appearing
The third infant also developed a similar presentation 5 infants. Pediatr Emerg Care, 2012. 28(6): p. 503-5.
minutes after being briefly held down circumferentially 4. Wells, L.C., et al., The child with a non-blanching rash: how likely is
meningococcal disease? Arch Dis Child, 2001. 85(3): p. 218-22.
around both upper thigh and legs for vaccinations. All of the
infants were afebrile and clinically well-perfused.
Abstract ID RSMPD06
Track Skin manifestations of other systemic disorders
Contact author Jazlyn Read

Country Australia
Organisation Lady Client Children's Hospital
Poster: View Here
A rare paediatric case of Plastic Plasmacytoid Dendritic Cell Neoplasm masquerading as a

chronic pretibial abscess
Case Presentation
A 13-year-old female presented with a pretibial skin tear after knocking her shin at the site of a
subcutaneous mass. The lesion had flared and then settled with antibiotics several times over the past
12 months, and an incision and curettage 4 months prior sampled necrotic debris and sheets of
lymphocytes in keeping with an abscess. On examination she had a violaceous bruise-like
40x50x10mm lesion with an area of skin loss, and surgical excision and debridement was performed.
On debridement, there was a necrotic centre with trabecular stranding, and dark tissue extended
subcutaneously at the margins. Tissue was sent for culture and histopathology with suspicion of chronic
atypical infection. No organisms were cultured, however microscopic examination revealed a diffuse
infiltrate of abnormal lymphoid cells in the dermis. A Grenz zone was present, with no epidermotropism.
The overall histological appearance of a cutaneous lymphoma was refined to a diagnosis of Plastic
Plasmacytoid Dendritic Cell Neoplasm (BPDCN) based on immunohistochemical combination of CD4
positive, CD56 positive cells and strong positive reactions with CD123 and TCL1. Subsequent bone
marrow aspirate identified low-level BPDCN marrow infiltration. Following further debridement and skin
grafting, she proceeded with multi-agent chemotherapy followed by myeloablative conditioning and
matched sibling donor bone marrow transfer.
Discussion
BPDCN is an aggressive and rare haematodermic neoplasm with less than 50 documented paediatric
cases. Most patients present with erythematous to violaceous cutaneous lesions, and the majority have
cutaneous onset prior to dissemination. Differential diagnosis includes lymphoma and other small cell
neoplasms, and it is often immunohistochemistry that provides diagnostic clues.
This case documents a rare neoplasm with cutaneous involvement as the only presenting feature, and
is clinically relevant due to the delay in diagnosis and multiple features that seemed consistent with a
chronic abscess. BPDCN has a historically poor prognosis, and therefore prompt identification is
essential. This case highlights the importance of formal biopsy and an appropriate
immunohistochemistry panel, as well as suspicion for haematodermic malignancy in the paediatric
population.
Poster RSMPD06
A rare paediatric case of Blastic Plasmacytoid Dendritic Cell Neoplasm

masquerading as a chronic pretibial abscess
Jazlyn Read1, Yun Phua1
1 Lady Cilento Children’s Hospital, Brisbane, Queensland, Australia
Case Presentation
A 13-year-old female presented with a pretibial skin tear after knocking her left shin at the site of a
subcutaneous mass. The lesion had flared and settled with antibiotics several times over the past 12
months, and incision and curettage 4 months prior sampled necrotic debris, haemosiderin pigment
deposition, and sheets of lymphocytes in keeping with an abscess. Ultrasound and MRI were consistent
with a haematoma or an abscess.
On examination she had a violaceous bruise-like 40x50x10mm lesion that was tender to palpation and
had no signs of active infection. There was a central area of skin loss, and surgical excision and
debridement was performed. On debridement, there was a necrotic centre with trabecular stranding, and
dark tissue extended subcutaneously at the margins. Tissue was sent for culture and histopathology with
suspicion of chronic atypical infection. No organisms were cultured, however microscopic examination
revealed a diffuse infiltrate of abnormal lymphoid cells in the dermis, larger than small lymphocytes and
encircling adipocytes and vessels. Haemorrhage and necrosis was present. A Grenz zone was present,
with no epidermotropism.
The overall histological appearance of a cutaneous lymphoma was refined to a diagnosis of Blastic
Plasmacytoid Dendritic Cell Neoplasm (BPDCN) based on immunohistochemical combination of CD4
positive, CD56 positive cells and strong positive reactions with CD123 and TCL1. Tumour cells were also
positive for CD68, CD163, and S100, and negative for CD3, CD20, CD57, CD117, CD138, PAX5,
CD21/35, and myeloperoxidase. EBV was negative. Bone marrow aspirate identified low-level BPDCN
marrow infiltration.
Figure 1: Left pretibial lesion at
Following further debridement and skin grafting, she proceeded with multi-agent chemotherapy followed
approximately 8 months post onset, just
by myeloablative conditioning and matched sibling donor bone marrow transfer.
prior to incision and drainage.
Figure 2: Left pretibial lesion at presentation, with overlying skin tear. Pen markings denote the raised Figure 3: Immediately post excision
area surrounding the lesion.
Discussion References
BPDCN is an aggressive and rare haematodermic neoplasm with less than 50 1. Kim MJ, Nasr A, Kabir B, de Nanassy J, Tang Kmenzies-Toman D, et
al. Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm: A
documented paediatric cases. Most patients present with erythematous to violaceous Systematic Literature Review. J Pediatr Hematol Oncol. 2017;39(7):
cutaneous lesions, and the majority have cutaneous onset prior to dissemination.1,2,3 528-537.
2. Pagano L, Valentini CG, Grammatico S, Pulsoni A. Blastic
Differential diagnosis includes lymphoma and other small cell neoplasms, and it is often plasmacytoid dendritic cell neoplasm: diagnostic criteria and
therapeutical approaches. Br J Haematol. 2016;174(2):188-202.
immunohistochemistry that provides diagnostic clues. Reactive plasmacytoid dendritic
3. Nguyen CM, Stuart L, Skupsky H, Lee YS, Tsuchiya A, Cassarino
cells may also be identified in cutaneous lupus erythematosus, and nodal tissue in some DS. Blastic Plasmacytoid Dendritic Cell Neoplasm in the Pediatric
lymphadenitis and lymphoproliferative disorders, but lack CD56 positivity.4 Population: A Case Series and Review of the Literature. Am J
Dermatopathol. 2015;37(12):924-8.
This case documents a rare neoplasm with cutaneous involvement as the only 4. Facchetti F, Cigognetti M, Fisogni S, Rossi G, Lonardi S, Vermi W.
Neoplasms derived from plasmacytoid dendritic cells. Mod Pathol.
presenting feature, and is clinically relevant due to the delay in diagnosis and multiple 2016;29(2):98-111.
features that seemed consistent with a chronic abscess. BPDCN has a historically poor
prognosis, and therefore prompt identification is essential. This case highlights the
Contact
importance of formal biopsy and an appropriate immunohistochemistry panel, as well as
Dr Jazlyn Read
suspicion for haematodermic malignancy in the paediatric population.
Email: uqjread4@uq.edu.au
Abstract ID: RSMPD27
Track: Skin manifestations of other systemic disorders
Contact Author: Joseph Boustany
Country: Australia
Organization: Liverpool Hospital Sydney Australia
Poster: View Here
Acute haemorrhagic oedema of infancy in a 10-month old male with Gastroenteritis - a

worrisome presentation with a benign course: Case report and Literature Review
Background: Acute haemorrhagic oedema of infancy (AHO) is characterised by the triad of fever;
oedema; and sudden onset rash in a nontoxic infant. The rash typically is depicted as a rosette-, annular
shaped purpuric skin change occurring primarily over the face, ears, and extremities. Its appearance
and rapidity of onset is dramatic often raising great clinical concern. First described in 1913, it has been
rarely reported in literature. The following case highlights a rare acute paediatric dermatological
emergency from our tertiary institution and literature review.
Case Presentation: We report a 10-month old male infant that presented to our tertiary emergency
department with maternal concern for significant purpura and oedema to the lower legs bilaterally
extending up to the knees. The child had been suffering from gastroenteritis in the preceding five days
however this had been resolving over the previous 24 hours. The patient was born premature per-
vaginally at 35+2 weeks gestation following spontaneous rupture of membranes requiring one week in
special care nursery for phototherapy and was otherwise medically well. Immunisations were up to date
and there were no known allergies or regular medications. The child was noted to be achieving
appropriate milestones, thriving and no risk factors for physical abuse. There was no history of bleeding,
bruising or recurrent epistaxis. Laboratory investigations showed mild thrombocytopenia, elevated INR
and lupus anticoagulant. This was attributed to a transient post-viral lupus anticoagulant; otherwise all
other results were within normal limits. A clinical diagnosis was made following dermatology
consultation of AHO.
Discussion: The aetiology is unknown although considered to be an immune complex–mediated

vasculitis. Eighty-four percent of reported cases were preceded by viral infections, medication use and
immunisations. Even though the presentation is worrisome, it has a benign course. It is crucial for the
medical professional involved in emergency paediatric care to make the diagnosis correctly to avoid
unnecessary work up and therapy, while simultaneously monitoring for rare but severe complications.
Prompt recognition of this rare disorder is important to also be able to reassure the parents of the child
of the benign nature and course of this disorder.
Poster: RSMPD27
Regional Scientific Meeting of Paediatric Dermatology 2018

Acute haem orrhagic oedem a of infancy in a 1 0 - m onth
old m ale w ith Gastroent eritis- a w orrisom e presentation
w ith a benign course: Case report and Literature Review
Joseph Boustany Department of Paediatrics, Liverpool Hospital Sydney, Australia

BACKGROUND
Acute haemorrhagic oedema of infancy (AHO) is characterised by the triad of fever; oedema; and sudden onset rash in a
nontoxic infant. The rash typically is depicted as a rosette-, annular shaped purpuric skin change occurring primarily over
the face, ears, and extremities. Its appearance and rapidity of onset is dramatic often raising great clinical concern. First
described in 1913, it has been rarely reported in literature.
CASE PRESENTATION
We report a 10-month old male infant that presented to our tertiary

emergency department with maternal concern for significant purpura and
oedema to the lower legs bilaterally and extending up to the knees. The
child had been suffering from gastroenteritis in the preceding five days
however this had been resolving over the previous 24 hours with improved
oral intake and cessation of diarrhoea. The patient was born premature per-
vaginally at 35+2 weeks gestation following spontaneous rupture of
membranes requiring one week in special care nursery for phototherapy
and was otherwise medically well. Immunisations were up to date and there
were no known allergies or regular medications. The child was noted to be
achieving appropriate milestones, thriving and no risk factors for physical
abuse. There was no history of bleeding, bruising or recurrent epistaxis.
Laboratory investigations showed mid thrombocytopenia, elevated INR and
lupus anticoagulant. This was attributed to a transient post-viral lupus
anticoagulant; otherwise all other results were within normal limits. A
clinical diagnosis was made following dermatology consultation of AHO.
DISCUSSION
The aetiology is unknown although considered to be an immune complex–

mediated vasculitis. Eighty-four percent of reported cases were preceded
by viral infections (acute upper respiratory tract infection, gastroenteritis),
medication use (namely antibiotics) and immunisations. Even though the
presentation is worrisome, it has a benign course. It is crucial for the medical professional involved in emergency
paediatric care to make the diagnosis correctly to avoid unnecessary work up and therapy, while simultaneously
monitoring for rare but severe complications. Prompt recognition of this rare disorder is important to make the diagnosis
correctly to avoid unnecessary work up and therapy and to be able to reassure the parents of the child of the benign
nature and course of this disorder.
REFRENECES
,
1. Acute haemorrhagic oedema of infancy in a 5-week-old boy referred to the Child Protection Unit. Hawkrigg S Johnson A, Flynn J, Thom
G, Wright H. J Paediatr Child Health. 2014 Jun;50(6):487-9. doi: 10.1111/jpc.12453. Epub 2013 Dec 23
2. Acute hemorrhagic edema of infancy: Finkelstein's disease. Long D, Helm K F. Cutis 199861283–284.
3. Infantile acute hemorrhagic edema: a variant of leukocytoclastic vasculitis. Ince E, Mumcu Y, Suskan E. et al Pediatr Dermatol 199512224–227
Abstract ID: NSD-10058
Track: Neonatal Skin Disorders
Contact Author: MichaelaTabalon
Country: Philippines
Southern Philippines Medical Center
Poster: View Here
Subcutaneous Fat Necrosis Of The Newborn, A Case Report
Case Presentation
We present a case of a 30-day old infant born term to a diabetic mother via cesarean section due to
PROM. On the 7th day of life, patient was noted to be irritable with frequent bouts of crying, also with
multiple erythematous nodules and plaques on the back and right deltoid. She was admitted under
pediatrics and was initially managed as a case of cellulitis given IV antibiotics. On physical examination,
there were multiple erythematous discrete, indurated nodules on the back, and a single erythematous
indurated plaque on the deltoid. Skin biopsy done revealed lobular inflammatory infiltrates composed
of lymphocytes, histiocytes, and multinucleate giant cells, as well as foci of fat necrosis and adipose
lobules with needle shaped clefts, consistent with subcutaneous fat necrosis. Serum total calcium,
triglyceride, and fasting blood sugar were normal. Patient was discharged with monthly monitoring of
calcium levels. At 6 months of age, calcium levels remained normal and the skin lesions had improved
significantly.
Discussion
Subcutaneous fat necrosis of the newborn (SCFN) is a rare transient panniculitis presenting with
erythematous to violaceous, frequently tender, nodules or indurated plaques over the back, shoulders,
arms, buttocks, thighs, and cheeks in first 6 weeks of life.
Diagnosis can be made clinically and can be confirmed with histopathology. Cellulitis is a common
differential diagnosis as it presents with erythema and induration. However, prolonged duration of
symptoms and poor response to antibiotics should alert the physician of this diagnosis. Presence of
radially arranged clefts of crystalline triglyceride within fat cells, granulomatous cellular infiltrate
composed of lymphocytes which confirms fat necrosis, and presence of histiocytes, multinucleated
giant cells, and fibroblasts are confirmatory.
SCFN is self-limiting. However, hypercalcemia, a common metabolic complication, can lead to lethargy,
hypotonia, irritability, vomiting, polyuria, polydipsia, constipation, and dehydration. If untreated, this may
lead to seizures, cardiac arrest and renal failure. Chronic problems may also arise due to metastatic
calcifications. Management should focus on prevention of this metabolic complications.
Our case emphasizes the need for prompt diagnosis to avoid unnecessary interventions and prevent
the serious complications of hypercalcemia in infants with SCFN.
Poster: NSD-10058
Subcutaneous Fat Necrosis of the Newborn, a Case Report

Tabalon, Michaela1, Jimenez, Claribel1
1 Department of Dermatology, Southern Philippines Medical Center, Bajada, Davao City, Philippines
Commercial funding: N/A Conflict of interest: N/A
Introduction
Subcutaneous fat necrosis of the newborn (SCFN) is a rare
transient panniculitis usually presenting in first 6 weeks of life. Its
precise incidence is unknown. 1 It presents with erythematous to
violaceous, frequently tender, nodules or indurated plaques over
the back, shoulders, arms, buttocks, thighs, and cheeks. 2 Its etiology
and pathogenesis remains elusive, however, it is linked to several
neonatal and maternal risk factors such as traumatic delivery,
perinatal asphyxia, hypothermia, maternal diabetes mellitus, and
preeclampsia of the mother.3 Risk factors involve hypothermia,
tissue hypoxia and mechanical pressure leading to crystallization of
neonatal subcutaneous fat, followed by tissue necrosis and
granulomatous reaction.1,4
Diagnosis can be made clinically and can be confirmed Fig. 1 Fig. 2
with histopathology. The following are diagnostic: radially arranged
clefts of crystalline triglyceride within fat cells, granulomatous
cellular infiltrate composed of lymphocytes which confirms fat
necrosis, and presence of histiocytes, multinucleated giant cells,
and fibroblasts.5,6
Cellulitis is a common differential diagnosis as it presents
with erythema, induration, and tenderness.7
While SCFN has a generally good prognosis, early
recognition is important since affected infants require monitoring
for associated metabolic complications such as hypercalcemia. 8,9
We report a 30-day old neonate born term to a diabetic mother via
cesarean section due to premature rupture of membranes, who
presented with multiple erythematous nodules and plaques on the
Case Report
back and right deltoid
Patient on her
was born 7th day
term to aof life. mother via caesarian
diabetic
section due to premature rupture of membranes. Following
delivery, patient was admitted at the neonatal intensive care unit
and received a week course of unrecalled intravenous antibiotic and
was discharged in good health.
On the first week of life, mother noted erythematous
nodules on patient’s back. Nodules were observed to increase in
size, with associated incessant crying of the infant, prompting
consult. Laboratory findings showed normal CBC w/ platelet count,
elevated CRP. She was admitted under Pediatrics and was managed
as a case of cellulitis, given IV antibiotics. On the 6th hospital day, no
improvement on the lesions were observed. Patient was then
referred to our service. On physical examination, there were
multiple erythematous discrete, indurated, tender nodules on the
back (largest measuring 2 cm on its widest diameter), single 4 cm x Conclusion
3 cm erythematous indurated non-tender plaque on the deltoid. Subcutaneous fat necrosis is a rare self-limited disease that can
(Fig. 1, 2) Skin biopsy was done on the erythematous, indurated present with metabolic complications particularly hypercalcemia. Cellulitis is
nodule on the lower back revealing a predominantly lobular a common differential diagnosis, however, prolonged duration of symptoms
inflammatory infiltrates composed of lymphocytes, histiocytes, and and poor response to antibiotics should alert the physician of this diagnosis.
multinucleate giant cells, as well as foci of fat necrosis and adipose This case emphasizes the need to identify early recognizable clinical features
lobules with needle shaped clefts, consistent with SCFN. (Fig. 3a, 3b, of SCFN coupled with typical history of maternal complications like gestational
3c) Patient was placed on prone position, given paracetamol. Serum diabetes that can lead the primary care physician to early and correct
total calcium, triglyceride, and fasting blood sugar were requested diagnosis to avoid unnecessary interventions and prevent the serious
revealing normal results. Patient was discharged with monthly complications of hypercalcemia in infants with SCFN. This will help in reducing
monitoring of calcium levels. At 6 months of age, calcium levels morbidity and mortality from SCFN in the newborn.
remained normal and the skin lesions had almost completely
resolved.
References
1. Del Pozzo-Magaña BR, Ho N. Subcutaneous Fat Necrosis of the Newborn: A 20-Year Retrospective Study. Pediatr Dermatol 2016; 33:e353.
2. Tran JT, Sheth AP. Complications of subcutaneous fat necrosis of the newborn: a case report and review of the literature. Ped iatr Dermatol 2003;20:257e61.
3. Burden AD, Krafchik BR. Subcutaneous fat necrosis of the newborn: a review of 11 cases. Pediatric Dermatology. 1999;16(5):384–387.
4. Coondoo, A., R. Lahiry, A. Choudhury, and S. Sengupta. 2013. Tender skin nodules in a newborn. Indian J. Dermatol. 58:328.
5. Weedon D. Panniculitis. In: Weedon's Skin Pathology, 3rd ed, Weedon D (Ed), Churchill Livingstone/Elsevier, Edinburgh 2010. p.1041.
6. Rapini RP. Practical Dermatopathology, Elsevier Mosby, Philadelphia 2005.
7. Grewal S. Subcutaneous fat necrosis of the newborn. Medscape. 2014
8. Chikaodinaka AA, Jude AC. Subcutaneous Fat Necrosis of the Newborn: A Case Report of a Term Infant Presenting with Malaise and Fever at Age of 9 Weeks. Case Reports in Pediatrics.
2015;2015:638962. doi:10.1155/2015/638962.
9. Tuddenham E, Kumar A, Tarn A. Subcutaneous fat necrosis causing neonatal hypercalcaemia. BMJ Case Reports. 2015;2015:bcr2014208460. doi:10.1136/bcr-2014-208460.
Abstract ID: GDT-10019
Track: Genodermatoses
Contact Author: Nirmala Ponnuthurai

Country: Malaysia
Hospital Kuala Lumpur , Malaysia
Poster: View Here
Juvenile Hyaline Fibromatosis: Case Report Of A Rare Inherited Disease
Background
Juvenile hyaline fibromatosis (JHF) is a rare inherited condition that occurs mainly in childhood and is
characterized by tumor-like growth of hyalinized fibrous tissue .
Case presentation
Herein we describe a case of a 14-month-old Orang Asli girl who presented with characteristic multiple
pink pearly papules and nodules over the perinasal, perioral, perianal region with gingival hypertrophy
and joint contractures.
Discussion
Review of etiology, histopathologic characteristics , gene mutation associated with this condition
,management and complications of this disease are further discussed.
Poster: GDT-10019
Juvenile Hyaline Fibromatosis:

Case Report Of A Rare Inherited Disease Institution
•1Nirmala Ponnuthurai, 1Sabeera B, 2Noraini M.Dusa al logo
•1Department Of Pediatric Dermatolology, 2Department of Pathology , Hospital Kuala Lumpur, Malaysia (optional)
Abstract
Juvenile hyaline fibromatosis (JHF) is a rare inherited condition that occurs mainly in childhood and is characterized by
tumor-like growth of hyalinized fibrous tissue .
Herein we describe a case of a 14-month-old Orang Asli girl who presented with characteristic multiple pink pearly papules
and nodules over the perinasal, perioral, perianal region with gingival hypertrophy and joint contractures. Review of
etiology, histopathologic characteristics , gene mutation associated with this condition ,management and complications of
this disease are further discussed.
Case Presentation
A 14 months old girl presents with multiple papules since 3 months of age. She was well till the second month of age when
her mother first started to notice the papules over the neck, ears and perianal region. By the age of one year old she had
progressive painless contractures and had difficulty in moving her limbs. Her mother also noticed progressive swelling of
her gums which made feeding difficult. The pinkish plaque over the perianal region gradually increased in size with age ,
however there were no defecation problems. Apart from skin lesions, joint contracture and failure to thrive there were no
systemic symptoms and her mental function was normal.
She was born term and is a product of non-consanguineous marriage. She is the youngest of 3 children. Her parents,
elder brother and sister are all well. There is no history of early neonatal/infantile death or abortion.
Skin examination showed discrete non follicular painless papules and nodules at the posterior neck, pinna of ears, nose
and chin. The nodules were larger at the lateral side of nose. There were also pinkish colored plaques with herniation seen
at the perianal region. Oral examination revealed gingival hyperplasia. Joint contractures were evident at the hips, wrists,
knees and ankles.
Original article:
Introduction
Materials and methods
Results
Discussion / Conclusion
HISTOLOGY
Child unable to stand due to contractures
DISCUSSION
Juvenile Hyaline Fibromatosis (JHF) is a very rare disease. It is characterized by the deposition of amorphous hyaline
material in the dermis and extracellular spaces of soft tissues .It is inherited by autosomal recessive mode of inheritance
but sporadic cases can occur. The gene related to JHF has been mapped to chromosome 4q21. The exact pathogenesis
is unknown however several theories have been proposed most attributing JHF lesions to aberrant production of
glycosaminoglycans by fibroblast or due to disordered collagen metabolism. The major diagnostic criteria for JHF are
pearl like skin papules, plaques and /or nodules, gingival hyperplasia, osteolytic bone lesions, joint contractures and
histologically deposition of amorphous hyaline material.There are 2 distinctive clinical syndromes , firstly Infantile systemic
hyalinosis (ISH) and secondly Juvenile Hyaline Fibromatosis(JHF).
The common skin findings are polymorphous papules pinkish to white located mainly at the face and neck, around the
nostrils, ears, paranasal folds and the chin. Nodular lesions or plaques are commonly seen at the scalp, limbs and
perianal regions .They grow slowly are painless and have a tendency to recur following excision
Gingival hyperplasia is common that can sometimes be severe and interfere with feeding and result in poor oral hygiene ,
infection and dental carries. Musculoskeletal involvement in JHF is frequent , and flexion contracture of large joints are
most debilitating and most become bedridden by adolescents . It is hypothesized that joint contracture occurs due to
infiltration of the capsules of the joint. Osteolytic bone lesions are commonly observed in the distal phalanges, skull, and
long bones.The Infantile Systemic Hyalinosis (ISH) is characterized by the above finding with the involvement of the
visera( gastrointestinal, cardiac, hepatic splenic and thyroid) with an inevitable fatal outcome.
The diagnosis of JFH can be confirmed by histology. The tumour consists of cords of spindle shaped cells embedded in a
homogenous eosinophilic matrix. They are often found in the subcutaneous, dermis and gingiva although the bones and
joints may also be involved. The common differential diagnosis includes Neuro fibromatosis ,Gingival fibromatosis ,
Nodular amyloidosis , Congenital generalized fibromatosis and Lipoid proteinosis .
There is no specific treatment for JHF. The treatment is only esthetic and its aim is to limit orthopedic diability. Early
surgical removal of skin lesions are may help but recurrences are common. Joint contracture may respond to intra
lesional systemic steroids, physiotherapy and capsulotomy. Gingivectomy may help but on long term gastrostomy and
feeding tube placement should be considered to improve nutrition. Genetic counselling is also of great importance for
future pregnancy.
REFRENCES:
Miyake I, Tokumaru H, Sugino H et al. Juvenile hyaline fibromatosis. Case report with five years’ follow-up. Am J Dermatopathol 17:584-590,1995
Rahman N, Dunstan M, Teare MD et al. The gene for juvenile hyaline fibromatosis maps to chromosome 4q21. Am J Hum Genet 71:975-980,2002.
Landing BH, Nadorra R. Infantile Systemic Hyalinosis: Report of four cases of disease, fatal in infancy, apparently different from juvenile systemic hyalinosis. Ped Path 6:55-79,1986
Adamicova K, Fetisovova Z, Mellova Y et al. Juvenile hyaline fibromatosis. Bratisl Lek Listy 99:587-596,1998
Contact Author: Retno Dwi Utami
Country: Indonesia
Poster: View Here
APLASIA CUTIS CONGENITA DUE TO INTRAUTERINE VARICELLA INFECTION: A CASE

REPORT
Background : Aplasia cutis congenita (ACC) is a rare congenital condition such as the absence of the
skin, most involving the epidermis and dermis, some cases in subcutaneus tissue; which commonly
localized or widespread or scarred at birth. The exact pathophysiology remains unclear, many possible
factors reported play a role. Varicella during pregnancy can be associated with severe ilnesses for both
the mother and her neonate. Varicella may cause intrauterine infection at any stage during pregnancy.
Case Presentation : A 4 days old baby boy, came with ulcers on his both knees since birth, no history
of birth trauma. The mother had varicella infection during 2nd trimester pregnancy. No history of intake
of drugs such as methimazole, carbimazole, misoprostol, heparin, valproic acid, nor heroin, cocain,
alcohol in mother during pregnancy. No skin disorders or abnormalities in family history. On
dermatological examination on genu dextra region there was an ulcer 2x2cm in size, and on genu
sinistra region there was an ulcer measuring 3x1cm in size; and there was inflammation around both
ulcers. The serology examination showed persistence of varicella-zoster virus immunoglobulin both
patient and mother. No significant abnormalities on babygram radiology examination. Histopathological
examination support ACC. Patient were given topical therapy using 0,1 ml conditioned-medium or
secretome derived from mesenchymal stem cells papillary dermis/HDP-CM (Human Dermal Papillae-
Conditioned Medium) + 10 gr water-based antioxidant gel, which showed improvement.
Discussion : Evaluation of a patient with ACC is directed by the detail history (including obstetric and
family history) which can help determine the etiology and type of ACC, further work-up should be
customized based on symptoms and clinical findings, so as ACC can be immediately administered to
decrease the complications. Literature stated prognosis of ACC on the extremities is commonly good,
and the prognosis in this patient is good, ACC heal with epithelization and leave an atrophic scars.
Complications are usually due to the associated abnormalities.
Poster: GDT-10038
APLASIA CUTIS CONGENITA DUE TO
INTRAUTERINE VARICELLA INFECTION:
A CASE REPORT
Retno Dwi Utami, Indah Julianto, Suci Widhiati
Department of Dermatovenereology
Medical faculty of Sebelas Maret University / Dr. Moewardi General Hospital, Surakarta, Indonesia
email : retnodwiutami@gmail.com
Background :
Aplasia cutis congenita (ACC) is a rare congenital condition such as the absence of the skin,
most involving the epidermis and dermis, some cases in subcutaneus tissue; which commonly
localized or widespread or scarred at birth. The exact pathophysiology remains unclear, many
possible factors reported play a role. Varicella during pregnancy can be associated with severe
ilnesses for both the mother and her neonate. Varicella may cause intrauterine infection at any
stage during pregnancy.
Case :
A 4 days old baby boy, came with ulcers on his both knees since birth, no history of birth trauma.
The mother had varicella infection during 2nd trimester pregnancy. On dermatological
examination on genu dextra region appeared ulcer ± 2x2cm in size, and ulcer ± 3x1cm in size on
genu sinistra region; and there are inflammation appearances around both ulcers. The serology
examination showed persistence of varicella-zoster virus immunoglobulin both patient and
mother. No significant abnormalities on babygram radiology examination. Histopathological
examination support ACC. Patient were given topical therapy using 0,1 ml conditioned-medium
or secretome derived from mesenchymal stem cells papillary dermis/HDP-CM (Human Dermal
Papillae-Conditioned Medium) + 10 gr water-based antioxidant gel, which showed improvement.
1a 2a 3a 4a 5a
1b 2b 3b 4b 5b
Figure. 1a: ulcer on genu dextra (1st visit); 1b: ulcer on genu sinistra (1st visit); 2a&2b: application of 0,1
ml conditioned-medium or secretome derived from mesenchymal stem cells papillary dermis/HDP-CM +
10 gr water-based antioxidant gel; 3a&3b: day 3 (2nd visit); 4a&4b: day 6 (3rd visit); 5a&5b: day 10 (4th
visit) showed improvement, ACC heal with epithelization and leave an atrophic scars.
Discussion :
Evaluation of a patient with ACC is directed by the detail history (including obstetric and family
history) which can help determine the etiology and type of ACC, further work-up should be
customized based on symptoms and clinical findings, so as ACC can be immediately
administered to decrease the complications. Literature stated prognosis of ACC on the
extremities is commonly good, and the prognosis in this patient is good, ACC heal with
epithelization and leave an atrophic scars.
References :
1. Gupta R, Gupta MK, Kakkar M. An Unusual Association of Aplasia Cutis Congenita with Twin Pregnancy and Maternal Varicella. Indian J
Clin Pract. 2014;24(9):854–5.
2. Karaman A, Kahveci H, Kacmaz E, Ataoglu S. Aplasia cutis congenita and limb anomaly : A case of non-scalp lesion. Goztepe Tip Dergisi.
2013;28(4):220–3.
3. Antaya R, Schaffer JV. Developmental Anomalies. In: Bolognia JL, Jorizzo JL, Schaffer JV, Callen JP, Cerroni L, Heymann WR, et al,
editors. Dermatology. 3rd ed. New York: Elsevier Saunders; 2012. p. 993-1010.
Contact Author: Kathleen Mitch Gomez
Poster: View Here
Neu-Laxova Syndrome: A Spectrum Of Multiple Congenital Anomalies And Collodion Membrane

In A Preterm Newborn Filipino Male
CASE REPORT
Introduction
Neu-Laxova syndrome (NLS) is a rare, heterogenous, autosomal recessive disorder characterized by
a spectrum of congenital malformations such as intrauterine growth retardation, cutaneous
manifestations, facial dysmorphism, limb deformities, central nervous system anomalies, and
ambiguous or hypoplastic genitalia. The degree of involvement and presence of these findings vary
among reported cases. Considered generally lethal, most afflicted infants are stillborn, or expire shortly
after birth. NLS is caused by gene mutations involved in the serine metabolism pathway. To date,
approximately 78 cases have been reported in the medical literature.
Case Presentation
We are presenting a case of a preterm newborn male born at 29 weeks age of gestation to non-
consanguineous Filipino parents. Upon birth, he manifested with typical features such as microcephaly,
sloping forehead, hypertelorism, sparse eyelashes, congenital cataracts, broad and flattened nasal
bridge, low-set ears, eclabium, cleft palate, micrognathia, short neck, collodion membrane, fissuring of
skin particularly around the flexural contractures of the upper and lower extremities, camptodactyly,
edematous rocker-bottom feet, and hypoplastic testes. The absence of intrauterine growth restriction in
our patient was the most notable feature of this case. Computed tomography of the head demonstrated
dilated ventricles, diffuse cerebral atrophy, compressed cortices with irregular margins, and
craniosynostosis. Chromosomal analysis of peripheral blood specimen revealed a normal male
karyotype (46, XY). Based on the preponderance of characteristic features, a diagnosis of NLS was
considered. Despite intensive monitoring, the patient succumbed to respiratory failure and expired after
5 weeks.
Conclusion
NLS is a multiple malformation syndrome affecting various anatomic systems. Associated with a
grave prognosis, emphasis on educating high-risk families regarding the importance of regular
consultations throughout the duration of the pregnancy is imperative. Counseling is also mandatory
due to the 25% risk of recurrence. Ichthyosis, a main feature of this syndrome, predisposes the
patient to thermal instability, dehydration, electrolyte imbalance, cutaneous infections, and sepsis. As
dermatologists, it is of utmost importance that we efficiently manage complex cases like NLS to
prevent further complications. A multi-disciplinary approach to management is crucial.
Poster: GDT-10040
N EU-LA XO VA SYN D RO M E:
A SPECTRUM O F M U LTI PLE CO N GEN I TAL AN O M ALI ES AN D
CO LLO D I O N M EM BRAN E IN A PRETERM N EW BO RN FI LI PI N O M ALE
Kat hl ee n M i t ch U. G o m ez, M D , D PD S, Be ned i ct o d L. Car p i o , M D , FPD S,

Ei l een Re g al ad o - M o r al es, M D , FPD S, A r m el i a Lap i t an- To r r es, M D , FPD S
Department of Dermatology, Ospital ng Maynila Medical Center, Manila, Philippines
I N T RO D U C T I O N
Neu-Laxova syndrome (NLS) is a rare autosomal
recessive disorder characterized by a spectrum of
congenital malformations. Generally lethal, most
afflicted infants are stillborn or expire shortly after
birth. To date, approximately 78 cases have been
reported in medical literature.
C A SE PRESEN T A T I O N
A preterm newborn male, born at 29 weeks age of
gestation to non-consanguineous Filipino parents,
presented with the following at birth: microcephaly,
sloping forehead, hypertelorism, sparse eyelashes,
cataracts, broad and flattened nasal bridge, low-set
ears, eclabium, cleft palate, micrognathia, short neck,
collodion membrane, skin fissuring around flexural
contractures, camptodactyly, edematous rocker-
bottom feet, and hypoplastic testes. Hematologic
examination showed pancytopenia. Chromosomal
analysis of peripheral blood revealed a normal male
karyotype (46, XY). Computed tomography scan of the
head demonstrated dilated ventricles, diffuse cerebral
atrophy, compressed cortices, and craniosynostosis.
Based on the preponderance of characteristic features,
a diagnosis of NLS was considered. The patient was
placed in an oxygen-supported humidified incubator,
and supportive therapy with intravenous fluids,
emollients, and antibiotics were initiated. Despite
intensive monitoring, he succumbed to respiratory
failure and expired after 5 weeks.
D I SC U SSI O N C O N C L U SI O N
NLS manifests as IUGR, cutaneous findings (ichthyosis, Emphasis on educating high-risk families regarding the
edema, collodion baby, harlequin fetus), facial importance of regular consultations throughout the
dysmorphic features (slanted forehead, hypertelorism, pregnancy is imperative. Counseling is mandatory due to
protruding eyes, ectropion, absent eyelids or the 25% risk of recurrence. Ichthyosis, a main feature of
eyelashes, flat/abnormal nose, low-set/malformed ears, NLS, predisposes the patient to thermal instability,
eclabium, micrognathia, cleft lip/palate), limb dehydration, electrolyte imbalance, cutaneous infections,
anomalies (flexion contractures, digit/limb deformity, and sepsis. A multi-disciplinary approach is crucial to
syndactyly, camptodactyly, rocker-bottom feet, prevent further complications.
scoliosis, poorly mineralized bones), central nervous RE F E RE N C E S
system anomalies (microcephaly, lissencephaly, Acuna-Hidalgo R, Schanze D, Kariminejad A, Nordgren A, Janecke A, Hoischen A, et al. Neu-Laxova
hypoplastic cerebellum, microgyria, dilated ventricles, syndrome Is a Heterogeneous Metabolic Disorder Caused by Defects in Enzymes of the L-Serine
Biosynthesis Pathway. Am J Hum Gen. 2014;95:285-293.
spina bifida), and ambiguous/hypoplastic genitalia. Benke P, Hidalgo R, Braffman B, Jans J, van Gassen K, Sunbul R, El-Hattab A. Infantile Serine Biosynthesis
Majority of cases occur in countries with high rates of Defect Due to Phosphoglycerate Dehydrogenase Deficiency: Variability in Phenotype and Treatment
Response, Novel Mutations, and Diagnostic Challenges. J Child Neurol. 2017:32(6):543-549.
consanguineous marriages. Parental consanguinity has Coto-Puckett WL, Gilbert-Barness E, Steelman CK, Stuart T, Robinson HB, Shehata BM. A Spectrum of
Phenotypical Expression of Neu-Laxova syndrome: Three Case Reports and a Review of the Literature. Fetal
been documented in 42% of cases. The exact Pediatr Pathol. 2010;29:108-119.
pathogenesis remains elusive. Recent studies suggest Manning MA, Cunniff CM, Colby CE, El-Sayed YY, Hoyme HE. Neu-Laxova syndrome: detailed prenatal
diagnostic and post-mortem findings and literature review. Am J Med Genet A. 2004;125:240-249.
mutations in genes involved in serine synthesis to be Mattos EP, Silva AA, Magalhaes JAA, Leite JCL, Segal SL, Gus-Kessler R, Perez JA, Vedolin LM, Torreblanca-
Zanca A, Lapunzina P, Ruiz-Perez VL, Sanseverino MTV. Identification of a premature stop codon mutation in
the cause among affected individuals. No formal the PHGDH gene in severe Neu-Laxova syndrome—Evidence for phenotypic variability. Am J Med Genet A.
2015:167(6):1323-9.
diagnostic criterion has been proposed; diagnosis is Ozcan D, Derbent M, Seckin D, Bikmaz Y, Agildere M, Sandre-Giovannoli A, et al. A Collodion Baby with
based on the uniform constellation of clinical findings. Facial Dysmorphism, Limb Anomalies, Pachygyria and Genital Hypoplasia: A Mild Form of Neu-Laxova
Syndrome or a New Entity? Ann Dermatol. 2013;25(4):483-488.
Contact Author: Kenneth Fong
Country: Singapore
Organisation: National Skin Centre
Poster: View Here
Molecular Analysis Of Circumscribed Scalp Leukotrichia In An Infant Born To A Mother With

Oculocutaneous Albinism Type 1
Oculocutaneous albinism is a heterogeneous group of pigmentary disorders characterised by partial or

complete absence of pigment in skin, hair and eyes due to an inherited defect in enzymes involved in
melanin synthesis. OCA can be divided into 4 main types (OCA1-4), depending on the underlying
mutated gene. OCA 1 results from autosomal recessive mutations of the TYR gene, resulting in reduced
or absent tyrosinase activity in melanocytes. Here, we describe a 6 month old Pakistani boy who
presented with a congenital patch of circumscribed leukotrichia over the right parietal scalp, born to a
mother with OCA1. Clinical examination did not reveal any other cutaneous or ocular abnormalities.
Molecular analysis was carried out to investigate somatic mosaicism as a cause. Sequencing of
maternal DNA extracted from peripheral blood revealed a homozygous c.1204C>T transition mutation
in TYR, resulting in a premature termination codon at position 402. Genomic DNA from the proband
was then extracted from 10 hairs plucked from lesional and unaffected areas of the scalp. TYR
sequencing of DNA from lesional and unaffected scalp both revealed heterozygous c.1204C>T
mutations, without evidence of loss of heterozygosity or a second TYR mutation in lesional DNA.
Additional blood and tissue samples were not available for further analysis. This suggests that somatic
mosaicism is unlikely to account for the circumscribed scalp leukotrichia in this patient harbouring a
heterozygous TYR mutation. Alternative causes may include other unknown genetic or epigenetic
modifiers, or other unrelated pigmentary disorders.
Poster:GDT-10071
Molecular analysis of circumscribed scalp leukotrichia

in an infant born to a mother with oculocutaneous
albinism type 1
Kenneth Fong, Joey Lai-Cheong, Joanna Gach, John McGrath
Introduction
Oculocutaneous albinism (OCA) is a heterogeneous group of pigmentary disorders characterised by partial or
complete absence of pigment in skin, hair and eyes due to an inherited defect in enzymes involved in melanin
synthesis. OCA1 results from autosomal recessive mutations in the tyrosinase (TYR) gene, resulting in reduced or
absent tyrosinase activity in melanocytes. Cutaneous mosaicism refers to the presence of two or more genetically
heterogeneous cell populations in skin. This may manifest in a number of specific patterns, depending on the
underlying cell component involved. Here, we describe a 6 month old Pakistani boy who presented with a congenital
patch of circumscribed leukotrichia over the right parietal scalp, born to a mother with OCA1. Molecular analysis
was carried out to investigate cutaneous mosaicism as a potential mechanism underlying this presentation.
Case Report
Fig 1. Circumscribed leukotrichia over the Fig 2. Pedigree illustrating autosomal recessive inheritance of OCA 1 in the
right parietal scalp of a 6 month old maternal lineage. There is a history of consanguinity among the proband’s
Pakistani boy. maternal grandparents, as indicated by a double line.
A 6 month old Pakistani boy presented with a patch of white hair over his scalp which was noted since birth (Fig
1). He was born to a mother with OCA1, with multiple affected individuals from the maternal side of the family
(Fig 2). Clinical examination revealed a circumscribed patch of leukotrichia over the right parietal scalp. No
other cutaneous lesions were noted, and the rest of his physical examination including hearing and vision were
normal. It was hypothesized that cutaneous mosaicism resulting from a loss of heterozygosity in the TYR gene
was a possible mechanism underlying this circumscribed scalp leukotrichia.
Genomic DNA from the proband was extracted from 10 hairs plucked from
affected and unaffected scalp respectively. Consent for peripheral blood
sampling and skin biopsies from the proband was not available. Genomic DNA
was also extracted from peripheral blood samples from both parents.
Polymerase chain reaction and Sanger sequencing of the TYR gene were carried
out according to standard protocols. All aspects of this study were conducted in
accordance with the Declaration of Helsinki Principles.
Sequencing of maternal DNA revealed a homozygous c.1204C>T transition

mutation in exon 4 of the TYR gene, which converts an arginine residue at that
position to a premature termination codon. Paternal DNA was homozygous for
the wildtype sequence. Sequencing of DNA obtained from the proband’s
Fig 3. Sanger sequencing of TYR reveals a
homozygous c.1204C>T mutation in
unaffected scalp revealed a heterozygous c.1204C>T mutation as expected.
maternal DNA. DNA from both affected and However, loss of heterozygosity was not observed in DNA obtained from
unaffected scalp are heterozygous for the affected scalp. Additional sequencing of the rest of the TYR gene did not reveal a
same mutation. Paternal DNA is wildtype. second TYR mutation.
Conclusion
Cutaneous mosaicism in monogenic skin disorders has been well described. In our case, genetic analysis did not
reveal loss of heterozygosity or a second TYR mutation. Additional underlying mechanisms may include other
genetic or epigenetic modifiers, which remain to be elucidated. Alternative unrelated causes could also include other
naevoid or pigmentary disorders. Nonetheless, this study illustrates how interesting clinical phenotypes can
potentially be used to help us understand fundamental mechanisms underlying skin diseases.
Contact Author: Tantari Sugiman
Country: Indonesia
Dr. Saiful Anwar General Hospital
Poster: View Here
Aplacia Cutis Congenita
Background: Aplasia cutis congenita is a congenital absence of almost or part of the skin layers that
can occur as a solitary defect or as multiple lesions. This congenital disease occurred very rarely in
new born. Although most commonly seen on the scalp, aplasia cutis congenita can affect any part of
the body, including the trunk and limb. Management of the ACC itself depends on the size, site or
magnitude of the defect ,which may lead to an infection.
Case Presentation: A new born male, presented with a wound on the scalp. There were no other
wounds in other part of bodies. The patient was born at 39 weeks of gestation with birth weight 4098
gram. There was history of intrauterine TORCH infection, no maternal history other drugs intake except
from those prescribed by her doctor. Physical examination showed healthy, infant,with temperature
37.5ÂºC, head length 37 cm, chest length 34 cm. Dermatological examinations on parietal scalp
showed a single ulcer with clean based, well defined with, irregularly edge, diameter 1cm. Gram staining
from ulcer pus showed polymorphonuclear lymphocyte. Head CT scan showed ventriculomegaly
without sign of hydrocephalus. The patient was diagnosed with aplacia cutis congenital. The treatment
was given wet dressing twice daily and sofratulle. Evaluation at 2 weeks showed closed wound
surrounded by well define erythematous patch covered by yellowish crust in the middle and without any
hair growth.
Discussion: Early management of injuries to congenital aploplectic aplasia that include closure or
moisturizing dressings may help to avoid scarring. A defect of less than 2 cm in diameter will generally
heal and close spontaneously with a dressing containing antibiotics. Approximate times of wound
healing in superficial lesion aplasia cutis congenita differ and depend on width and depth of the lesion.
Closed wound left a hairless scar.
Keywords: Aplacia cutis congenita, ulcer, wet dressing.

Poster: GDT-10074
Contact Author: Michael Hartanto Angriawan
Country: Indonesia
Departement of Dermatology and Venereology,

Faculty of Medicine Universitas Indonesia
Poster: View Here
PACHYONYCHIA CONGENITA WITH ORAL LEUKOKERATOSIS IN A 2 YEARS OLD BOY

A CASE REPORT
Background: Pachyonychia congenita is a rare genodermatosis. Prevalence is estimated at between

5000-10000 worldwide. It affects nails, skin, oral mucous, larynx, hair, and teeth.
Case presentation: A 2-year-old boy, has 20 nails abnormality present since age of 9 days old. Parents
noticed hoarseness of his voice since birth. Family history was unremarkable and no history of
consanguinity. From physical examination there were multiple follicular papules, hyperpigmented
plaques on the neck, chest, back, and the both side of his medial thigh, elbow and knee. All nails are
dystrophic, discolored with subungual hyperkeratosis producing a distal elevation of the nail plate and
wedge-shaped deformity, and palmoplantar keratoderma. Oral mucosal examination revealed
asymptomatic oral leukokeratosis over the dorsum of the tongue. Skin biopsy from a follicular papule
on the knee showed acanthosis and lamellar hyperkeratosis. Patient was referred for laryngoscopy
examination and found leukokeratosis in the posterior commissures and 1/3 of midline.
Discussion: The clinical features of this case is consistent with the diagnosis of pachyonychia congenita
type 1 with clinical manifestations of skin, nails, oral, and larynx. There was no history of similar
diseases in the family, this case's condition might be caused by spontaneous mutations. Currently,
there was no satisfactory therapy and patient was given supportive and symptomatic treatment.
Keywords: pachyonychia congenita

Poster: GDT-10081
Email: dr_michaelhartanto@yahoo.com
BACKGROUND
Pachyonychia congenita is a rare genodermatosis. Prevalence is estimated at between 5000-10000 worldwide. It affects nails,
skin, oral mucosa, larynx, hair, and teeth.1,2
CASE REPORT
Histopathology
• A 2-year-old boy, has 20 nails abnormality since age 9 days and
hoarseness since birth.
• Family history was unremarkable without consanguinity.
• Physical examination: multiple follicular papules, hyperpigmentation
plaques on the neck, chest, back, and the both side of his medial thigh,
elbow and knee, all nails plate are dystrophic, discolored with subungual
hyperkeratosis producing a distal elevation of the nail plate and wedge-
shaped deformity, and palmoplantar keratoderma.
• Oral mucosal examination revealed asymptomatic oral leukokeratosis
over the dorsum of the tongue. • Skin biopsy from a follicular papule on
the knee showed unspecific findings
(acanthosis and lamellar hyperkeratosis,
HE 40X).
Clinical Manifestations • No features of psoriasis or lichen planus
were presented.
Laryngoscopy
Leukokeratosis was found on laryngoscopy

examination in the posterior commissures and
1/3 of midline.
DISCUSSION
This case was consistant with pachyonychia congenita type 1 featuring all clinical manifestations of skin, nails, oral, and
larynx.3 As there was no history of similar diseases in the family, it might be caused by spontaneous mutations. Currently,
there was no satisfactory therapy and patient was given supportive and symptomatic treatment.3,4
References
1. Smith FJD, Hansen CD, Rull PR, Kaspar RL, McLean WHI, O’Toole E, Sprecher E. Pachyonychia Congenita. 2006 Jan 27 [udpated 2017 Nov 30]. In: Adam MP, Ardinger HH,
Pagon RA, etal., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle;1993-2018.
2. Shah S, Boen M, Kenner-Bell B, Scwhartz M, Rademaker A, Paller AS. Pachyonychia Congenita in Pediatric Patiens: Natural History, Features, and Impact. JAMA Dermatol.
2014; 150(2): 146-53.
3. Rathore PK, Khullar V, Das A. Pachyonychia Congenita Type 1: Case Report and Review of the Literature. Indian J Dermatol. 2016; 61(2):196-99.
4. Rodríguez H, Cuestas G, Zanetta A, Balbarrey Z. Pachyonychia congenita with involment of the larynx. Acta Octolarrinolaringol Esp. 2014;65(3):208-10.
Contact Author: Rodinda Hutabarat
Country: Indonesia
Indonesian Medical Association (Ikatan Dokter

Indonesia)
Poster: View Here
Ichthyosis Vulgaris
Introduction: Ichthyoses are a family of genetic diseases characterized by dry, thickened, scaling skin.
Among of all ichthyoses, ichthyosis vulgaris is the most common type. Prevalence of ichthyosis vulgaris
is 1:100 in Europe with relatively mild scaling disorder. The onset of the disease often manifests within
the first year, although infants usually have normal skin. Mutations of filaggrin (FLG), the human gene
encoding profilaggrin that contains multiple filaggrin molecules, have a role to play in ichthyosis vulgaris.
Case: A 10 year-old male child, 27 kilograms, had scaling and cracking skin all over the body, especially
the lower extremities, since he was six month-old. Collodion membrane and erythroderma was not
found at birth. Hair and mucous membrane were normal. Growth and development were
normal.Unknown topical medication was applied onto the skin. No history of family of similar skin
problem. Differential diagnosis of this patien were ichthyosis vulgaris, keratosis pilaris and xerosis cutis.
Patient was diagnosed with ichthyosis vulgaris based on clinical manifestation. Genetic assesment was
not done. Patient was treated with moisturizer (ceramide) and retinoid acid 0,05% solution. Omega-3,
fatty acid, and vitamin A supplement consumption was recommended. After 2 months, clinical
improvement was seen.
Discussion: In ichthyosis vulgaris, filaggrin mutation may cause reduction of natural moisturizing factor.
Therefore, application of moisturizer (ceramide) is needed. Ceramide is an occlusive moisturizer that
form a hydrophobic film to retard transepidermal water loss (TEWL) of stratum corneum. Furthermore,
application of retinoid acid 0,05% solution as keratolytic enhances proliferation of basal keratinocytes
that induces thickened epidermis and consequent peeling of stratum corneum to reduce scaling and
cracking skin. In addition, supplementary vitamins and minerals are important to maintain normal skin
growth.
Key Word: child, ichthyosis vulgaris, ceramide, retinoid acid

Poster: GDT-10086
ICHTHYOSIS VULGARIS
Rodinda Hutabarat, Margareth Hutabarat, Rointan Simanungkalit
e-mail: rodindamarsha@yahoo.co.id
INTRODUCTION
Ichthyoses are a family of genetic diseases characterized by dry, thickened, scaling skin. Among of all ichthyoses, ichthyosis
vulgaris is the most common type. Prevalence of ichthyosis vulgaris is 1:100 in Europe with relatively mild scaling disorder. The
onset of the disease often manifests within the first year, although infants usually have normal skin. Mutations of filaggrin (FLG),
the human gene encoding profilaggrin that contains multiple filaggrin molecules, have a role in ichthyosis vulgaris.
CASE
A 10 year-old male child, 27 kilograms, had scaling and cracking
skin all over the body, especially the lower extremities, since he
was six month-old. Collodion membrane and erythroderma was
not found at birth. Hair and mucous membrane were normal.
Growth and development were normal. History of topical
medication was found, but the patient’s family didn’t remember Figure 1. Scaling and cracking skin all over the body, especially the lower
extremities, since six month-old.
the medication. No history of family with same problem.
Differential diagnosis of this patien were ichthyosis vulgaris,
keratosis pilaris and xerosis cutis. Patient was diagnosed with
ichthyosis vulgaris based on clinical manifestation. Genetic
assesment was not done. Patient was treated with moisturizer
(ceramide) and retinoid acid 0,05% solution. Omega-3, fatty
acid, and vitamin A supplement consumption was
Figure 2. Clinical improvement after application of moisturizer (ceramide)
recommended. After 2 months, clinical improvement was found. and retinoid acid 0,05% and consumption of omega-3, fatty acid, and
vitamin A supplement in 2 months.
DISCUSSION
In ichthyosis vulgaris, filaggrin mutation may cause reduction of natural moisturizing factor. Therefore, application of moisturizer
(ceramide) is needed. Ceramide is an occlusive moisturizer that form a hydrophobic film to retard transepidermal water loss
(TEWL) of stratum corneum. Furthermore, application of retinoid acid 0,05% solution as keratolytic enhances proliferation of
basal keratinocytes that induces thickened epidermis and consequent peeling of stratum corneum to reduce scaling and cracking
skin. In addition, supplementary vitamins and minerals are important to maintain normal growth.
REFERENCES
1. Foundation for Ichthyosis & Related Skin Types, Inc. Ichthyosis summary. http://www.firstskinfoundation.org/filesDownload.cfm?dl=Ichthyosis%20Summary.pdf. [22 Jan 2018].
2. Oji V, Metze D, Traupe H. Inherited disorder of cornification. In: Burns T, Breathnach S, Cox N, Griffiths C, editor. Rook’s textbook of dermatology. Volume 2. Ninth Edition. London:
John Wiley & Sons, Ltd.; 2016: 65.2-4.
3. Chien AL, Voorhees JJ, Kang S. Topical retinoids. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, editor. Fitzpatrick’s dermatology in general medicine. Volume 2.
Eighth Edition. New York: The McGraw Hill Companies; 2012: 2665-72.
4. Hebert AA at all. Asean atopic dermatitis summit 2014 consensus: understanding the role of emollients in atopic dermatitis management. Singapore: MIMS Pte. Ltd. 2014: 7-9.
5. Anderson B. The effect of dietary change in a patient with ichthyosis vulgaris: a case report. Interg Med. 2015; 14 (3): 55-8.
Contact Author: Charmaine Lim
Country: Singapore
Organization: National Skin Centre
Poster: View Here
Acrokeratosis Verruciformis Of Hopf - A Case Report
Case presentation:
An 18-year-old male patient, a known case of atopic dermatitis since childhood, consulted us for a new
onset of upper limb papules of 2 years duration. Examination showed multiple flesh-coloured flat-topped
papules on the hands, forearms, and knees. Biopsy of the lesions revealed hyperkeratosis, epidermal
hyperplasia and papillomatosis, histologically consistent with acrokeratosis verruciformis of Hopf.
Clinical improvement was observed with application of topical tretinoin.
Discussion:
Acrokeratosis verruciformis of Hopf (AKV) is a rare genodermatosis allelic to Darier’s disease, arising
from an autosomal dominant mutation in the ATP2A2 gene, which encodes for the sarcoplasmic
reticulum calcium-ATPase 2 (SERCA2) pump. We report this case to highlight the importance of clinical
and histopathological examination in differentiating AKV from conditions such as Darier’s disease,
lichen nitidus, plane warts, and epidermodysplasia verruciformis.
Poster: GDT-10105
Acrokeratosis Verruciformis
Of Hopf - A Case Report
Charmaine E Lim, HY Chia, Madeline SL Ho.
National Skin Centre, Singapore.
Acrokeratosis verruciformis of Hopf (AKV) is a disorder of keratinization, classically autosomal
dominant in inheritance. We report a rare sporadic case, and highlight the role of clinical and
histological examination in the diagnosis of AKV.
Case report
An 18-year-old Chinese male with a
background history of atopic dermatitis
presented with asymptomatic upper and lower
limb papules of 2 years’ duration. They were
not preceded by eczematous rashes or
scratching. He had no familial history of similar
skin lesions.
Examination showed multiple flesh-coloured
flat-topped papules on the dorsum of the
hands, and the extensor surfaces of the
Figure 1 – Multiple flesh coloured flat-topped papules on the left
forearms and knees, admixed with excoriated hand dorsum
eczematous papules and old scars. No nail
changes or palmoplantar pits were observed.
Biopsy of the lesions revealed hyperkeratosis,
epidermal hyperplasia and papillomatosis.
Human papilloma virus immunostain was
negative. Based on the clinical and histological
findings, a diagnosis of acrokeratosis
verruciformis of Hopf was made.
The patient was started on tretinoin 0.025%
cream. On review 3 months later, there was
improvement observed with flattening of the
papules. Figure 2 – Biopsy of a papule on the hand dorsum showed
hyperkeratosis, epidermal hyperplasia and papillomatosis
Discussion (hematoxylin and eosin, original magnification x 40)
AKV is a rare genodermatosis, first described by Hopf in 1931. It is allelic to Darier’s disease, arising
from an autosomal dominant mutation in the ATP2A2 gene, which encodes for the sarcoplasmic
reticulum calcium-ATPase 2 (SERCA2) pump. The disease presents at birth or early childhood and is
characterized by small flat-topped verrucous papules typically on the dorsum of hands and feet. Other
features include palmoplantar punctate keratoses and pits, V-shaped nicks and longitudinal grooves of
the nails. A few sporadic cases with a later age of onset have been described in the literature.1
Diagnosis can be challenging because of its rarity, as well as clinical resemblance to various other
conditions. Histological examination can differentiate AKV from Darier’s disease, plane warts, and
epidermodysplasia verruciformis. Key features include orthokeratosis, hypergranulosis, and
acanthosis. Marked papillomatosis, often resembling church spires, is characteristic. While AKV and
the acral lesions of Darier’s disease are clinically similar, AKV may be differentiated histologically by
the absence of acantholytic dyskeratosis.
Treatment options include destructive modalities, such as cryotherapy or laser ablation, if the patient
has cosmetic concerns. In our case, topical tretinoin was effective in reducing the thickness of the
lesions.
Reference: 1. Bang CH, Kim HS, Park YM, Kim HO, Lee JY. Non-familial Acrokeratosis Verruciformis of Hopf. Ann Dermatol Vol. 23, Suppl. 1, 2011.
Abstract ID RSMPD09
Track Genodermatoses
Contact author Wai Leong Kok
Country Singapore
Organisation National Skin Centre
Poster: View Here
A rare case of ectodermal dysplasia - hypotrichosis with juvenile macular dystrophy
Case Presentation
A 16-year old Indian male presented with thin sparse hair since childhood with normal eyebrows and
facial hair. There was no family history of a similar presentation. He had no nail or teeth abnormalities,
nor did have other orthopaedic or organ involvement.
His past medical history was significant for juvenile-onset macular dystrophy on regular follow up with
the ophthalmologist. He does not have developmental delay. There was also no family history of similar
problems. He is the youngest child of two non-consanguineous parents and has two elder brothers who
were unaffected.
He underwent gene testing and was found to have a CDH3 (P-cadherin) gene mutation. His diagnosis
was hypotrichosis with juvenile macular dystrophy (HJMD). He was subsequently lost to follow-up.
Discussion
HJMD is an autosomal recessive disorder that presents with hypotrichosis and progressive macular
degeneration resulting in severe visual impairment. Affected individuals have normal bone, teeth and
nail development. Early gene testing to establish the diagnosis remains crucial and the management
may require a multidisciplinary approach and long term follow up for complications.
In summary, it is important to maintain a high index of suspicion to establish the diagnosis of ectodermal
dysplasias. Dermatologists should consider referral for genetic testing early if there is a suspicion of
ectodermal dysplasia in the appropriate clinical setting.
Poster: RSMPD09
A rare case of ectodermal dysplasia -

hypotrichosis with juvenile macular dystrophy
WL Kok, MTW Chio. National Skin Centre, Singapore.
Case Presentation
1
A 16-year old male presented with thin sparse hair since
childhood with normal eyebrows and facial hair. There was
no family history of a similar presentation. He had no nail,
teeth abnormalities or other skeletal involvement. He was
seen in the outpatient dermatology clinic for rashes on the
face, upper limbs and trunk of a 3-year duration.
Examination revealed erythematous folliculocentric
papules in the aforementioned distribution and he was
treated for keratosis pilaris with topical therapy.
His past medical history was significant for juvenile-onset

macular dystrophy. He developed early amblyopia since
childhood and is on regular follow up with the
ophthalmologist. He does not have developmental delay.
There was also no family history of similar issues. He is
the youngest child of two non-consanguineous parents
and his siblings were unaffected.
He underwent gene testing and was found to have a

CDH3 (P-cadherin) gene mutation. The diagnosis was
hypotrichosis with juvenile macular dystrophy (HJMD). He 2
was unfortuantely subsequently lost to follow-up.
Discussion
Ectodermal dysplasias represent a rare group of inherited

disorders with gene mutations leading to structural and
functional abnormalities in tissues derived from the
ectoderm. These lead to anomalies of the hair, teeth, nails,
and sweat glands. However, clinical features may be
subtle with phenotypic variation.
HJMD is an autosomal recessive disorder that presents

with hypotrichosis and progressive macular degeneration
resulting in severe visual impairment1. Affected individuals
have normal bone, teeth and nail development. Early gene
testing to establish the diagnosis remains crucial and the
management may require a multidisciplinary approach and
long term follow up for complications.
The association of keratosis pilaris and HJMD is

uncommon but has been reported2. Keratosis pilaris has Figures
1: Hypotrichosis in this patient with sparse hair seen
also been reported with other ectodermal dysplasia
throughout the scalp.
syndromes like Odonto-Onycho-Dermal Dysplasia, 2: Erythematous folliculocentric papules on the trunk
Witkop's syndrome and Ectodermal Dysplasia and Cleft suggestive of keratosis pilaris.
lip/palate (Magarita Island syndrome).
References:
1. Kjaer K.W., Hansen L., Schwabe G.C. Distinct CDH3 mutations cause
In summary, it is important to maintain a high index of ectodermal dysplasia, ectrodactyly, macular dystrophy (EEM
suspicion to establish the diagnosis of ectodermal syndrome) J Med Genet. 2005;42:292–298..
2. Indelman M, Hamel CP, Bergman R, et al. Phenotypic diversity and
dysplasias. Dermatologists should consider referral for mutation spectrum in hypotrichosis with juvenile macular dystrophy. J
genetic testing early if there is a suspicion of ectodermal Invest Dermatol 2003;121(5):1217–20.
dysplasia in the appropriate clinical setting. Acknowledgements:
Dr Ng See Ket for his clinical input in the management of this case..
Abstract ID RSMPD13
Contact author Shi Yun Chia
Country Singapore
Organisation KK Women's and Children's Hospital
Poster: View Here
Focal facial dermal dysplasia: Two Case Reports with distinctive features
Introduction
Focal facial dermal dysplasias(FFDD) are a group of rare genetic disorders characterized by
atrophic/dysplastic lesions localized to the temporal or pre-auricular areas. FFDDs are further classified
into 4 subtypes based on location, facial features and inheritance pattern. We present 2 cases with
distinct features.
Case description
Case A
A Vietnamese-Singaporean-Chinese newborn was noted on routine screening to have atrophic lesions
over bilateral temporal regions. He failed routine universal newborn hearing screen on the right, had
right microtia and complete right ear canal atresia. There was positive family history of similar skin
lesions in the mother’s 22 year old cousin. The child was planned for Magnetic-Resonance-Imaging of
the brain and right ear canal. Eye examination by the ophthalmologist was normal. This patient did not
have facial features suggestive of Brauer/Setleis syndrome. Presence of right microtia, auditory canal
atresia with hearing loss has not been described in literature, thus could be a new subtype with unique
phenotypic features.
Case B
A two month old Chinese girl was referred from the polyclinic for atrophic lesions over bilateral pre-
auricular areas. A small aplasia cutis lesion involving the scalp was noted; cranial ultrasound revealed
no abnormality. She did not have any other visible extra-cutaneous abnormalities and was likely to have
type IV FFDD. Aplasia cutis on the scalp has not been described with type IV FFDD. This could be a
coincidental finding or could add on to the existing features of type IV FFDD.
Skin biopsy was not necessary to establish diagnosis in both patients as the clinical features were
distinctive.
Discussion
FFDDs could be misdiagnosed as aplasia cutis congenita. Clinicians must be aware of these rare
entities. There are various classification systems for FFDD described in the literature. Recent studies
have identified mutations and genetic aetiologies, providing insights into the phenotypic and genotypic
heterogeneity underlying these dermatological developmental defects. In a patient with clinical
suspicion of FFDD, it is pertinent to examine and investigate for other facial and intracranial
abnormalities, which may be associated with the underlying genetic aetiology. A detailed family history
may guide further genetic investigations.
Poster: RSMPD13
Focal Facial Dermal Dysplasia: Two Case

Reports with Distinctive Features
Chia Shi Yun1, Priya Bishnoi2, Wong Mun Yee Sharon1,2
1 Department of Paediatrics, KK Women's and Children's Hospital, Singapore
2 Dermatology Service, KK Women’s and Children’s Hospital, Singapore
Introduction
Focal facial dermal dysplasias (FFDD) are a group of rare genetic disorders characterized by atrophic/dysplastic
lesions localized to the temporal or pre-auricular areas. FFDDs are further classified into 4 subtypes based on
location, other facial features and inheritance pattern. We present here 2 cases with distinct features.
Case A Case B
A Vietnamese- Singaporean Chinese newborn was A two month old Chinese girl was referred from the
noted on routine screening to have atrophic lesions polyclinic for atrophic lesions over bilateral pre-
over bilateral temporal regions, with more involvement auricular areas. A small aplasia cutis lesion involving
of the left than the right. He had failed the routine the scalp was also seen; cranial ultrasound revealed
universal newborn hearing screen on the right, and
no abnormality. The child did not have any other
subsequently found to have right microtia and
visible extra-cutaneous abnormalities and is thus
complete right ear canal atresia. There was a positive
family history of similar skin lesions in the mother’s 22 likely to have type IV FFDD. Aplasia cutis on the scalp
year old cousin. The child was planned for Magnetic has not been previously described with type IV FFDD
Resonance Imaging of the brain and right ear canal. Eye and thus this could just be a coincidental finding or
examination by the ophthalmologist was normal. At could add on to the existing features of type IV FFDD.
present this patient did not have any characteristic
facial features suggestive of Brauer/Setleis syndrome.
Presence of right microtia and auditory canal atresia
with hearing loss have not been described in the
literature before and thus could be a new subtype with
unique phenotypic features.
B E F
Clockwise from
left:
D – Aplasia
Cutis lesion on
scalp
E – Left pre-
auricular lesion
F – Right pre-
auricular lesion
Clockwise from left: G – Left pre-
A – Bilateral auricular lesion
temporal lesions 4 months later
B – Right temporal (at 6 months of
atrophic lesions D age) G
A
C – Left temporal
atrophic lesions
C
Skin biopsy was not necessary to establish diagnosis in both patients as the clinical features were distinctive.
Discussion and Conclusion
FFDDs could be misdiagnosed as aplasia cutis congenita, thus clinicians must be cognizant of these rare entities.
There are various classification systems for FFDD described in the literature. Recent studies have also identified
mutations and genetic aetiologies, providing insights into the phenotypic and genotypic heterogeneity
underlying these dermatological developmental defects. In a patient with clinical suspicion of FFDD, it is
pertinent to examine and investigate for other facial and intracranial abnormalities, which may be associated
with the underlying genetic aetiology. A detailed family history may also guide the need for further genetic
investigations.
Abstract ID RSMPD15
Contact author Nia Srie Haryati
Country Indonesia

Organisation Faculty of Medicine Universitas
Padjadjaran/Hasan Sadikin Hospital, Bandung
Poster: View Here
Blau Syndrome: Identical to Juvenile Idiopathic Arthritis or Not?
Background: Blau syndrome (BS) is a rare autosomal dominant granulomatous inflammatory syndrome
caused by mutation of CARD 15 or NOD2 gene, while juvenile idiopathic arthritis (JIA) is a systemic
autoimmune disorder with unknown ethology. BS affects early childhood and JIA occurs at children
under 16 years old. Both disease difficult to differentiate since have same symptoms as skin lesion,
arthritis, and ocular involvement.
Case: An 11-year old boy presented with recurrent of erythematous papules since 2.5-years old and
arthritis with blurred vision since age of 3-years old. Patient was diagnosed as JIA by Paediatric
Department. From physical examination there were generalized papular skin lesion, boggy cysts of both
wrist joints, knees, and ankle joints, and accompanied pan uveitis, with secondary glaucoma and
cataract. Laboratory examination revealed negative result for rheumatoid factor and antinuclear
antibody test. Histopathology examination from upper arm showed a non-caseating granulomatous
reaction. He was treated with 0.1% mometasone furoate cream for skin lesion, methotrexate 10 mg
subcutaneous once a week for two months, and trabeculectomy for glaucoma. The skin manifestation
was improved as the red macules became hyper pigmented macules, but boggy cysts still remain until
now.
Discussion: BS was differentiated with JIA based on specific features as boggy cyst and non-caseating
granuloma in histopathology examination. Thus, comprehensive examination is need to established
diagnosis of BS.
Keywords: Blau syndrome, boggy cyst, juvenile idiopathic arthritis

Poster:RSMPD15
BLAU SYNDROME: IDENTICAL TO

JUVENILE IDIOPATHIC ARTHRITIS OR NOT?
Nia Srie Haryati, Dwi Putri Larasati, Reiva Farah Dwiyana, Inne Arline Diana, R.M Rendy Ariezal Effendi
Faculty of Medicine Universitas Padjadjaran / Dr. Hasan Sadikin Hospital, Bandung Indonesia
Email: niasrieharyati@gmail.com
INTRODUCTION
Blau syndrome (BS) is a rare autosomal dominant inflammatory syndrome,1-3 caused by mutation of CARD 15 or NOD2
gene.2-4 while juvenile idiopathic arthritis (JIA) is a systemic autoimmune disorder with unknown etiology.5 BS affects early
childhood and JIA occurs at children under 16 years old. Both disease difficult to differentiate since have same symptoms as
skin lesion, arthritis, and ocular involvement.6
Generalized maculopapular rash

CASE REPORT
A 11-year old boy presented with recurrant
of erythematous papules since 2,5-years
old and arthritis with blurred vision since
age of 3-years old. Patient was diagnosed
as JIA by Pediatric Department. From
physical examination there were Boggy cyst
generalized papular skin lesion, boggy cysts
of both wrist joints, knees, and ankle joints,
and accompanied panuveitis, with
secondary glaucoma and cataract.
Laboratory examination revealed negative
result for rheumatoid factor and
antinuclear antibody test. Histopathology Before treatment
examination from upper arm showed non-
caseosing granulomatous reaction. He was
treated with 0,1% mometason furoate
cream for skin lesion, methotrexate 10 mg
subcutaneus once a week for two months,
and trabeculectomy for glaucoma. The skin
manifestation was improved as the red
macules became hyperpigmented macules,
but boggy cysts still remain until now.
After treatment
DISCUSSION Non-
caseosing
Histopathology
granuloma
BS was differentiated with JIA based on
specific features as boggy cyst and non- Cataract
caseosing granuloma in histopathology
examination.6 Thus, comprehensive
examination are need to established
diagnosis of BS. 5-1
6
References
1. Wouters C, Maes A, Foley K, Bertin J, Rose C. Blau syndrome, the prototypic auto-inflammatory granulomatous disease. Pediatr Rheumatol. 2014:12:1-9
2. Caso F, Galozzi P, Costa L, Sfriso P, Cantarini L,dkk. Autoinflammatory granulomatous disease: from Blau syndrome and early-onset sarcoidosis to NOD2-mediated disease and Chron’s disease. RMD
open.2015:1;1-8.
3. Pillai P, Sobrin L. Blau syndrome-associated uveitis and the NOD2 gene. Semin Ophtalmol. 2013:28;327-32.
4. Paller A, Mancini A. Skin sign of other systemic disease. in: Paller A, Mancini A, penyunting. Hurwitz Clinical paedriatric dermatology. 4th Edisi . Sidney. Elsevier; 2011. hlm.562-79.
5. BecMartin T, Doyle T, Doyle T, Rose C. Interstitial pneumonitis in Blau syndrome with documented mutation in CARD15. Arthritis Rheum. 2007:56;1292-4.
6. Glass D, Maender J, Metry D. Two pedriatic cases of Blau syndrome. Dermatol Online J. 2016:12;1-4.
Abstract ID RSMPD19
Contact author Yuri Yogya
Country Indonesia
Perhimpunan Dokter Spesialis Kulit dan

Organisation
Kelamin Indonesia
Poster: View Here
Two Cases of Xeroderma Pigmentosum in One Family:

Different Clinical Severity in Cutaneous Malignancies
Background: Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder, characterized by

cutaneous photosensitivity, premature aging, and the early development of cutaneous and ocular
malignancies. Patients with XP younger than 20 years of age have a 10,000-fold increased risk of
cutaneous basal cell carcinoma (BCC), squamous cell carcinoma (SCC), or melanoma. Development
of cutaneous malignancies may differ among blood relative involving the amount of unscheduled
deoxyribonucleic acid (DNA) synthesis in their survival after ultraviolet (UV) exposure.
Case: Two cases of XP with cutaneous malignancies in one family had been reported. First case of XP
patient with suspected BCC in 14-year-old boy that had been operated of his ulcerated tumor on the
nose. The ulcerated tumor appears at 10 years old. The second case was 8-year-old-boy with SCC on
the scalp and multiple BCC (5 tumours) on face. Tumor on retroauricular area was diagnosed as SCC
based on history taking, physical examination, and histopathological examination. Excision tumor
procedures with Mohs surgery followed by full thickness skin graft reconstruction were performed by
Oncology Surgery Department. Tumor on scalp and face diagnosed as BCC based on dermoscopy and
treated with 5-fluorouracil cream.
Discussion: An XP gene may account for a substantial proportion of genetic predisposition to sun-
induced non-melanoma skin cancer (NMSC) . Some XP genes may predispose to skin cancer and
others may not. Cells from XP patients differ in the amount of unscheduled DNA synthesis in their
complementation group, and in their survival after UV exposure. In our case, the severity of NMSC
differs from patient 1 and patient 2. The patient 2 have more NSMC and was much younger for the
development of carcinoma. The different normal rate of DNA repair may explain this condition. The
limitation of our case is the absence of a genetic study. Treatment is challenging as these patients tend
to develop multiple malignant lesions over time in spite of rigorous and regular protection against UV
radiation. Malignant tumor could be treated with topical, systemic, and surgery.
Poster: RSMPD19
Two Cases of Xeroderma Pigmentosum in One Family:

Different Clinical Severity in Cutaneous Malignancies
Yuri Yogya, Inne Arline Diana, Reiva Farah Dwiyana, R.M Rendy Ariezal Effendi
Departement of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran,
Dr. Hasan Sadikin General Hospital, Bandung
email: yuri.yogya@yahoo.com
¨ Introduction
Xeroderma pig mentosum (XP) is a rare autosomal recessive disorder, characterized by cutaneous photosensitivity,
premature aging, and the early development of cutaneous and ocular malig nancies.1 Patients with XP younger than 20
years of age have a 10,000-fold in creased risk of cutaneous basal cell carcinoma (BCC), squamous cell carcinoma
(SCC), or melanoma.2 Development of cutaneous malignancie s may differ among blo od relative in volvin g the amount
of unscheduled deoxyribonucleic acid (DNA) synthesis in their survival after ultraviolet (UV) exposure.3
¨ Case Report
Two cases of XP with cutaneous malignancies in one family had been reported (Fig 1A). First case of XP patient with
suspected BCC in 14-year-old boy that had been operated of his ulcerated tumor on the nose (Fig 1B). The ulcerated
tumor appeared at 10 years old. The second case was 8-year-old -boy (Fig 1C) with SCC on the scalp (Fig 2A-B) and
multiple BCC on face (Fig 3A-F). Tumor on retroauricular dextra diagnosed as SCC based on history taking, physical
examination, and histopathological examination (Fig 2D). Excision tumor prosedures with Mohs surgery followed by full
thickness skin graft reconstruction (FTSG) (2C) were performed by Oncology Surgery Department. Tumor on scalp
and face diagnosed as BCC based on dermoscopy and treated with 5-fluorouracil cream.
3A 3B
2A
1A 2B
3C 3D
2C
3E 3F
1B 1C
FIG. 2. BCC on the face. (A) Dermoscopic
2D 2E examination showed blue gray globule,
arborizing telangiectasia, blue white color, (B)
FIG. (1A) Family pedigre e. (B) Patient 1, 14-year-old boy FIG. (2A-B) SCC presented as tumor with keratotic surface. (2C) arborizing telangiectazia, blue white color, (C)
with history of ulcerate d tumor on th e nos e, (C) Patient 2, 8- Excisio n tumor w ith Mohs s urg ery foll owe d by FTSG. (2D) Foll ow u p blue-gray globule, leaf-like structure.
year-old boy with SCC and multiple BCC. 4 month after surgery. (2D) Histopath ol ogic al exam in ation sh owe d
tumor mass, horn pearls (yellow arrow) and hyperchromatic nucleus.
¨ Discussion ¨ References
Some XP genes may predispose to skin cancer and others 1. Moriwaki S, Kanda F, Hayashi M, Yamashita D, Sakai Y, Nishigori C.
may not.3 Cells from XP patients differ in the amount of Xeroderma pigmentosum clinical practice guidelines. J Dermatol.
unscheduled DNA synthesis in their comple mentatio n group, 2017 Aug 3.
2. Tamura D, DiGiovanna JJ, Khan SG, Kraemer KH. Living with
and in their survival after UV exposure.4,5 In our case, the xeroderma pigmentosum: comprehensive photoprotection for highly
severity of NMSC differ from patient 1 and patient 2. The photosensitive patients. Photodermatol Photoimmunol Photomed
2014; 30: 146–152.
patient 2 have more NSMC and the median age are much 3. Bowden NA, Beveridge NJ, Ashton KA, dkk. Understanding
younger for development of carcinoma. The different normal xeroderma pigmentosum complementation group using gene
expression profiling after UV-light exposure. Int J Mol Sci.
rate of DNA repair may explain this conditio n.5 The limitation of 2015;16:15985-96.
our case is the absence of a genetic study.1 4. Kaloga M, Dioussé P, Diatta BA, Bammo M, Kourouma S, Diabate A,
dkk. Squamous cell carcinoma in African children with xeroderma
Conclusion: An XP gene may account for a substantial pigmentosum: three case reports. Case Rep Dermatol. 2016 Nov
15;8(3):311-318.
proportion of genetic predispositio n to sun-induced non- 5. D’Errico, Calcagnile A, Canzona F, Didona B, Posteraro P, dkk. UV
melanoma skin cancer. Treatment is challenging as these mutation signature in tumor suppressor genes involved in skin
patients tend to develop multip le malignant lesio ns over time in carcinogenesis in xeroderma pigmentosum patients. Oncogene
2000;19:463-67.
spite of rigorous and regular protection against UV radiation.
Contact Author: Margaret Stephanie Jimenez
Southern Philippines Medical Center
Poster: View Here
A Case Report On A Rare Disease: Dyskeratosis Congenita In A 28 Year Old Male
Introduction: Dyskeratosis congenita is a rare type of genodermatoses which is characterized by the

triad of oral leukoplakia, nail dystrophy and reticulate skin pigmentation. It involves a genetic defect
leading to telomere shortening or defective telomere maintenance. Its incidence is 1 in 1 million people.
It may be associated with development of aplastic anemia, myelodysplastic syndrome, anemia and
solid tumors.
Case Report: We report a 28-year old male who presented initially with reticulate skin pigmentation and
followed by nail dystrophy with associated adermatoglyphia. Further physical examination showed oral
leukoplakia and palmoplantar keratoderma. Dermoscopy findings show irregularly shaped and
unevenly distributed pigmented ‘‘lines and dots with,’’ hypopigmented ‘‘holes consistent with
poikiloderma. Referral to Ophthalmology wad done due to complaint of epiphora, of which noted
lacrimal duct stenosis. Biopsy showed orthokeratotic stratum corneum overlying an atrophic epidermis
with focal basal vacuolar changes with the dermis revealing mild perivascular lymphocytic inflammatory
infiltrates with scattered melanophages. Baseline laboratory results were unremarkable except for
thrombocytopenic finding on complete blood count. Imaging showed compression fracture on L1.
Genetic testing was done which excluded rare type ectodermal dysplasia. Clinical findings, laboratory
and imaging results, as well as genetic testing led to the assessment of Dyskeratosis congenita.
Conclusion: Treatment is multidisciplinary as it shows multi-organ involvement. This is the third case
reported in the Philippines and the first adult case.
Poster: RSMPD28
Dyskeratosis Congenita
in a 28 year-old Filipino Male
Margaret Stephanie L. Jimenez, MD,

Mary Jo Kristine S. Bunagan MD, FPDS, Claribel L. Jimenez, MD, FPDS
Department of Dermatology
Southern Philippines Medical Center, Davao city, Philippines
INTRODUCTION
Dyskeratosis congenita (DKC) or otherwise
known as Zinsser-Engman Cole Syndrome is
associated with the mucocutaneous triad of
nail dystrophy, oral leukoplakia and abnormal
C
reticulate skin pigmentation.1 A
CASE PRESENTATION
Chief complaint: macules
The patient had a 22-year history of reticulate D

pigmentation spreading cephalocaudally. This
was associated with longitudinal ridging and
nail splitting on both toes and nails, loss of
ridging on both palmar aspects, and
hyperhidrosis prompting consult.
Review of systems show epiphora and history

of back pain. E
Physical examination shows generalized skin Fig (A): Oral Leukoplakia. (B)Reticulate skin pigmentation
(C) Nail dystrophy (D)Adermatoglyphia (E)
pigmentation, palmoplantar keratoderma, oral Hypopigmented holes and pigmented skin on dermoscopy
leukoplakia, and epiphora
Dermoscopy shows irregularly shaped CONCLUSION

pigmented lines and dots with pigmented holes DKC has been notably associated with defect
of DKC1 gene causing the X-linked disease. It is
REFERENCES needed for the telomerase complex for telomere
1. Vulliamy T, Marrone A, Knight S, Walne A, survival to last a human life span and slow down
Mason P, Dokal I. Advances in the Understanding telomere loss. 1
of Dyskeratosis Congenita. Blood Apr The diagnosis of DKC can be confirmed by
2006; 107 (7) 2680-2685. gene testing and telomere length. It may also be done
2. Knight S, Vulliamy T, Copplestone A, Gluckman
based on presence of the major features:
E, Mason P, Dokal I. Dyskeratosis
mucocutaneous triad and bone marrow failure.2 DKC
Congenita Registry: Identification of new features
of Dyskeratosis Congenita Br J is a multisystem disorder with principal cause of
3.Fernandez Garcia MS, Teruya-Feldstein J. The mortality being bone marrow failure but are also
diagnosis and treatment of dyskeratosis congenital: predisposed to malignancy and pulmonary fibrosis3.
a review. J Blood Med 2014; 5:157-67. This requires partnership of various subspecialties.
Abstract ID: PMS-10002
Track: Pigmentary Disorders
Contact Author: Rini Hastuti
Country: Indonesia
Dokter Moewardi General Hospital Surakarta

Indonesia
Poster: View Here
COMBINATION OF ORAL MINI PULSE DOSE CORTICOSTEROID WITH

NARROW BAND ULTRAVIOLET B AND TOPICAL CORTICOSTEROID
IN CHILDHOOD VITILIGO
Background:
Vitiligo is a skin depigmentation disorder that frequently occurs, characterized with demarcated,
depigmented macula and patch caused by progressive loss of melanocytes. There are various therapy
modalities for vitiligo. Systemic corticosteroids may halt the progression of the disease and cause
significant repigmentation. Topical corticosteroid is the first line of therapy because it is easy to apply
on children. Narrow band ultraviolet B (NB UVB) is proven to be a safe treatment option for vitiligo in
children with lesions ≥ 20% of body surface area. There is no single therapeutic method that has proven
effectiveness with minimal side effects.
Case:
A 5-year-old girl, complained of increasingly widespread white patches in the pubic area in the last
three years . Physical examination of the scalp region showed poliosis, and multiple discrete
depigmented patches were seen in upper extremities, inguinal and genital area. Score of vitiligo
disease activity score (VIDA) is +2 and score of vitiligo scoring area index (VASI) is 2.685. Additional
examination with potassium hydroxide (KOH) 10% gave negative results and the examination using
Wood's lamp reveals white fluorescence. Routine blood examination, glucose levels were within normal
limits and levels of 25-OH vitamin D was 24 ng / ml. Combination therapy of corticosteroid mini pulse
dose, NB UVB and topical corticosteroids showed clinical improvement.
Discussion:
The choice of treatment in pediatric patients with vitiligo is a challenge for dermatologists, because not
all therapeutic modalities can be used in pediatric patients. In this patient, combination therapy of oral
corticosteroid mini pulse dose, NB UVB and topical corticosteroids gave satisfactory clinical
improvement so it can be considered as a treatment of choice in pediatric patients.
Keywords: mini pulse dose corticosteroid, NBUVB, vitiligo

Poster: PMS-10002
Contact Author: An Jian Leung
Country: Singapore
National University Singapore, Yong Loo Lin

School of Medicine
Poster: View Here
Cutaneous Mosaicism: An Interesting Case Of Becker's Nevus Presenting In A Checkerboard

Mosaic
Introduction: Becker’s nevus (BN) is a late-onset epidermal nevus due to epidermal overgrowth,
melanocytes and hair follicles. It typically occurs during adolescence with a male predominance and
may enlarge over time. Malignant transformation is extremely rare. They appear as reticulated
hyperpigmented patches/macules characterized by hypertrichosis. Occasionally, acneiform papules
may develop over the patches. It may rarely be associated with Becker’s nevus syndrome, comprising
of ectodermal abnormalities.
Case presentation: The patient, an 18-year-old Chinese male, presented to our tertiary centre with a
3-year history of multiple enlarging brown patches over his left hypochondria, right upper anterior
chest and beneath his left eye. On examination, there was a 12x10cm reticulated brown patch over
his right anterior chest, a 3x4cm brown patch over his left hypochondria and reticulated brown
macules over the left infraorbital region. They were also hair bearing. No ophthalmic or musculosketal
abnormalities were present. A clinical diagnosis of BN with checkerboard mosaicism was made. He
was referred for laser treatment.
Discussion: While BN is a common hamartomatous finding, its presentation as multiple lesions with a
checkerboard pattern of cutaneous mosaicism is rare. Few case reports exist in the literature currently
despite being described as characteristic of the condition. Cutaneous mosaic patterns include
blaschkoid, phylloid, checkerboard, lateralization or large patches without midline separation. Other
forms of Spitz or achromic naevi presenting in the checkerboard pattern is even rarer. This interesting
feature of BN speaks to the underlying aetiology of its derivation from mesodermal origins during early
embryonic development.
Conclusion:
BN is a benign pigmentary condition which exists in several distinct patterns with little malignant
potential. Its presentation in a checkerboard pattern is rare.
Poster: PMS-10018
Cutaneous mosaicism: An interesting case of Becker' s

Nevus presenting in acheckerboard mosaicism
An Jian LEUNG1, May LIAU2, Sam S YANG2, Lucinda TAN3
1. Yong Loo Lin School of Medicine, National University of Singapore
2. National University Hospital, Singapore, 3. National Skin Centre, Singapore
Background Clinical pictures
Becker’s nevus (BN) is a late-onset epidermal nevus due to Figure 1 (left):

epidermal overgrowth, melanocytes and hair follicles1. It 3 x 4 cm brown
patches in the
typically occurs during adolescence with a male
infra-orbital
predominance and may enlarge over time. Malignant region of the left
transformation is extremely rare. They appear as reticulated eye extending
hyperpigmented patches/macules characterized by close to the
hypertrichosis. Occasionally, acneiform papules may lower eye lid
margin. Right
develop over the patches. It may rarely be associated with
eye was
Becker’s nevus syndrome, comprising of ectodermal unaffected. No
abnormalities. ophthalmic
complications
were noted.
Case presentation
An 18-year-old Chinese male presented with a 3-year history

of multiple enlarging reticulated brown patches beneath his
left eye (fig. 1), a 3x4cm brown patch over his
left hypochondrium and a 12x10cm reticulated brown patch
over his right anterior chest. There is hair growth over these
brown patches. There were no associated ophthalmic
or musculosketal abnormalities noted to be present.
A clinical diagnosis of BN with checkerboard mosaicism was

made.
Discussion
While BN is a common hamartomatous finding, it’s

presentation as multiple lesions with a checkerboard
pattern of cutaneous mosaicism is rare1. Few case reports
exist in the literature currently despite being described as
characteristic of this condition. Cutaneous mosaic patterns
include blaschkoid, phylloid, checkerboard, lateralization or
large patches without midline separation3. Other forms of
Spitz or achromic naevi presenting in the checkerboard
pattern is even rarer.
Figure 2 (above):
This interesting feature of BN may be due to its derivation 12 x 10 cm and 3 x 4 cm reticulated brown patches over right anterior
chest wall above the nipple extending to the infra-clavular region and
from mesodermal origins during early embryonic left hypochondrium respectively. These patches were hair bearing were
development. not associated with acneiform papules.
Conclusion References & acknowledgements
BN is a benign pigmentary condition which exists in several 1. Ruggieri, M. and Praticò, A. (2015). Mosaic Neurocutaneous Disorders
and Their Causes. Seminars in Pediatric Neurology, 22(4), pp.207-233.
distinct patterns with little malignant potential. Its 2. Rogers, M. (1992). Epidermal Nevi and the Epidermal Nevus Syndromes:
presentation in a checkerboard pattern is rare. A Review of 233 Cases. Pediatric Dermatology, 9(4), pp.342-344.
3. Happle, R. (1993). Mosaicism in human skin. Understanding the patterns
and mechanisms. Archives of Dermatology, 129(11), pp.1460-1470.
Contact Author: Djohan Kho
Country: Indonesia
Haji Adam Malik General Hospital / Faculty of

Medicine Universitas Sumatera Utara
Poster: View Here
Piebaldism: A Case Report
Background
Piebaldism is a rare autosomal dominant disorder, which caused by mutation in the c-kit protooncogen
that affect the migration of melanocyte in embryo. It can affect both genders. The depigmented lesions
are commonly presented at birth and stable throughout life with pigmented macules that can develop
around or inside the lesions. It is a benign condition of the skin but can affect the patient psychosocially..
Case Presentation
We presented a case of female baby, 3 months old, with hypopigmented spots on her forehead, chest,
abdomen, forearm and upper thigh area with white forelock that appear since birth. There are several
hyperpigmented dots distributed on her back, upper buttocks, and right leg. The skin lesions remain
stable and did not increase in size. Generally, there are no complaints on her growth and development.
Based on the clinical features, we diagnosed the patient with piebaldism.
Discussion
There are various presentations of cutaneous hypopigmentations in children. The diagnosis of
piebaldism in our patient was based on medical history and clinical evaluation. Piebaldism is
characterized by congenital depigmentation macules that distributed symmetrically mainly on forehead,
ventral trunk, and limbs, which can be seen in our patient. There are several varieties in phenotype,
ranging from mild to severe that correlate with the site and type of mutation. Treatment of piebaldism is
challenging but necessary to improve the quality of life. It consists of cosmetic camouflage, surgical
treatment, and a combination of laser with autologous cultured epidermal grafting. The use of sunscreen
is recommended to protect the amelanotic area from sunburns.
Poster:PMS-10057
Contact Author: Deo Adiel Wong
Jose R. Reyes Memorial Medical Center Department of

Dermatology
Poster: View Here
A Rare Case Of Linear And Whorled Nevoid Hypermelanosis With Global Developmental Delay,
Scoliosis And Retinal Degeneration With Dermoscopic Features
Background:
Only 40 cases of the rare sporadic Linear and Whorled Nevoid Hypermelanosis (LWNH) are described
in medical literature worldwide, characterized by hyperpigmented, reticulated, streaky and whorled
patches along Blaschko lines, without atrophy or preceding inflammation. It reflects an underlying
mosaicism or chimerism and occasionally associated with systemic abnormalities.
Case Presentation:
A 5 year-old female presented with multiple uniformly hyperpigmented patches with midline
demarcation over right posterior trunk, some reticulate and blaschkoid over lower extremities and some
linearly arranged over right upper extremity. Lesions were asymptomatic and unchanged since birth.
Dermoscopy showed brown structureless zones interrupted by dotted perifollicular hypopigmentation
over right posterior trunk and anterior thigh, reticular pattern over right posterior thigh and linear brown
streaks with alignment along lines of Blaschko over right upper extremity. Histopathology revealed basal
layer hyperpigmentation, sparse superficial lymphocytic dermal infiltrates, melanocytic hypermelanosis
and flat-topped papillomatosis. Hematological and biochemical tests were non-contributory. The patient
has decompensated thoracic scoliosis with 2-cm divergence from plumb line. Developmental Pediatrics
referral revealed global developmental delay and examination under anesthesia showed peripheral
retinal degeneration on both eyes.
Discussion:
A form of neurocutaneous syndrome in which embryological migration of melanoblasts and cortical
neurons from the neural crest may explain the common mechanism, the pathogenesis of LWNH is
unknown though developmental somatic mosaicism leading to proliferation and migration of two mixed
populations of melanocytes with different potential for pigment production and genetic factors are
thought to be possible causes with nearly all cases occurring sporadically. It can be cosmetically
disfiguring though may decrease in severity with age. While data is lacking because of its rarity, affected
individuals should be evaluated for disease associations (delays, skeletal and other systemic
abnormalities). Different dermoscopic features described in three case reports before were all present
in our patient (net-like, parallel streaks, dotted perifollicular hypopigmentation) which is in keeping with
its mosaic nature. No promise of chromosomal abnormalities can be found using sequencing. Together
with the limited information regarding effective treatment (peels, hydroquinone 2%, depigmenting
agents, lasers) without much success, the combination of features has been presented with its rarity
kept in mind.
Poster: PMS-10076
Contact Author: Aryani Inda Astri
Country: Indonesia
Faculty of Medicine Universitas Indonesia/Dr. Cipto

Organisation:
Poster: View Here
12 YEARS OLD GIRL WITH ERYTHEMA DISCHROMICUM PERSTANS
Background: Erythema dischromicum perstans (EDP) is pigmentary disorder of unknown etiology,

characterised with asymptomatic macular hyperpigmentation over the body. It usually favors individuals
with skin types II to IV, frequently appears during the first to third decade of life. In general, proposed
treatments for EDP are unsuccessful. Spontaneous remission can occur, but many patients have EDP
for years. In this paper, we present a 12 years old girl with EDP.
Case Presentation: A 12 years old girl with skin type IV complained of a 2 months history of progressive
asymptomatic gray-brown macules and patches on the trunk, extremities and face. The lesions started
with erythematous papules on axilla, then become a hyperpigmented lesions, which distributed on the
facial and extremities. She did not take any prescription, denied contact with any substances and denied
history of infection. Physical examination revealed a symmetric, non scaly, gray-brown macules to
patches on the facial, extremities and truncus along lines of cleavage, no erythematous elevated
borders. Routine laboratory tests were normal. Histopathological findings showed vacuolar
degeneration of basal cell, melanin focal incontinencia, mild infiltration of lymphocyte on the dermis.
She was treated with topical momethasone furoate and moisturizer.
Discussion: Erythema dischromicum perstans (EDP) is a rare asymptomatic, slowly progressive,

macular hyperpigmentation, symmetrically distributed over the neck, trunk, upper and lower limbs and
the face. A thin erythematous border may be present in acute lessions and disappear after several
months. These findings are similar with the patients.There are some triggers such as drugs (antibiotics,
pesticides) and infections. Any triggers in this case still not known. Histopathological of EDP is vacuolar
degeneration of basal cells, some pigmentary incontinence of epidermal, and perivascular lymphocytes
infiltration in the upper dermis, which supported diagnosis of EDP in this patient. Our patient was treated
with topical momethasone furoate and moisturizer, showed reduction in hyperpigmentation.
Poster: PMS-10099
12 YEARS OLD GIRL WITH

ERYTHEMA DISCHROMICUM PERSTANS
Inda Astri Aryani1, Radema Maradong2, Fitriani3, Soenarto Kartowigno4
Faculty of Medicine Sriwijaya University/Dr. Mohammad Hoesin General Hospital Palembang,
Indonesia
INTRODUCTION
• Erythema dischromicum perstans (EDP) is a rare pigmentary disorder of unknown etiology, characterized
by asymptomatic macular hyperpigmentation with slightly raised erythematous border over the body. It
usually favors individual with skin types III to IV.1
• It affected from childhood to old age, most frequently in young age.2 It can triggered by drugs, chemical
agents and infections.3 Patients with EDP can have a spontaneous remission, but some of them have
EDP for years.4 There is no consistently effective treatment.2
CASE
A 12 years old girl with skin type IV, had a history of progressive asymptomatic gray-brown macules and patches on the
face, trunk and extremities since two months ago. It started with an erythematous papules and patches on the axilla,
trunk, and extremities then become hyperpigmented in one month. No history of any prescription or exposure to any
substances and history of infection couldn’t be defined. Physical examination: a symmetric non scaly gray-brown
macules to patches on the face, extremities and along lines of cleavage of the trunk. Routine laboratory tests were
normal, histopatological finding showed dermal pigmentary incontinence and lymphocyte infiltration
CLINICAL FINDING
After treatment
Baseline Follow up
HISTOPATHOLOGICAL FINDING TREATMENT

Lymphocyte • Momethasone furoate 0,1% cream 2x/day
infiltration • Urea 10% cream 2x/day
• Vitamin C 500mg/day/oral
Melanin
incontinence
DISCUSSION
• Erythema dischromicum perstans is a slowly progressive hyperpigmentation, with a slightly raised of erythematous
border.4 The border disappear after several months.4 These findings are similar with the patients
• The trigger factors still not known. We suspected that she had a history of pityriasis rosea because the lesion was
distributed along lines of cleavage of the trunk, and might be contributed as a trigger factors. This finding is similar
with a report by Antonov et al. 4 Histopathological findings in this patient supported diagnosis of EDP.
• Application of topical corticosteroid can reduce the melanin incontinence.3 The patient treated by momethasone
furoate, urea and oral vitamin C, which showed reduction of hyperpigmentation.
CONCLUSION
Erythema dischromicum perstans is a rare, slowly progressive, characterized by hyperpigmentation with slightly raised
of erythematous border. In this case, pityriasis rosea was suspected to be a trigger factor of EDP. Spontaneous
remission can occur. Corticosteroid usage in this patient is useful to reduce the hyperpigmentation.
REFFERENCES
1.Chang MW. Disorder of hyperpigmentation. In: Bolognia JL, Jorizzo JL, Scaffer JV, eds. Dermatology.3rd ed. Mosby: Elsevier Limited.2012:.p.1050
2.Van geel N, Speeckaert R. Ashy dermatosis and erythema dischromicum perstans. In: Griffiths CEM, Barker J, Bleiker T, Chalmers R, Creamer D, editors.
Rook’s Textbook of Dermatology. 9th ed. Oxford: John Wiley and Sons; 2016. p.88.32-33
3.Numata T, Harada K, Tsuboi R et al. Erythema dischromicum perstans: identical to ashy dermatosis or not?. Case Rep Dermatol.2015;7:146-150
4.Antonov NK, Braverman I, Subtil A et al. Erythema dischromicum perstans showing resolution in an adult. J American Acad Dermatol.2015;1(4):185-7
Abstract ID: VII-10035
Track: Viral Infections
Contact Author: Hoi Ling Wong
Country: Malaysia
Poster: View Here
Treatment Of Perianal Warts In Children With 5% Imiquimod Cream: Case Series
Background:
The incidence of anogenital warts among Paediatric population is reported to be on the rise, with
preponderance of female. These lesions are caused by Human Papilloma Virus ( HPV) , mostly
serotypes 6 and 11. We report 3 paediatric cases of perianal warts which were successfully treated with
topical 5% imiquimod cream, an immunomodulatory agent.
Case Presentation:
Case 1
A 17-month-old Chinese girl presented with 2-month history of multiple brownish confluent verrucous
papules at the perianal region. Her grandmother , her main caretaker , has viral warts over the hands.
Detailed history and physical examinations showed no evidence of sexual abuse. She was treated with
imiquimod 5% cream for 24 weeks and more than 90% of her lesions have resolved.
Case 2
A 25-month- old Malay girl complained of multiple skin-coloured papules around the perianal region for
1 month of duration. No history of viral wart among the care takers or herself. There was no evidence
of sexual abuse as well. She was given imiquimod 5% cream for 18 months and her lesions resolved.
Case 3
A 28-month-old Malay boy was found to have multiple greyish verrucous plaques and papules at the
perianal area for 1 month. Care takers and patient himself have no viral warts anywhere else in the
body. History and physical examinations were not suggestive of sexual abuse. He was treated with
imiquimod 5% cream for 12 weeks and the perianal lesions have resolved.
Discussion
The transmission routes of HPV in children are different from adults. Anogenital warts in children below
1 year old are believed to be vertically transmitted. Other proposed routes of transmission include direct
contact, autoinoculation and sexual abuse. Chemical and physical destruction are the treatment
options. However, cryotherapy, laser therapy and ablative electrocautery are painful procedures which
usually done under general anaesthesia. Topical imiquimod 5% cream has recently been reported to
be effective in treating anogenital warts in children. As compared to other published data, our patients
appeared to respond well to topical imiquimod despite taking a longer duration. Besides, topical
imiquimod has been well tolerated by our patients.
Poster: VII-10035
Treatment of Perianal Warts in Children with 5%

Imiquimod Cream: Case Series
WONG HL, LEONG KF, SABEERA BKI
PAEDIATRIC DERMATOLOGY DEPARTMENT, INSTITUT PEDIATRIK HOSPITAL KUALA LUMPUR
INTRODUCTION
The incidence of anogenital warts among the Paediatric population is reported to be on the rise. These lesions are caused by
human papilloma virus (HPV), mostly serotypes 6 and 11. Despite being an uncommon condition, anogenital warts frequently
presents a therapeutic challenge, especially when it is extensive. We report 3 cases of perianal warts in children which were
successfully treated with topical 5% imiquimod cream, an immunomodulatory agent.
Case Age Gender History Treatment Photo pre treatment Photo post treatment
No duration
1 17 Girl • 2 month history of multiple brownish confluent 24

month verrucous papules at the perianal region . weeks
• Detailed history and physical examination did not
reveal any features of sexual abuse.
• Her grandmother, one of her caretakers was
reported to have viral warts over the hands.
• Her mother reports no history of genital warts
during pregnancy.
2 25 Girl • noted to have multiple skin-coloured papules 18
month around the perianal region for 1 month duration. Weeks
• Her mother is her sole care taker.
• She herself reports no history of warts.
• There is no history or features suggestive of
sexual abuse.
3 28 Boy • complained of having multiple greyish verrucous 12

month papules around the perianal region for 1 month. Weeks
• His parents are main care takers
• His parents have no history to warts.
• Further history and physical examination
revealed no evidence of sexual abuse.
DISCUSSION
The prevalence of anogenital warts in children is about 1.5% with the female preponderance. The incubation period remains
unknown in pediatric population. The transmission routes in children are different from adult. Anogenital warts in children
under the age of 1 year old are believed to be vertically transmitted. Hence, it is important to explore the presence of genital
warts in the mothers during antenatal and perinatal periods. Other proposed routes of transmission of HPV include direct
contact e.g. through caretakers with hand warts, formite transmission, autoinoculation and sexual abuse. Social history and
focused physical examinations to look for evidence of sexual abuse must be emphasized when one encounter anogenital
warts in a child. Diagnosis of anogenital warts is mainly made on physical examination.
Treatment of anogenital warts include chemical and physical destruction of the lesions. Cryotherapy, laser therapy and
ablative electrocautery are painful procedures and usually require general anaesthesia. Topical imiquimod 5% cream have
been reported to be effective in treating anogenital warts in children although it has not been licensed for use in children
below 12 years old. Imiquimod induces keratinocytes to produce interferon alfa and activate the cytokines pathway to inhibit
viral propagation. Duration of therapy in other published studies range between 2 and 12 weeks. In our cases, the duration of
therapy ranged between 12-24 weeks. The prolonged duration of treamtnent in case one was due to non compliance.
However, imiquimod 5% cream is well tolerated by our patients due to its painless application and convenience with low
recurrence rate. There has been no adverse reaction reported.
CONCLUSION
Imiquimod 5% cream could be the first line of treatment for anogenital wart in children , especially for extensive lesions.
However, the high cost could be a potential hindrance as the first line therapy.
REFERENCES
1. Takayuki et al Effectiveness of imiquimod 5% cream for treatment of perianal warts in a 28-month-old child, Paeidatrics International Volume 53, Issue 5 October 2011 Pages 764–766
2. Nives Sikanic Dugic et al.Treatment of anogenital warts in an 18-month-old girl with 5% imiquimod cream. Acta Dermatovenerol Croat 2014;22(1):40-43
3. Meltem Dinleyici et al. Giant Condyloma Acuminate Due to Human Papillomavirus Type 16 in an Infant Successfully Treated with Topical Imiquimod Therapy,Dermatol Reports. 2015 Dec 3; 7(3):6134.
.
Abstract ID: VII-10059
Track: Viral Infections
Contact Author: Ariyati Yosi
Country: Indonesia
Haji Adam Malik General Hospital / Faculty of
Medicine Universitas Sumatera Utara
Poster: View Here
Molluscum Contagiosum In Children On Anogenital Area: A Serial Case
Introduction
Molluscum contagiosum is benign viral infection caused by molluscum contagiosum virus (MCV). There
are variations in incidence but it is continue to rise in these few decades. The condition is more common
in children especially in tropical country with lesion frequently found on the limbs and trunks. The lesions
can be found in anogenital area and can be categorized as sexually transmitted disease in adults who
are sexually active. Ano-genital lesion can be detected in children but it is usually not caused by sexual
contact.
Case Ilustration
We presented two cases of molluscum contagiosum in children, both female, aged 6 years old and 3
years old. In both cases, the lesions were found only in anogenital area four weeks prior to visit. From
the first case, we found multiple skin-colored papules with erythematous base that are typical for
molluscum contagiosum lession. The same characteristic lesions were also found in the second case,
but there was no inflammation. There is no family member and friends with the same condition. In the
second case, the patient has a history of using public swimming pool. We did not found any history of
trauma and sexual contact in patients.
Discussion
Generally molluscum contagiosum lesions are asymptomatic and do not affect quality of life but
occasionally symptoms such as pruritus can occur. There are complications, includes erythema and
secondary bacterial infection. Erythematous papules were found in the first patient and it is usually
caused by host reaction against viral infection and usually heals spontaneously. In the second patient,
we found pruritic lesion that often caused autoinoculation. Both of patients showed atypical distribution
of molluscum contagiosum lesion that localized only on anogenital area. MCV transmission possibly
caused direct contact, fomites, and autoinoculation. Furthermore, the usage of public swimming pool
has been accepted as one of risk factor for MCV transmission.
Keywords: molluscum contagiosum, anogenital lesion, children.

Poster: VII-10059
Abstract ID: BCI-10011
Track: Bacterial Infections
Contact Author: Thigita Aga Pandaleke
Country: Indonesia
Dermatovenereology department of Medical Faculty,

University of Sam Ratulangi, Manado-Indonesia
Poster: View Here
STAPHYLOCOCCAL SCALDED SKIN SYNDROME :

THREE CASES REPORT
Background: Staphylococcal Scalded Skin Syndrome (SSSS) is a potentially life threatening and occurs
predominantly in infants and children under 5 years of age. The phage group II staphylococci as a
dominant etiology particularly strains 52, 55 and 71. Cutaneous eruption such as macular erythematous
rash which develop to blisters and erosions in 24 – 48 hours. The eruption is accentuated in flexures
as well as trauma prone areas. Early diagnosis and adequate treatment may prevent complications.
Case: Three cases of SSSS in infants aged 11 days, 12 days, and 8 months were reported with
established diagnoses based on anamnesis, physical examination, and laboratory findings. Culture
examinations of the suspected focus infection revealed Staphylococcus Aureus.
Discussion: In SSSS, early recognition of this condition should be prompt. These three cases were
given intravenous and oral antibiotics along with other supportive therapies such as fluid, emollients
and dressings for lubrication and pain relief. All three cases showed a good response to treatment.
Poster: BCI-10011
STAPHYLOCOCCAL SCALDED SKIN SYNDROME : THREE CASES REPORT
Thigita Pandaleke, Ferra Mawu, Herry Pandaleke

Dermatovenereology Department of Medical Faculty Samratulangi University
Prof. dr. R.D Kandou Hospital Manado
Introduction
Staphylococcal Scalded Skin Syndrome (SSSS) potentially
life threatening and occurs predominantly in infants and
children under 5 years of age. It is cause predominantly by
phage group II staphylococci, particularly strains 52, 55 and
71. Cutaneous eruption such as macule erythemathous, in
24 – 48 hours established blisters and erosions. The
eruption is accentuated in flexural and the areas often
affected by trauma. The adequate therapy can prevent
complications.
Case 1. An infant 11 days
Cases
Clinical manifestation at these 3 cases showed rash
characterized by erythema that progresses to large, with
superficial fragile blisters that rupture easily, leaving
descuamation. The eruption involve the entire surface area
of skin. The Nikolsky sign(+). Gram stain and culture
founded coccus and staphylococcus aureus. These 3 cas-
es showed leukocytosis.
Discussion
Predisposing factors of SSSS such as renal immaturity,
leading to decreased toxin clearance, local infection at the
respiratory tract, ear, conjungtiva and umbilicus. The Case 2. An infant 12 days
eruption is most marked in flexural areas, but may involve
the entire surface area of skin. Management of SSSS
should be directed towards eradication of staphylococci
from the focus of infection. Usually, antibiotic therapy can
be substituted within several days. In addition to antibiotics,
topical supportive skin care and appropriate attention to
fluid and electrolyte management. In these 3 cases have a
good respon with antibiotic clindamycin and topical therapy
with fusidic acid or silver sulphadiazine, where these 2
topical treatment is bacteriostatic and effective against
staphylococcus aureus and strains methicillin resistant. Case 3. An infant 8 days
Reference
1. Paller AS, Mancini AJ. Staphylococcal Scalded Skin Syndrome. Dalam: Bonnet C, Gabbedy R, Mortimer A, Lowson K, Harrison R, penyunting. Hurwitz Clinical Pediatric
Dermatology; edisi ke-4. Beijing: ElSevier Saunders Inc;2011.h.331-3
2. Millett CR, Heymann WR, Manders SM. Pyodermas and Toxin-mediated Syndromes. Dalam: Irvine AD, Hoeger PH, Yan AC, penyunting. Harper’s Textbook of Pediatric
Dermatology;edisi ke-3, volume 1: West Sussex: Blackwell Publishing Ltd;2011.h.54.8-9.
3. Travers BJ, Mousdicas N. Gram-Positive Infections Associated with Toxin Production. Dalam : Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ,
penyunting. Fitzpatrick’s Dermatology in General Medicine; edisi ke-8. New York: Mc Graw Hill Inc; 2012.h.2148-56
9th RSMPD 2018

“Regional Scientific Meeting of Paediatric Dermatology”
Singapore
Contact Author: Sheau Szu Heah
Country: Malaysia
Institut Pediatrik, Hospital Kuala Lumpur, Malaysia
Poster: View Here
TWO CASE REPORTS OF PAEDIATRIC LEPROSY IN INSTITUT PEDIATRIK, HOSPITAL KUALA

LUMPUR
Leprosy is a chronic granulomatous disease caused by infection by mycobacterium leprae, an acid fast
bacilli. It affects 2 per 10,000 population worldwide, 10% of which in children below 15 years old, mostly
paucibacillary type. The mode of transmission is still uncertain but postulated to spread from person to
person in nasal droplets.
We report two cases of 9 year-old Malaysian girls from urban area who were diagnosed of leprosy in
Institut Pediatrik, Hospital Kuala Lumpur.
The first child presented with 3 years history of progressive anaesthetic skin rash and coarse facies,
subsequently a skin biopsy and slit-skin-smear(SSS) examination showed lepromatous leprosy. She
did not have any obvious contact history. The second child was diagnosed following a family screening
after the mother was diagnosed of lepromatous leprosy with Lucio phenomenon. She presented with
multiple hypopigmented skin rash with thickened peripheral nerve and failure to thrive. Though her SSS
was negative, her skin biopsy showed borderline tuberculoid leprosy.
Both were started on WHO MDT regime. While the first child is recovering with good adherence to
medications and regular follow-up; the second child had problem with treatment adherence due to
underlying poor socioeconomic support and needed to be referred to nearest health facility for
subsequent monitoring.
Though not common, leprosy in children is still a problem in Malaysia. A high clinical suspicion and
physicians’ awareness are important to not miss the diagnosis of this treatable, contagious and
debilitating disease. Good social and health support are important to ensure adherence and successful
treatment of childhood leprosy
Poster: BCI-10068
TWO CASE REPORTS OF PAEDIATRIC LEPROSY IN

INSTITUT PEDIATRIK, HOSPITAL KUALA LUMPUR
SS Heah, Sabeera BKI
Paediatric Dermatology Unit, Paediatric Institute, Hospital Kuala Lumpur, Malaysia.
INTRODUCTION
.
Leprosy is a chronic granulomatous disease caused by infection by mycobacterium leprae, an acid fast bacilli. The mode of transmission is still uncertain but postulated to
spread from person to person in nasal droplets. It affects 3 per100,000 population worldwide, 10% of which in children below 15 years old, mostly paucibacillary type. In
Malaysia, though leprosy incidence is low at 0.66 per 100,000 population (year 2016), paediatric leprosy constituted 5.2% of all new cases, mostly from rural area. We report two
cases of urban children being diagnosed of leprosy in Institut Pediatrik, Hospital Kuala Lumpur.
CASE 1 CASE 2
RS was a 9 year old girl presented with a left elbow rash for past three years and loss NMK was a 9 year-old girl referred together with her 3 other siblings for contact
of sensation for 1 year. There was no loss of power or function in the left arm. She screening after their mother was diagnosed of lepromatous leprosy. The mother
was also noted to have progressively thickened chin and earlobes. No family member developed lepromatous leprosy during peripartum period of her recent fifth
had similar symptoms. She stayed in a crowded 3-bedroom flat with 8 family childbirth. NMK had been having some scaly skin rash for the past 6 to 9 months but
members. Her mother was the sole bread-winner for the family working as a petty did not seek any medical attention. She had also not been gaining weight for the
trader. past one year. Otherwise there was no fever, painful skin swelling or weakness
Clinical examination revealed a normal built child with a coarse facies- flattened nasal noted. Her family of six stayed in a 2 bedrooms house with another 4 paternal
bridge with thick ala nasi, prominent supraorbital ridge, indurated nodular chin, thick extended family members, and had problems of poverty, unemployment and
nodular earlobes and loss of eyebrows laterally(Fig.1). There was an ill-defined substance abuse. It was unsure how the family
. was exposed to leprosy contact.
hypopigmented patch on her elbow without sensation(Fig.2).The hands and feet were On examination, the girl was obviously underweight (<3rd centile),malnourished and
mildly edematous (Fig.3). The peripheral nerves were not thickened nor tender. Other unkempt. There were multiple hypopigmented macules , patches and plaques on
neurological and systemic examinations were normal. her face, trunk and limbs (Fig. 7 and Fig.8). She had thickened ulnar nerves (Fig. 9)
and a hypoaesthetic patch on her back. There was however no motor weakness.
Cranial nerves examination was normal.
Fig.1 Before Fig. 2 Fig. 3 Fig 4 After
A skin biopsy over the elbow lesion showed sheets of foamy macrophages in the Fig.7 Fig.8
dermis without epithelioid cells. Wade-Fite stain showed numerous bacilli. A slit skin Slit skin smear from 6 body sites did not yield any
smear (SSS) showed bacteriological index(B.I.)1.8 and morphological Index (M.I.) Fig.9 Palpation of peripheral
finding. However a skin biopsy over her shin lesion nerves in leprosy
4.5(Fig.5) Therefore a diagnosis of lepromatous leprosy was made. confirmed tuberculoid leprosy.
She was started on WHO Multi-Drug Therapy (MDT) Multibacillary regime
consisting of Rifampicin, Clofazimine and Dapsone for 12 months. Fig.4 shows the She was started on MDT Paucibacillary regime (Fig.10) with adjusted dose
gradual resolution of coarse facies though she developed clofazimine-induced according to body weight. Her medication adherence and disease progress
hyperpigmentation. Directly-Observed-Treatment (DOT) method monthly ensured monitoring were challenging with frequent default due to underlying poor socio-
good treatment adherence. Contact screening was done. A repeat SSS 2 months economic support. Her other siblings were negative for leprosy but remained at
later showed M.I. 0 and B.I. 4.7. The MDT regime was tolerated well. (Fig. 6) high risk.
Fig.11 WHO Cardinal Signs for the Clinical Diagnosis of

Leprosy (any one)
Hypopigmented or slightly erythematous macules
with evident sensory loss
Thickened peripheral nerves
Positive acid-alcohol-fast smear or skin biopsy
Fig. 12 WHO Classification of Leprosy Clinical Spectrum
Fig. 5 Slit skin smear showing Fig. 6 Fig.10 Description Paucibacillary (PB) Multibacillary (MB)
Rifampicin 10mg/kg
dense collection of acid fast bacilli * Skin Lesion Less than 6 lesions Six or more lesions
Dapsone 2mg/kg
in clumps called globi (arrow)
Slit skin smear Negative Positive
* Used when slit skin smear and biopsy are not available
DISCUSSION & CONCLUSION

The above cases illustrate the clinical features of lepromatous and tuberculoid leprosy in two urban poor children. An uncommon disease in children nowadays, leprosy
infection is still present and frequently under-diagnosed, as the clinical features develop insidiously and can mimic other conditions like tinea skin infection. Clinical
manifestations in children are typically subtle and mild resulting in a delay in seeking medical attention too. Nevertheless, a high clinical suspicion of the cardinal signs (Fig.11)
with slit skin smear or skin biopsy can help in diagnosis and management.
The first case child had no contact history while the second case probably was infected by the mother. This shows the importance of effective contact tracing and screening.
As the incubation period of leprosy can range from a few months to more than 20 years, contact history might not be remembered.
The individual immune status determines the different clinical spectrum of leprosy, with intense cell-mediated immunity presenting at the tuberculoid pole and absent specific
immune response at the opposite lepromatous pole. Newer studies have shown possibility of genetic susceptibility to leprosy which might explain the absence of disease in
other children of the above two families. Though WHO classification of leprosy clinical spectrum (Fig.12) allows the disease to be classified without microscopic examination ,
the use of slit skin smear and skin biopsy made the treatment choice clearer.
Overcrowding, poor hygiene, malnutrition and prolonged close contact became important risk factors in both cases. This reminds us of the issues facing our urban poors
especially the most vulnerable group of children. In fact, poor socio-economic support had rendered difficulty in monitoring treatment progress and drug adherence in the
second case. A monthly DOT method is important throughout the long duration of MDT regime (Fig.6 & 10). Furthermore with completion of treatment, long term surveillance
is needed to avoid relapse. Therefore, concerted effort from all quarters namely the policy-makers, public health office, social welfare service, cannot be overemphasized
REFERENCES
1. TB/ Leprosy Control Unit, Ministry of Health Malaysia 3. Eichelmann K, et al. Leprosy. An update: Definition, pathogenesis, classification,
2. WHO Weekly epidemiological record diagnosis, and treatment . Actas Dermosifiliogr.2013;104:554-63
Contact Author: Mila Darmi
Country: Indonesia
Medical Faculty of Universitas Sumatera Utara / H.
Adam Malik General Hospital Medan
Poster: View Here
Erythema Nodosum Leprosum In Children
Background : Leprosy is a chronic infectious disease caused by Mycobacterium leprae which could
occur in any age groups. Erythema Nodosum Leprosum (ENL) is a leprosy reaction characterized by
red and painful nodules with fever that can occur before leprosy is diagnosed, but mostly during the
course of treatment and after treatment. Impairment in neurologic function and other organ systems
may be involved including arthritis, iridocyclitis, orchitis, and etc. Reactions are much less frequent in
childhood than in adults. It is probably due to lower incidence of nerve involvement.
Case Presentation : A 16 year old male with chief complain of red patches and painful nodules over his
face and extremities along with fever and arthritis of knee for a duration of 2 weeks. Initially there were
anaesthetic red patches appeared on his upper left arm and lower right leg since 7 years ago. Then
they spread eventually but he did not seek any medical attention and ignored those rashes.
Dermatological examination revealed multiple anaesthetic red patches and painful nodules over his
face and extremities. Voluntary muscletTest showed right fifth finger muscle weakness. Claw hand were
found as well. Slit skin smear showed a Bacterial Index of 2+. CBC showed leucocytosis (25.100
units/ÂµL). Renal and liver function test were within normal range. Immunoserological examination
showed total IgE of 722 IU/mL, ANA Test of 24.1 IU/mL, and normal Anti ds-DNA, Quantitative CRP,
and Rheumatoid Factor. Knee and Chest X-Ray showed no abnormality. This patient was diagnosed
with Multibacilar Leprosy with ENL. The patient was treated with WHO Multidrug Therapy (MDT) based
on age and prednisone with an initial dose of 40 mg.
Discussion : The diagnosis was made based on cardinal signs and laboratory findings. Leprosy
reactions are acute episodes of inflammation that commonly occur in leprosy patient, although it rarely
found in children. MDT for multibacilar leprosy consists of rifampicin 600 mg/month, Clofazimine 300
mg/month and 50 mg/day, and Dapsone 100 mg/day for 12 months. The reaction is managed with initial
dose of 40 mg Prednisone. Early diagnosis and prompt treatment will prevent deterioration of current
deformities and development of new deformities.
Poster: BCI-10069
Contact Author: Ramona Dumasari Lubis
Country: Indonesia
faculty of medicine university of sumatera utara/ H.
Adam Malik General Hospital Medan
Poster: View Here
BORDERLINE TUBERCULOID LEPROSY WITH TYPE I LEPROSY REACTION IN CHILDREN

ONSET
Abstract
Introduction : Leprosy is a chronic infection due to Mycobacterium leprae that affects skin and nerves
in all ages. In children, it affects those usually between 5-14 years-old in leprosy endemic countries.
The proportion of children leprosy in Southeast Asia region ranges from 2.99% in Thailand to 11.4%
in Indonesia. Global leprosy prevalence was 0.2 in 10,000 , almost five times higher in Indonesia.
Case : A 12-year-old girl presented with gradual onset of enlarging white patches with loss of sensation
on her face, arms, legs since she was 1 year old Patient’s aunt, who was her childminder was treated
with leprosy 2 years ago. Physical examination was unremarkable and no evidence of peripheral nerves
enlargement. On dermatologic examination, multiple irregular large anaesthetic hypopigmentation
macules were seen on the face, upper arms, and thighs. On slit skin smear, acid-fast bacilli (+1) was
positive. Patient was diagnosed with multibacillar leprosy and treated with child multi-drug-therapy
(MDT)-MB and vitamin-B twice/daily. After 2-months of MDT-MB treatment, patient noticed the macules
became red with some skin thickening, but no numbness or peripheral nerves enlargement. On further
dermatologic examination, scaly plaques were seen on on previous macular rash. Patient was
diagnosed with type I leprosy reaction and treated with MDT-MB and 30 mg/daily prednisone.
Discussion : Accurate diagnosis of leprosy in children is very important, because of psychosocial impact
to family. Criteria for diagnosis of leprosy is based-on hypopigmentation/erythematous lesions,
peripheral nerves involvement, and acid-fast bacilli on slit skin smear. One of the most prominent
feature of borderline tuberculoid leprosy was susceptibility to type I reaction, characterized with rapid
changes from existed plaques to oedematous lesions with/without abrupt neuritis. This reaction could
lead to improvement/deterioration. MDT should be started/continued immediately to eliminate M.
Leprae and decrease antigenic load from skin and nerves. Prednisolone is still the main therapy for
reversal reaction, with initial dose 1-2mg/Kg/daily followed by slow tapering. In children, spontaneous
regression appeared in 33-75% cases. Prognosis of childhood leprosy is excellent and they respond
well to recommended therapy.
Poster:BCI-10070
Abstract ID RSMPD03
Track Bacterial infections
Contact author IGAA Praharsini
Country Indonesia
Department of Dermatovenereology Faculty of Medicine

Organisation University of Udayana, Sanglah General Hospital,
Denpasar, Indonesia
Poster: View Here
STAPHYLOCOCCAL SCALDED SKIN SYNDROME IN CHILDREN AND NEONATES :

A CASE SERIES REPORT
Background: Staphylococcal Scalded Skin Syndrome (SSSS) is a bullous disease of the skin caused
by toxin secreted by certain Staphylococcus aureus. Although rarely occurs, it is commonly experienced
by neonates and can cause serious morbidity. This case report highlights SSSS and its challenges in
diagnosis and treatment.
Case I: A-2 year old girl came with the complaints of clear blisters on her face and body since 1 day
before hospitalization, initially started with redness on the skin of the neck, around the mouth and body
parts. There was history of sore throat 1 week before lesions appeared. On physical examination, we
found multiple tissue paper-like erosions. Positive Nikolsky's sign was also found, as well as
desquamation and multiple erosions in the area around the mouth. Tzanck test revealed acantolytic
cells and gram positive coccus in the gram stain. Laboratory test showed leukocytosis. Management
included NaCl intravenously, amoxicillin and clavulanic acid, and paracetamol syrup. Lesions were
treated with paraffin gauze dressing and fusidic acid cream.
Case II: A 10 day-old neonate complained skin peeling since 3 days before hospitalization. It initially
began with easily-broken blisters on the chin spreading to the chest, back, inguinal, arms and legs with
history of diarrhoea 3 days ago. On physical examination, multiple erosions and exfoliation were found.
Nikolsky's sign was positive. We also obtained multiple loose-walled bullous containing yellowish liquid
on the chest, right and left arms. Gram stain found leucocytes. There was growth of S.aureus in culture.
Patient was treated with cefotaxime intravenously, mupirocin ointment topically, and open dressing with
NaCl 0.9%.
Discussion: Both diagnoses were established through anamnesis, physical examination, and additional
investigations. Treatments were aimed to eradicate bacterial infections with intravenous antibiotics
administration. Fluid monitoring and skin care were essential to speedy healing. Successful results
were achieved in these cases due to timely and correct management.
Keywords: Staphylococcal scalded skin syndrome, children, neonates, therapy.

Poster: RSMPD03
STAPHYLOCOCCAL SCALDED SKIN SYNDROME

IN CHILDREN AND NEONATUS : A CASE SERIES REPORT
Praharsini IGAA, Karmila IGAA Dwi, Dewi Angeline
Department of Dermatology and Venerology of Udayana University Faculty of Medicine/ Public General
Hospital of Sanglah Denpasar
Background:
Staphylococcal Scalded Skin Syndrome (SSSS) is a bullous disease of the skin caused by toxin secreted by certain
Staphylococcus aureus. Although rarely occurs, it is commonly experienced by neonates and can cause serious
morbidity. These case report highlights SSSS and its challenges in diagnosis and treatment.
Case I: INITIAL PRESENTATION

A-2 year old girl came with the complaints of clear blisters on her
face and body since 1 day before hospitalization, initially started
with redness on the skin of the neck, around the mouth and
body parts. There was history of sore throat 1 week before
lesions appeared. On physical examination, we found multiple
tissue paper-like erosions. Positive Nikolsky's sign was also
found, as well as desquamation and multiple erosions in the area
around the mouth. Tzanck test revealed acantolytic cells and 10 DAYS AFTER THERAPY
gram positive coccus in the gram stain. Laboratory test
showed leukocytosis. Management included NaCl intravenously,
amoxicillin and clavulanic acid, and paracetamol syrup. Lesions
were treated with paraffin gauze dressing and fusidic acid cream.
INITIAL PRESENTATION
Case II:
A 10 day-old neonate complained skin peeling since 3 days before
hospitalization. It initially began with easily-broken blisters on the chin
spreading to the chest, back, inguinal, arms and legs with history
of diarrhoea 3 days ago. On physical examination, multiple erosions
18 DAYS AFTER THERAPY
and exfoliation were found. Nikolsky's sign was positive. We also
obtained multiple loose-walled bullous containing yellowish liquid on
the chest, right and left arms. Gram stain found leucocytes. There was
growth of S.aureus in culture. Patient was treated with cefotaxime
intravenously, mupirocyn ointment topically, and open dressing with
NaCl 0.9%.
Discussion:
Both diagnosis were established through anamnesis, physical examination, and additional investigations.
Treatments were aimed to eradicate bacterial infections with intravenous antibiotics administration.
Fluid monitoring and skin care were essential to speedy healing. Successful results were achieved in
these cases due to timely and correct management.
Reference :
1. Jeffrey B. Travers, Nico Mousdicas. Gram-Positive Infections Associated with Toxin Production. In : Klaus Wolff, Lowell A
Goldsmith, Stephen I Katz, Barbara A Gilchrest, Amy S Paller, David J Leffell. Eds. Fitzpatrick’s Dermatology in General
Medicine. 7th ed. New York : McGraw-Hill; 2007. p1710-1714
2. Mueller E, Haim M, Petnehazy T, Roschitz BA, Trop M. An Innovative Local Treatment for Staphylococcal Scalded Skin
Syndrome. Eur J Clin Microbiol Infect Dis. 2010: 29; 893-97.
3. Randall W King, Paul R de Saint, Staphylococcal Scalded Skin Syndrome; eMedicine Emergency Medicine from WebMD.
http://www.eMedicine-Article, Last updated May 4th 2010
Abstract ID RSMPD14
Contact author Dia Febrina
Country Indonesia
Organisation Hasan Sadikin Hospital
Poster: View Here
METASTATIC TUBERCULOUS ABSCESS THAT DIFFICULT TO DISTINGUISH FROM

SCROFULODERMA IN A CHILD WITH PULMONARY AND SKELETAL TUBERCULOSIS
Abstract
Background: Cutaneous tuberculosis (CTB) is a chronic infection caused by Mycobacterium
tuberculosis (M. TB) with vary clinical manifestations. Metastatic tuberculous abscesses (MTA) or
tuberculous gummi is a rare form of CTB, result from the disseminated haematogenous spread of non-
cutaneous primary focus, usually from lung. Clinical manifestation of MTA is similar to scrofuloderma,
presented as single or multiple nodules which may become abscess and break down to form ulcer and
sinuses.
Case: We reported a case of MTA presented as multiple nodules on left dorsal of hand and feet which
became abscesses and broke down to form ulcers in 9-year-old malnourished girl. There was history
of blunt trauma on left elbow before skin lesion appeared, accompanied by weight loss, and history of
close contact with TB patient in her family. The left elbow had become swollen and painful. Chest
roentgenogram showed active pulmonary tuberculosis. The roentgenograms of the left proximal ulna,
fifth metacarpal and fifth metatarsal showed spina ventosa which was suggestive of osteomyelitis TB.
The diagnosis of MTA established based on clinical features, positive polymerase chain reaction and
GeneXpert for M. TB from lesional biopsy, and histopathological examination. The diagnoses of
pulmonary and skeletal TB were based on roentgenogram and positive tuberculin test. Treatment of
this patient was started on four antituberculosis drugs at doses adjusted for body weight consist of
rifampin (R); isoniazid (H); pyrazinamide (Z); and ethambutol (E) for two months, and then RH for 10
months. Ulcers were treated with hydrocolloid dressing for five months. After six months of treatment,
all lesions cleared leaving only some small scars and gained weight.
Discussion: MTA and scrofuloderma which presented as abscesses are difficult to distinguish clinically.
Therefore, definite diagnosis of CTB type requires careful history, detailed physical examination, and
further examinations to identify primary focus of infection. In our case, primary focus of TB were lung
and left elbow that distant from the site of lesions. It meant that skin lesions did not spread per continuum
as in scrofuloderma.
Keywords: metastatic tuberculous abscess, pulmonary tuberculosis, skeletal tuberculosis, tuberculous

gumma
Poster: RSMPD14
Abstract ID RSMPD17
Contact author Angeline Dewi
Country Indonesia
Department of Dermatovenereology, Faculty of
Organisation Medicine Universitas Udayana, Sanglah
Hospital, Bali, Indonesia
Poster: View Here
Borderline Tuberculosis Type of Modbus Hansen with Leprosy Disability Grade 2 in Children
Background:
Modbus Hansen (MH) or leprosy is a chronic granulomatous infection caused by Mycobacterium Larea,
which may affect the peripheral nerves, skin and other tissues. Borderline tuberculosis (BT) is the
common type of leprosy in children. Early and prompt treatment is needed to prevent disability in MH
patients.
Case:
An 11 year-old boy, a Balinese, came with a chief complaint of wound and bends in the fourth and fifth
fingers of his right hand. The wound initially appeared as a small lesion since a week prior to consult,
then being widened without any pain. His fingers bend since 6 months prior to consult, accompanied
with tingling sensation. Patient also complained of numbness white patches on his face, arms and legs
since a year. On physical examination, we found hypopigmentation macules with a decrease sensibility
of touch, temperature and pain. There was also erosion covered by brownish crust, contracture, and
ulnar nerve thickening in the fourth and fifth finger of right hand. Based on the history taking, physical
examination and histopathology, patient was diagnosed with BT type of MH with leprosy disability grade
2 and secondary infection. Patient was treated with paucibacillary multi drug therapy (MDT) for children,
B1B6B12 vitamin, paraffin gauze dressing and topical antibiotic. Consultation to rehabilitation medicine
and neurology division was planned.
Discussion
Children are more susceptible to MH because their immune system is not fully developed. Diagnosis of
MH is made based on cardinal signs of skin lesions, anaesthesia, nerve thickening at the predilection
site and the presence of acid-fast bacilli on slit skin smears. BT type is the most common type found in
children. Nerve damage in BT type is more prone to occur and may cause permanent disability. Besides
MDT, keeping the wound clean and avoiding trauma is necessary in the management of leprosy to
prevent further disability. Prognosis will depend on the spectrum of disease.
Keywords: Morbus Hansen, leprosy, disability, child

Poster:RSMPD17
Borderline Tuberculoid Type of Morbus Hansen

with Leprosy Disability Grade 2 in Children
Dewi Angeline 1 , Bya Margareth Juniawati2, Rusyati Luh Made Mas 3, Karmila IGAA Dwi4,
Department of Dermatovenereology, Faculty of Medicine Universitas Udayana,
Sanglah Hospital, Bali, Indonesia
Background:
Morbus Hansen (MH) or leprosy is a chronic granulomatous infection caused by Mycobacterium Leprae, which may
affect the peripheral nerves, skin and other tissues. Borderline tuberculoid (BT) is the common type of leprosy in
children. Early and prompt treatment is needed to prevent disability in MH patients.
Case : INITIAL PRESENTATION

An 11 year-old boy, a Balinese, came with a chief complaint
of wound and bends in the fourth and fifth fingers of his right
hand. The wound initially appeared as a small lesion since a
week prior to consult, then being widened without any pain.
His fingers bend since 6 months prior to consult,
accompanied with tingling sensation. Patient also complained
of numbness white patches on his face, arms and legs since a
year. On physical examination, we found hypopigmentation
macules with a decrease sensibility of touch, temperature
and pain. There was also erosion covered by brownish crust,
contracture, and ulnar nerve thickening in the fourth and Fig 1. Multiple hypopigmentation macules on the
fifth finger of right hand. Based on the history taking, physical face, upper extremities and right leg
examination and histopathology, patient was diagnosed with
BT type of MH with leprosy disability grade 2 and secondary
infection. Patient was treated with paucibacillary multi drug
therapy (MDT) for children, B1B6B12 vitamin, paraffin gauze
dressing and topical antibiotic. Consultation to rehabilitation
medicine and neurology division were planned.
Fig 2. Claw hand dextra and erosion covered by
brownish crust in the fifth finger of right hand
Histopathological examination 14 DAYS AFTER THERAPY
Fig 3.1. Two Groups of Fig 3.2. Granulomas are

granuloma appeared. composed of epithelioid Fig 4. Multiple hypopigmentation Fig 5. erosion
granuloma cells, with slight macules on the face, and upper become dry in the
invasion of lymphocytes in extremities . fifth finger
granulomas.
Reference :
Discussion: 1. Bryceson A, Platzgraff RE. Symtoms and
signs. In: Leprosy. 3rd ed. New York:
Children are more susceptible to MH because their immune system is not Longman Group Limited; 1990: 25-56.
fully developed. Diagnosis of MH is made based on cardinal signs of skin 2. Rea TH, Modlin RL. Leprosy. In: Wolff K,
lesions, anesthesia, nerve thickening at the predilection site and the Goldsmith LA, Katz SI, Gilchrest BA, Paller
presence of acid-fast bacilli on slit skin smears. BT type is the most AS, Leffell DJ, editors. Fizpatrick's
Dermatology in General Medicine. 7th ed.
common type found in children. Nerve damage in BT type is more prone New York: Mc Graw Hill companies, 2008;
to occur and may cause permanent disability. Besides MDT, keeping the 1786-96.
wound clean and avoiding trauma is necessary in the management of 3. Singal A, Sonthala S, Pandhi D. Childhood
leprosy to prevent further disability. Prognosis will depend on the leprosy in a tertiary-care hospital in Delhi,
India: A reappraisal in the post-elimination
spectrum of disease.
era. Lepr Rev. 2011; 82:259-69
Abstract ID RSMPD18
Contact author Angeline Dewi
Country Indonesia
Department of Dermatovenereology, Faculty of

Organisation Medicine Universitas Udayana, Sanglah
Hospital, Bali, Indonesia
Poster View Here
Reversal Reaction in Paediatric Patients with Modbus Hansen: A Case Series Report
Background:
Modbus Hansen (MH) is a chronic granulomatous infection caused by Mycobacterium Larea which may
affect the peripheral nerves and skin. Reversal reaction, a type of reaction in MH can also occur in
children and lead to disability.
Case 1:
An 11-years-old Balinese girl came with complaint of red patches on her face and body since a year
ago without any systemic complaint. Patients have never being vaccinated with BCG. Based on
anamnesis and other examinations, she was diagnosed with borderline lepromatous (BL) type of MH.
She was being treated with multidrug therapy (MDT) for multibacillary leprosy in children. A month later,
the old lesions became more reddish and thicker, with a sense of heat on palpation, and tingling in both
hands. There was no new lesion. Then she was diagnosed with BL type of MH with mild reversal
reaction. MDT was continued and Paracetamol was given if necessary.
Case 2:
A 10 years-old Balinese boy with a history of BT type of MH and currently in MDT-PB treatment came
because his old lesions become more reddish, thicker and feel warm. Patient also complained of some
new erythematous patches, face swelling, and fever since 3 days. He was diagnosed with BT type of
MH with severe reversal reaction. Management of this patient was bedrest, paracetamol as needed
and methylprednisolone 16mg once daily then tapered-off every 2 weeks. MDT was continued.
Discussion
Leprosy reaction is a common sign and symptom of acute inflammation in MH. Nerve tissue
inflammation in reversal reaction may lead to disability, so an early and prompt treatment is needed.
Mild reversal reaction can be treated symptomatically with analgesic, antipyretic, or sedative. While
severe reversal reaction may present new painful lesions and systemic symptoms such as fever and
malaise and need steroid to prevent further nerve impairment. MDT should be continued and triggering
factor should be avoided.
Keywords: Modbus Hansen, reversal reaction, leprosy, child

Poster: RSMPD18
Reversal Reaction in Pediatric Patients with Morbus Hansen:

A Case Series Report
Dewi Angeline1, Rusyati Luh Made Mas 2, Praharsini IGAA3
Department of Dermatovenereology, Faculty of Medicine Universitas Udayana,
Sanglah Hospital, Bali, Indonesia
Background:
Morbus Hansen (MH) is a chronic granulomatous infection caused by Mycobacterium Leprae which may
affect the peripheral nerves and skin. Reversal reaction, a type of reaction in MH can also occur in children
and lead to disability
INITIAL PRESENTATION Case 1:

An 11-years-old Balinese girl, came with complaint
of red patches on her face and body since a year
ago without any systemic complaint. Patients have
never being vaccinated with BCG. Based on
anamnesis and other examinations, she was
diagnosed with borderline lepromatous (BL) type of
MH. She was being treated with multidrug therapy
(MDT) for multibacillary leprosy in children. A
14 DAYS AFTER THERAPY month later, the old lesions became more reddish
and thicker, with a sense of heat on palpation, and
tingling in both hands. There was no new lesion.
Then she was diagnosed with BL type of MH with
mild reversal reaction. MDT was continued and
Paracetamol was given if necessary.
INITIAL PRSENTATION
Case 2:
A 10 years-old Balinese boy with a history of BT type of
MH and currently in MDT-PB treatment came because
his old lesions become more reddish, thicker and feel
warm. Patient also complained of some new
erythematous patches, face swelling, and fever since 3 14 DAYS AFTER THERAPY
days. He was diagnosed with BT type of MH with severe
reversal reaction. Management of this patient was
bedrest, paracetamol as needed and
methylprednisolon 16mg once daily then tapered-off
every 2 weeks. MDT was continued.
Discussion
Leprosy reaction is a common sign and symptom of acute
Reference :
inflammation in MH. Nerve tissue inflammation in reversal 1. Bryceson A, Platzgraff RE. Diagnosis. In:
reaction may lead to disability, so an early and prompt Leprosy. New York: Longman Group Limited;
treatment is needed. Mild reversal reaction can be treated 1990: 57-75.
symptomatically with analgetic, antipyretic, or sedative. While 2. Sehgal VN. Leprosy in children. In: Clinical
severe reversal reaction may present new painful lesions and Leprosy. New Delhi: Jaypee Brothers; 2014:
78-82.
systemic symptoms such as fever and malaise and need 3. Walker S, Lockwood D. Leprosy type 1
steroid to prevent further nerve impairment. MDT should be (reversal) reaction and their management.
continued and triggering factor should be avoided. Lepr Rev. 2015; 79: 372-86.
Abstract ID: FUI-10066
Track: Fungal Infections
Contact Author: Angie Regina Sutrisno
Country: Indonesia

Faculty of Medicine Universitas Sumatera Utara
Poster: View Here
Case Report: Black Dot Form Of Tinea Capitis In Children
Background : Tinea capitis is dermatophyte infection of hair and scalp that caused by Trichophyton and
Micropsorum species which usually occur in children. The clinical appearance of tinea capitis is
commonly characterized by alopecia with scaly, erythematous, and pruritic lesions.
Case Presentation : A 9 year-old girl came with alopecia a month ago. First it appeared as itchy
erythematous macule covered with scales. Patient had frequent contact with cats. There is no family
member suffered from the same condition. Dermatology examination, revealed broken hair covered
with scales on left parietal region with diameter of 5 cm. Microscopic examination of skin scrapings
using KOH 20% showed hyphae and spores. From the cultured specimen, we identified Trichophyton
mentagrophytes. We diagnosed the patient with a black dot form of tinea capitis and was treated with
griseofulvin 500 mg/day orally for 6-8 weeks and selenium sulfide 1% shampoo twice/week. After 1
week of therapy, we found less broken hair with new hair begun to grow back. We advised the patient
to continue the treatment and encouraged her for regular visit every 2 weeks.
Discussion : Tinea capitis can be transmitted through pets such as cats or dogs. Oral therapy, such as
griseofulvin, is recommended as a gold standard for the main treatment of tinea capitis. Application of
1% selenium sulfide shampoo were used to reduce further transmission of the spores and treatment of
tinea capitis should be in combination.
Keywords : tinea capitis, black dot, children

Poster: FUI-10066
Abstract ID: PAI-10085
Track: Parasitic Infections
Contact Author: Agassi Suseno Sutarjo
Country: Indonesia
Dermatology and Venereology Department, Faculty

of Medicine Universitas Indonesia
Poster: View Here
Crusted Scabies Presenting As Erythroderma
Background:
Scabies is an infestation of the skin by Sarcoptes scabiei, var. hominis. It is passed by direct, prolonged,
skin-to-skin contact with a person who is already infested or by indirect transmission. Known as the,
“great imitator,” scabies is frequently misdiagnosed because the conditions resembles other common
skin conditions. Crusted scabies, also known as Norwegian scabies, is a rare manifestation of scabies
characterized by an uncontrolled proliferation of mites in the skin. This severe condition is mainly
observed in children with immunosuppresion or patients with mental illness. Crusted scabies rarely
presents as erythroderma necessitating suspicions for its diagnosis.
Case presentation:
A-16-year-old girl was referred from rural area on Belitung Island with a 1-year history of progressive
erythrosquamous lesions, which was previously diagnosed as erythroderma caused by seborrheic
dermatitis. There was no history of preexisting dermatologic or medical conditions. She was treated
with potent topical corticosteroid for a long period. Physical examination revealed low nutritional status
and extensive non-uniform erythematous scaly patches involving her entire skin surface and white-
yellowish crust on the head, trunk, and extremities. Both the patient and her father complained of an
intense pruritus, especially at night. Physical examination of the patient and her father revealed
hyperpigmented papules disseminated mainly in extremities. Microscopic examination of skin scrapings
from the abdomen revealed eggs and mites. Diagnosis of a crusted scabies was made. The patient
was given permethrine 5% cream applied twice weekly.
Discussion:
Crusted scabies is a disease of immunocompromised hosts, meaning that it is a result of a compromise
of the immune system rather than the increased virulence of parasite. In this case, it was due to low
nutritional status and long term use of corticosteroid. History of iching in family and intense nocturnal
itching should raise suspicion of scabies Laboratory confirmation using skin scraping should be
obtained whenever possible to help confirm the diagnosis. It is important to note that an untreated case
of crusted scabies may be the source of an outbreak .
Keywords: crusted scabies, erythroderma, immunosuppresion

Poster: PAI-10085
Abstract ID RSMPD05
Track Parasitic infections
Contact author Djohan Kho
Country Indonesia
Organisation Adam Malik General Hospital / FK USU
Poster: View Here
CUTANEUS LARVA MIGRANS IN INFANT SUCCESFULLY TREATED WITH TOPICAL

ALBENDAZOLE
Introduction : cutaneous larva migrans (CML) is a parasitic skin disease caused by the migration of
hookworm larvae in the epidermis of human skin. Transmission occurs when naked skin contact with
contaminated soil. The diagnosis is essentially clinical. It is supported by a recent travel history and
exposure to soil. The clinical sign is a small reddish papule followed by serpiginous, slightly elevated,
erythematous track moving forward in the skin. Itching becomes more intense at night. The drug of
choice is ivermectin in single dose. Treatment with albendazole are a good alternative in countries when
ivermectin not available.
Case : A 10-months-old girl patient, came with erythematous papules, serpiginous on the right foot
since 3 days ago. The initial lesion was a small papule on the right sole which evolved to become an
erythematous serpiginous rash tracking upwards. She played barefooted on the beach 1 week ago. In
dermatological examination, a single serpiginous, erythematous track with papules on the right plantar
pedals these patient treat with topical albendazole then close with occlusive tape 3 times a day shows
improvement.
Discussion : Cutaneous larva migrans is one of the most common skin diseases which occurs when
naked skin contact with contaminated soil. The pruritic serpiginous track is pathognomonic. Ivermectin
and albendazole are orally contraindicated in infant and topical treatment must be considered. In this
patient treatment with topical application of albendazole is considered safe treatment for cutaneous
larva migrans in infant and proven to be effective.
Poster: RSMPD05
Abstract ID: SDI-10043
Skin Disorders of Immunodeficiency (primary or

Track:
acquired)
Contact Author: Youjia Zhong
Country: Singapore
Organisation: National University Hospital
Poster: View Here
Hypersensitivity To Mosquito Bites As A Presentation Of Epstein-Barr Virus (EBV) Associated

NK-cell Lymphoproliferative Disorder
Case:
We present a 14-year-old girl with hypersensitivity to mosquito bites (HMB) who was diagnosed with
EBV-LPD. Our patient had a history of HMB since three years of age with fevers developing twelve
hours after mosquito bites, lasting two to three days associated with skin blistering, subsequent necrosis
and ulceration with eventual scar formation. She presented to us with worsening of hypersensitivity
reactions over the preceding 6 months, with more rapid onset and higher peaks of fever up to 40
degrees Celsius, associated with more severe cutaneous ulcerations. She was otherwise healthy, with
normal growth and development. Clinical examination revealed necrotic ulcers, partially-healing lesions
and old atrophic scars on her lower limbs. There was no hepatosplenomegaly or lymphadenopathy.
Investigations revealed EBV viraemia with evidence of previous EBV infection on serological testing as
well as elevated transaminases and high total IgE levels. Full blood count was normal but lymphocyte
subsets by flow cytometry showed NK cell lymphocytosis which was supportive of the diagnosis of EBV-
LPD, NK-cell type. She has been started on low dose everolimus while awaiting a haematopoietic stem
cell transplant.
Discussion:
HMB, which is rare and distinct from the more common (mosquito bite allergy), is characterised by
intense local cutaneous reactions accompanied by systemic symptoms of fever, lymphadenopathy and
liver dysfunction and is a manifestation of EBV T/NK-cell LPD. Usually occurring in the first 2 decades
of life, the median age of patients with HMB was 6.7 years in one study.
Epstein-Barr Virus (EBV)-associated T/NK-cell lymphoproliferative disorders (LPD) occur almost

exclusively in East Asians. Instead of the usual B cell acting as a host for EBV, T and NK cells are
infected leading to clonal proliferation. Other manifestations include chronic active EBV (CAEBV), a
prolonged severe form of infectious mononucleosis, severe hydroa vacciniforme and haemophagocytic
lymphohistiocytosis. EBV T/NK-cell LPD predisposes to malignant transformation, especially T/NK-type
leukaemias and lymphomas which confer high mortality.
This is the first case reported in Southeast Asia. Dermatologists should have a high index of suspicion
for underlying lymphoproliferative disorder when a child develops systemic symptoms together with
severe local cutaneous reaction from mosquito bites.
Poster: SDI-10043
Hypersensitivity to mosquito bites as a presentation of Epstein-

Barr Virus (EBV) associated NK-cell lymphoproliferative disorder
Youjia Zhong*1, Patricia Ng2
1Khoo Teck Puat-National University Children’s Medical Institute, National University Health System,
Singapore 2Dermatology Associates, Singapore
History
We present a 14-year-old with hypersensitivity to Case: history Bone m arrow
transplant
mosquito bites (HMB) who was diagnosed with EBV
associated lymphoproliferative disorder (LPD). She had 13 years old:
M odified H LH -2004
protocol
a history of HMB since three years of age, with 6-12 months
before
Presented at
13 years old May to Dec 2018 27 Jan 2018
increasing intensity over the preceding 6 months. She 2-3 years old presentation (Apr 2017)
was one of a pair of identical twins, and was otherwise

healthy, with normal growth and development. Fever 2-3 days
Continues to
after mosquito Fever higher 1.9cm x 1.3cm Fever with
T40C, few hours have fever hepatospleno
bite (T38C, 12h) deep ulcerated
Examination lesion (most
with mosquito megaly
Bite bites
Bite à darken
Clinical examination revealed necrotic ulcers, partially- à black scab à blister /
severe episode
of ulceration Fevers w ith U RTIs
healing lesions and old atrophic scars on her lower limbs. à atrophic scar
in 2-3 weeks
necrosis
à à scab
thus far) treated w ith
dexam ethasone
(See photos below) There was no hepatosplenomegaly or Trial of everolim us
lymphadenopathy. (N ov-D ec 2017)
IL-2-expanded NK cells: EBV detection by Western Blot (LMP1)
unstim IL-2 IL-2 + IFN-a

Investigations
Jurkat (EBV-)
Unrelated ctrl
Unrelated ctrl
Unrelated ctrl
LCL (EBV+)
Related ctrl
Related ctrl
Related ctrl
Hb 13.5 WBC 7.29 neutrophils 3.2

Patient
Patient
Patient
lymphocytes 3.4 Platets 239

AST 63 ALT 79 Non-specific band (in EBV- Jurkats)
Total IgE 1789 IgG / M / A normal LM P1 Specific band (absent in EBV+ Jurkats)
EBV DNA 1.1E+03 copies DNA/ml plasma Actin

EBV VCA IgM negative, IgG positive
Lymphocyte subsets: NK cell 1929 /mm3 EBV latent membrane protein-1 (LMP-1) is expressed specifically in patient’s
(275-456), T / B normal expanded NK cells using Western Blot. The co-staining of EBV LMP-1 on NK cells
LMP1 is detectable by WB: Jurkat negative control and LCL positive control is clear.
confirms that
Upper band there is EBV
is non-specific band,infection
lower bandin NK cells
is LMP1 band.in our patient.
Patient progress LMP1 is present in Patient but not in controls in all three conditions.
Our patient was trialed on a low dose of everolimusLMP1
without effect.
expression While
is higher awaiting
in IL-2 stimulatedhaematopoietic stem cells.
and IL-2 + IFN-a stimulated cell transplantation, she
developed haemophagocytic lymphohistiocytosis (HLH) with fever, hepatosplenomegaly, thrombocytopenia,
hypertriglyceridaemia, and transaminitis. She was started on HLH-2004 protocol with corticosteroid, cyclosporin and etoposide.
Discussion
HMB, which is rare and distinct from the common “mosquito bite allergy”, is characterized by intense local cutaneous reactions
accompanied by systemic symptoms of fever, lymphadenopathy and liver dysfunction. It is a manifestation of T / NK-cell
lymphoproferative disorder.
EBV-LPD occur almost exclusively in East Asians (mainly Japan and Taiwan), and usually occurs in the first two decades of life. EBV
infects T or NK cells, leading to clonal proliferation. [1] Patients are predisposed to malignant transformation, especially to T/NK-
type leukaemias and lymphomas which confer high mortality. [2]
This is the first case reported in South East Asia. Dermatologists should have a high index of suspicion for underlying
lymphoproliferative disorder when a child develops systemic symptoms together with severe local cutaneous reaction from
mosquito bites.
References:
[1] Qingqing Cai, Kailin Chen, Ken H Young. Epstein-Barr virus-positive T/Nk-cell lymphoproliferative disorders. Exper Mol
Med 2015 (47): 1-13
[2] Hiroshi Kimura, Yoshinori Ito, Shinju Kawabe et al. EBV-associated T/NK-cell lymphoproliferative diseases in
nonimmunocompromised hosts: prospective analysis of 108 cases. Blood 2012; 119(3):673-686
Copyright © 2018 The Authors. *Corresponding Author: Zhong Youjia, Tel: +65 67795555 Email: youjia_zhong@nuhs.edu.sg
Abstract ID: VTM-10014
Track: Vascular Tumours and Malformations
Contact Author: Dayang Siti Yuhana Ag Mat Yusof
Country: Malaysia
Queen Elizabeth Hospital, Sabah, Malaysia
Poster: View Here
A Single Centre Experience With Oral Sirollimus For The Treatment Of Complex Vascular
Anomalies.
Introduction
Complex vascular anomalies pose a challenge in management and may become life threatening. In
recent years, sirolimus has become the drug of choice in the treatment of complex vascular anomalies
in the paediatric age group. Here we present our experience using oral sirolimus to treat paediatric
patients with different vascular anomalies.
Methods
We did a retrospective review of our paediatric patients with complex vascular anomalies between 2015
and now, who were treated with oral sirolimus 0.8mg/m2/dose twice daily. The target trough level was
10-15ng/mL.
Results
Four patients were included in this case series: a 7-month old male with Kaposiform
hemangioendothelioma (KHE), a 10-month old male with KHE, a 13-year old male with Complex Slow
Flow Venous Capillary Lymphatic Malformation, and a 4-month old female with CLOVES syndrome.
Both children with KHE had presented with Kasabach Meritt Phenomenon(KMP) and was initially
treated with weekly IV Vincristine 0.05mg/kg however did not respond in a satisfactory manner. The
mean duration of oral sirolimus for the KHE patients was 10 months. KMP resolved within one month
of treatment with sirolimus. Both KHE patients achieved almost complete reduction in size of tumour
clinically and radiologically. The other two patients are still on oral sirolimus and have shown gross
reduction of the size of their tumours. The duration of oral sirolimus before clinical improvement was
seen was between 3-8 weeks. Oral sirolimus was tolerated well by our patients, 2 patients developed
reversible Grade 2 and Grade 4 neutropenia respectively.
Conclusion
Oral sirolimus is an efficacious and safe treatment for our patients with KHE and lymphatic dominant
complex vascular malformations. However standard dose and duration of treatment especially in the
very young age group need to be evaluated.
Poster: VTM-10014
Contact Author: Jenny Yen LingTan
Country: Malaysia
Poster: View Here
Facial Venolymphatic Malformation With Associated Developmental Venous Anomalies (DVA):

Case Report
Case presentation
We report a case of a 5 year old girl with extensive facial venolymphatic malformation and incidental
finding of developmental venous anomalies (DVA) in bilateral cerebral hemispheres. She has no
neurological symptom and her development is normal.
Discussion
Developmental venous anomalies have a remarkable prevalence of 20% in patients with extensive
facial venous malformations. Therefore, the initial imaging of these patients should include the brain to
search for cerebral developmental venous anomaly.
Poster VTM-10062
Facial Venolympha c Malforma on with associated

Developmental Venous Anomalies (DVAs): Case report
Jenny TYL1, Rohazly2, Leong KF1, Sabeera BKI1
1Department of Paediatric Dermatology, Institute Pediatric Hospital Kuala Lumpur
2Department of Radiology, Hospital Kuala Lumpur
Introduction
DVAs are medullary or cortical veins draining normal cerebral parenchyma. They usually represent an adaptation related to
absence of a superficial or deep collector. DVA, generally asymptomatic, is present in 20% of patients with cervicofacial venous
malformations.
Case report
A 5 years old girl was referred for huge swelling of the left fronto-
temporal region extending towards the left orbit causing proptosis and
facial asymmetry. The swelling was present at birth and gradually
increasing in size. She was initially treated as hemangioma and given
propanolol for 6 months but there was no improvement. Lesion
progressively increases in size causing difficulty in opening her left eye
and visual disturbance.
On examination, she has a huge swelling on her left forehead,
which is covering her left eye. The swelling was soft, non tender with no
thrill or bruit. There were no changes of the overlying skin.
Transillumination was negative.
Magnetic resonance imaging (MRI) studies (T2WI) demonstrated Le : Pa ent at 1 month old
a large heterogenous iso and hyperintense mass with presence of Right: Pa ent at 5 years old before star ng sirolimus
phleboliths in the left frontal region in keeping with venous malformation.
Post gadolinium images showing heterogenous enhancement of the left
frontal venous malformation. Enhancing linear striations are seen within
the cerebrum on both sides, more prominent on the left side in keeping
with developmental venous anomalies (DVA).
Sclerotherapy cannot be done as the lesion is compact, hence
not suitable for sclerotherapy. She is currently on oral sirolimus and noted
to have some improvement clinically. A repeat MRI will be done after 6
months of oral sirolimus to monitor her progress.
Discussion
DVAs, previously termed venous angiomas are said to be the
most common anomaly of the intracranial vasculature. Cerebral deep
venous angiomas are frequently found in patients with vascular lesions of Le : T1WI axial. Right: T1 post gadolinium axial
face orbit, including arterio-vascular malformations (AVM), capillary Post gadolinium images showing heterogenous enhancement of
malformations, venous vascular malformations, orbital venous varices, the le frontal venous malforma on. Enhancing linear stria ons
sinus pericranii and some vascular tumors e.g. hemangio-lymphangioma. are seen within the cerebrum on both sides, more prominent on
In patients exhibiting cervicofacial venous malformation 20% have the le side in keeping developmental venous anomalies (DVA)
concurrent cerebral DVA.
DVA is usually an incidental finding but rarely can be associated
with hemorrhage, headaches or other neurologic symptoms. The clinical
significance of DVA lies primarily in recognizing this entity as a variation of T1 post gadolinium
normal rather than a pathological process to prevent iatrogenic coronal.
exacerbation of symptoms. Because hemorrhage is not a clinical concern Enhancing linear
stria ons represen ng
and the venous structures drain normal brain parenchyma, no further
DVAs are seen in
therapy surgical or intervention is warranted. The other concern of DVA is bilateral temporal lobes
that planned or inadvertent occlusion during treatment of associated and le parietal lobe.
lesions frequently leads to venous infarction of the surrounding normal No ce also the dilated
brain. Therefore, its important to look for presence of DVA before le cor cal vein due to
intervention of associated lesion. increased venous
drainage.
Conclusion
Developmental venous anomalies have a remarkable prevalence of 20%
in patients with extensive facial venous malformations. Therefore, the
initial imaging of these patients should include the brain to search for
cerebral developmental venous anomaly.
References
1. Boukobza M, Enjolras O, Guichard JP, Gelbert F, Herbreteau D, Reizine D, Merland JJ: Cerebral developmental venous anomalies associated with head and neck venous
malforma ons. Am J Neuroradiol 17: 987–994, 1996.
2. Pryor J, Se on A, Bernstein A. Venous anomalies and associated lesions. Neurosurg Clin North Am 1999;10:519-23.
Abstract ID: BMT-10065
Track: Other Benign and Malignant Tumours
Contact Author: Widyaningsih Oentari
Country: Indonesia
Department of Dermatology and Venereology,
Faculty of Medicine Universitas Sumatera Utara
Poster: View Here
Case Report: Giant Congenital Melanocytic Nevus In An Infant
Background
Giant congenital melanocytic nevus (GMCN) is benign tumor of nevus cells, which presented at birth
with largest diameter of more than 20 cm in adulthood. It is often characterized by brown lesion with flat
or mammillated surface, well-demarcated borders and hypertrichosis. It is considered as a rare case
and tends to be seen in female. This condition is associated with increased risk of malignant melanoma
and neurocutaneous melanoma (NCM) that have poor prognosis. There are several options of
treatment but it should be adjusted individually.
Case presentation
We presented a case of GMCN in 3 month old female baby with multiple dark brown spots on her scalp,
abdomen, back, and upper thigh since birth. The surface was uneven and covered with hairs, without
any complaints of pain, easily traumatized, or pruritus. There is no family member with the similar
condition. The patient did not have any history seizures and signs of high intracranial pressure. We
advise the family member to have regular follow up to detect any changes in patient’s skin conditions
and neurological symptoms. The patient was planned for surgical treatment with flap extended after
she is older.
Discussion
The GCMN diagnosis in this patient was based on clinical examination. Considering the location and
diameter of the nevus, there are higher risks of malignant melanoma and NCM in this patient hence
regular follow up is recommended. Furthermore, we recommend for Magnetic Resonance Imaging
(MRI) examination to detect whether there is asymptomatic NCM. Although there are still
disagreements in timing of surgery, we planned the surgery after the patient is older minimalize the
risks and complications of the procedure.
Keywords: giant congenital melanocytic nevus, infant, rare case

Poster: BMT-10065
Abstract ID: BMT-10082
Track: Other Benign and Malignant Tumours
Contact Author: Ferra Olivia Mawu
Country: Indonesia
Department of dermatovenereology, Faculty of
Medicine, Sam Ratulangi University, Manado-
Indonesia
Poster: View Here
Multiple Facial Clyndromas
Case report
A pair of 10-year old twin girls presented with a 3 year history of asymptomatic multiple small tumours
nearly on the same site of their faces. First appearance of the lesions was on the nose area and slowly
increase in numbers which spread over the face. At the time of attendance, the twin was in generally
good condition. On examination, multiple rounded, smooth surfaced, firm, skin colored papules and
nodules of varying diameter (about 0.1-0.5cm) were seen on the face (nose, cheek, chin and forehead).
The lesions were painless, not itchy, never bleed. There was no lesion on the other parts of their bodies.
According to their parents, no other family members had this condition .
All the laboratory investigations were within normal range. Histopathologically, both skin biopsies
showed similar features of non encapsulated dermal tumour composed of nests of epithelial cells fit
together forming jigsaw-puzzle appearance. The nests of tumour are separated one to each other by
thin band of hyaline material. This finding support the diagnosis of cylindromas.
Discussion
Cylindromas are a type of benign skin adnexal tumor thought to arise from eccrine differentiation.
Clinically, they are in the form of papules and nodules on the skin mainly on the face and scalp and can
be single or multiple. Multiple cylindromas can have a syndromic association as seen in Brooke-
Spiegler syndrome and familial cylindromatosis. We present a case of non-familial multiple facial
cylindromas in a 10 year-old twin using histopathology examination to support the diagnosis.
Poster BMT-10082
Multiple Facial Cylindromas: A Case Report

Ferra O. Mawu, Melany Durry, Thigita A. Pandaleke
Dermatovenereologist and Pathologist
Faculty of Medicine, University of Sam Ratulangi, Manado, Indonesia
Siloam Hospitals Manado, RSUP Prof. dr. RD Kandou Manado
INTRODUCTION SUPPORT EXAMINATION

Cylindroma is a skin tumour traditionally regarded as showing All the laboratory
sweat gland lineage with a characteristic histology that usually investigations were within
manifests as nodules or tumours of the scalp and face. This is normal range.
an uncommon tumour. It is frequently familial and an Histopathologically, both
autosomal dominant gene determines its inheritance. The skin biopsies showed
onset is usually in early adult life, but may be in childhood or similar features of non
adolescence. It involves females more frequently than males. encapsulated dermal
The tumours are frequently multiple, smooth, firm, pink to red tumour composed of nests
in colour and often somewhat pedunculated. It ranges in size of epithelial cells fit together
from a few millimeters to several centimeters. forming jigsaw-puzzle
Multiple cylindromas are seen in Brooke–Spiegler syndrome, appearance. The nests of
an autosomal dominant condition with variable penetrance tumour are separated one
and expressivity, that presents with multiple cylindromas, to each other by thin band
eccrine spiradenomas, and trichoepitheliomas. Small tumours of hyaline material. This
are difficult to diagnose, and must be distinguished from finding support the
trichoepithelioma, steatocystoma or basal cell carcinoma if diagnosis of cylindromas.
solitary.
The treatment of choice for a cylindroma is surgical excision.
CASE REPORT
A 10-year old twin girls presented with a 3 year history of
DISCUSSION
asymptomatic multiple small tumours nearly on the same site
of their faces. First appearance of the lesions was on the
Cylindromas are a type of benign skin adnexal tumor thought
nose area and slowly increase in numbers which spread
to arise from eccrine differentiation. Clinically, they are in the
over the face. At the time of attendance, the twin was in
form of papules and nodules on the skin mainly on the face
generally good condition. On examination, multiple rounded,
and scalp and can be single or multiple. Multiple cylindromas
smooth surfaced, firm, skin colored papules and nodules of
can have a syndromic association as seen in Brooke-Spiegler
varying diameter (about 0.1-0.5cm) were seen on the face
syndrome and familial cylindromatosis. We present a case of
(nose, cheek, chin and forehead). The lesions were painless,
non-familial multiple facial cylindromas in a 10 year-old twin
not itchy, never bleed. No lesions on the other part of their
using histopathology examination to support the diagnosis. No
bodies. According to their parents, there were not similar
history of the same condition in family. At first glance the
condition in their family history.
lesions could be common kind of benign skin tumours.
Surprisingly, the histopathologic examination revealed the
characteristic appearance of cylindroma.
REFERENCES
Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffel DJ, Wolff K, eds. Fitzpatrick’s
dermatology in general medicine. 8th ed. New York: McGraw Hill, 2012.
James WD, Berger TG, Elston DM, editor. Andrew’s disease of the skin clinical dermatology.
10th ed. Canada: Saunders Elsevier; 2006.
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, eds. Rook’s Textbook of

Dermatology. 9th ed. United Kingdom: Wiley Blackwell; 2016
RESEARCH POSTER PRESENTATION DESIGN © 2015
www.PosterPresentations.com
Abstract ID: ADE-10064
Track: Atopic Dermatitis and Other Eczematous Conditions
Contact Author: Jing Yi Vanessa Cheong
Country: Singapore
Poster: View Here
Pharmacists' Counselling Improve Knowledge Of Caregivers Of Paediatric Eczema Patients
Introduction
Eczema is a common skin condition in childhood. Management of eczema can be complex and
caregivers may have lack of information on the condition and its management. Education has been
closely associated with compliance and is recommended for all patients. Currently, there is a lack of
studies looking at impact of pharmacist-led eczema counselling service on caregivers’ knowledge.
Objectives
The primary objective was to assess the impact of pharmacist-led eczema counselling service on
caregivers’ knowledge. The secondary objective was to evaluate caregivers’ satisfaction of service and
their confidence in handling the patients’ condition after counselling.
Methods
This was a prospective, questionnaire based observational study conducted at the outpatient pharmacy
of KK Women’s and Children’s Hospital (KKH), Singapore. The study was approved by the hospital’s
central institutional review board. Study participants were caregivers of new paediatric patients
diagnosed with mild to moderate eczema by our paediatric dermatologist, and referred for eczema
counselling. After obtaining informed consent, caregivers were given questionnaire A which contained
items on demographics and knowledge assessment. Counselling session by a trained pharmacist
would then be conducted. After counselling, questionnaire B containing items on satisfaction and
confidence was administered. Follow-up phone call was conducted 4 weeks after initial counselling
session, during which knowledge based questionnaire C would be administered.
Results
Data from 32 participants were included in the analysis. There was significant improvement in
caregivers’ knowledge score after receiving counselling, with mean pre-counselling score of 8.38±3.92
and mean post-counselling score of 13.88±3.65 (p<0.001 ). Majority found the information provided
during counselling easily understood, that the counselling session was useful, and time spent on
counselling session was appropriate. Majority was also confident in handling their children’s condition
after receiving counselling.
Conclusions
Pharmacist-led eczema counseling as an educational intervention has a positive impact on caregiver’s
knowledge and hence potentially improves compliance and patient care. This service was also well
received. More time should be devoted to education of patients and caregivers for paediatric eczema
patients.
Poster: ADE-10064
Contact Author: Purvis Diana
Country: New Zealand
Organisation: Starship Children's Hospital
Poster: View Here
Longterm Effect Of Methotrexate For Childhood Atopic Dermatitis
INTRODUCTION
Methotrexate is increasingly used for treatment of severe paediatric atopic dermatitis (AD). This study
aimed to look at the effect of methotrexate on AD severity both while on treatment and afterwards.
METHODS
Medical records of all children treated with methotrexate for AD at Starship Children’s Hospital,
Auckland, New Zealand between 2011-16 were reviewed. Demographics, previous treatment, length
and dose of methotrexate treatment, hospitalisations and use of other systemic treatment were
recorded. Parents were subsequently contacted by phone to assess response to treatment, AD
relapses, use of additional systemic treatment, and completed a patient oriented eczema measure
(POEM).
RESULTS
Forty-three patients aged 2-16 years (median 10) were included. Four (9%) had previous phototherapy
or other systemic immunosuppressives, and 14 (33%) previous hospitalisation for AD (28 admissions).
Methotrexate was given at a median dose of 0.33mg/kg (IQR 0.26-0.40mg/kg) for a median duration of
17 months (2-53 months). Adverse effects were recorded for 4 patients, all gastrointestinal upset. No
blood monitoring abnormalities occurred. Post methotrexate initiation only 6 (14%) were subsequently
hospitalised (9 admissions).
Telephone follow-up occurred in 30 patients, median 29 months post-methotrexate (IQR 14-45months).
Seven (23%) reported a change or a slight improvement€ • on methotrexate, and 23 (77%) reported
that AD was a lot better.€ • After stopping methotrexate only 15 (50%) reported AD recurrence, 10
(33%) reporting the recurrence to be as severe as the pre-methotrexate condition, 8 (27%) had
recommenced systemic treatment (6 methotrexate, 2 cyclosporin). Median POEM at follow-up was 6
(IQR1-17). Twenty-two (73%) had a POEM score <17 indicating mild-moderate AD, with only 8 (27%)
having a POEM of 17 or greater, indicating severe disease and 11 (37%) having a score <3 indicating
they were clear or almost clear. Two of the 13 children not available for telephone follow-up were known
to have had further systemic treatment.
CONCLUSION
Methotrexate treatment for severe AD appeared to be effective in three quarters of patients treated,
with both decreased severity scores and reduced hospital admissions. These improvements were
evident at a median of more than two years follow-up, suggesting persistance of treatment effect
following discontinuation of treatment.
Poster ADE-10092
Long term effect of methotrexate

for childhood atopic dermatitis
M Lee1, D Purvis2,3, K Agnew2, N Birchall2, S Dalziel2,3
1 Auckland City Hospital , 2 Starship Children’s Health, 3 University of Auckland, New Zealand
Introduction Figure 1 Patient flow Figure 2

• The use of systemic immunosuppressants such as
methotrexate (MTX) for severe atopic dermatitis (AD) in
POEM score at a median
childhood is included in treatment guidelines but there is follow-up of 29 months
limited data. Although benefit on MTX has been reported, 43 patients after stopping MTX n=30
long-term outcome after stopping MTX is unknown.
• We have used MTX as a firstline systemic agent for the
treatment of children with severe AD for over 7 years in
38 completed a course of 5 patients still on
tertiary paediatric dermatology clinics. MTX treatment
• The aims of this study were to assess the effect of MTX

while on treatment, and to report long term outcome after
stopping MTX treatment.
8 patients unable to be
30 patients contacted
contacted
Methods
• Medical records of all children treated with MTX for AD at
Starship Children’s Hospital, Auckland, New Zealand 5 – ‘no change’ at end 2 – ‘slight improvement’ 23 – ‘a lot better’ at end
of MTX treatment at end of MTX treatment of MTX treatment
between 2011-16 were reviewed.
• Demographics, previous systemic treatments, duration
and dose of MTX treatment, adverse events and 1 restarted MTX
hospitalisations were recorded. 2 ciclosporin
1 azathioprine No recurrence of AD 10 AD returned 13 no recurrence of AD
• MTX (once weekly oral dose) was offered if severe AD was 1 no further systemic
treatment
Clear 0-2 33%
not controlled with topical corticosteroids and emollients.
Mild 3-7 23%
• Topical therapy was continued as needed.
• Laboratory monitoring of full blood count, liver and renal 4 restarted MTX
Moderate 8-16 13%
function was performed initially weekly then 1-3 monthly.
Severe 17-24 23%
• Telephone follow-up of parents in 2017 recorded child’s
response to MTX treatment, AD relapses, additional Very severe 25-28 3%
systemic treatment and current Patient-Oriented Eczema 6 no further systemic
treatment
Measure (POEM) score over the last week (0-28).1
• Response to MTX was graded as:

(i) a lot worse
Table 1 Table 2
(ii) slightly worse
(iii) no change Demographics and MTX treatment Telephone follow-up
(iv) slightly better Demographics N=43 N (%) Parent reported outcome N=30 N (%)
(v) a lot better or minimal/no AD Gender Male:Female 21:22 (Male 49%) Time to telephone follow- Median (months) 29 (IQR 14-45)
Ethnicity European 19 (44%) up after stopping MTX
AD at end of MTX ‘A lot better’
• POEM score: Maori/Pacific 12 (28%)
treatment
23 (77%)
‘Slightly better’ 2 (7%)
0-2 clear or almost clear of AD Chinese/SE Asian 7 (16%)
‘No change’ 5 (17%)
Indian 5 (12%)
3-7 mild AD ‘Slightly worse’ 0
Previous systemic Phototherapy 2 (5%)
8-16 moderate AD treatments
Cyclosporin 2 (5%)
‘A lot worse’ 0
AD recurrence in those Recurrence (any) 10 (40%)
17-24 severe AD Prednisone 1 (2%) responding to MTX n=25 ‘As bad as before MTX’ 6 (24%)
25-28 very severe AD Previous Children admitted 14 (33%)
Mild recurrence 4 (16%)
hospitalisations
Number of hospitalisations 28
No recurrence 15 (60%)
Age at starting MTX Median 10 Years (IQR 7-12)
Median time to recurrence 24 (IQR 10.5-28.5)
Results MTX dose Median weekly oral dose
(mg/kg)
0.33 (IQR 0.26-0.40) (months)
Systemic treatment
4 (16%)
• 43 patients were identified (Figure 1). Duration of treatment Median (months) 17 (IQR 7.5-20)
restarted
Systemic treatment after Cyclosporin 2 (7%)
• Demographic details are shown in Table 1. MTX Azathioprine 1 (3%)
• MTX treatment was started at a median age of 10 years, Adverse effects Gastrointestinal upset 4 (9%)
Restarted MTX 6 (20%)
Cataracts 1 (3%)
at median dose of 0.33mg/kg, for a median duration of POEM score at time of Median 6 (IQR 1-17)
Laboratory monitoring 0 follow up
17 months. abnormalities <3 ‘Clear or near clear’ 11 (37%)
• Hospitalisation for AD was required by 33% of children Hospitalisation after Children admitted 6 (14%) 3-16 ‘Mild-moderate AD’ 11 (37%)
starting MTX >17 ‘Severe AD’ 8 (27%)
prior to starting MTX, but only 14% after MTX (p=0.07). Number of hospitalisations 9
• MTX was generally well tolerated:

• 1 patient stopped due to GI side effects.
• 1 patient was diagnosed with cataracts, but had Discussion
prior treatment with periorbital topical • This is the first study to report long term outcome • A study of 15 children treated with cyclosporin found
corticosteroids and oral prednisone which were after stopping MTX treatment. that 12 (80%) responded to treatment, of whom 5
thought to be causative. • MTX treatment for severe AD was effective (42%) required further systemic treatment at around
• No other serious adverse events were reported. • improved AD in more than 80% of children two years follow up. 4
• Telephone follow-up occurred with 30 parents (70%) at a • half the number of children hospitalised for AD • Limitations of our study include retrospective design,
median of 29 months after stopping MTX (Table 2). • Of those who responded to MTX, 40% had a vulnerability to recall bias, lack of use of a
• No improvement on MTX was reported by 5 (17%). recurrence of AD and only 16% required further standardised AD severity measure while on MTX.
• A positive response on MTX was reported by 25 (83%), systemic treatment. • It is also possible that the long term improvement
with 23 (77%) ‘a lot better’ and 2 (7 %) ‘slightly better’. • Importantly, MTX was associated with ongoing found in our study represents a natural history of
• AD recurred in 10 (40%) of responders, at a improvement in AD to a median of 29 months after improvement of AD through childhood.
median of 24 months after stopping MTX. MTX was stopped. • Ideally, future studies of MTX for AD would gather
• Severe AD recurred in 6 (24%). • 73% had no, mild or moderate AD data prospectively using both objective and subjective
• Systemic treatment was restarted in 4 (16%). • A third remained clear/near clear of AD measures. Long term outcome should be included in
• Median POEM at follow-up was 6 (mild AD), with 22 • These findings agree with other studies of MTX for studies of systemic therapies, as AD is a chronic
(73%) having no, mild or moderate AD (Figure 2). childhood AD which report good efficacy and disease and long term outcomes are important for
tolerability of MTX while on treatment.2,3 families and clinicians in making treatment decisions.
Conclusion
MTX was effective in over 80% of children with severe AD, with decreased severity and reduced hospital admissions.
73% reported no, mild or moderate AD at a median follow up of 29 months after stopping MTX,
suggesting long lasting treatment benefit following discontinuation.
REFERENCES: 1 Spuls et al The Patient-Oriented Eczema Measure: development and initial validation of a new tool for measuring atopic eczema severity from the patient’s perspective. Arch Dermatol 2004; 140:1513-19. 2 Deo et al. Methotrexate for the treatment of atopic dermatitis in children and adolescents. Int J Dermatol
2014;53(8):1037. 3 Dvorakova et al Methotrexate for severe childhood atopic dermatitis, clinical experience in a tertiary centre. Pediatr Dermatol 2017;34(5):528. 4 Sibbald et al Retrospective review of relapse after systemic cyclosporine in children with atopic dermatitis Pediatr Dermatol 2015;32(1):36-40.
Contact Author: Leung Theresa NH
Country: Hong Kong
Organization: The University of Hong Kong
Poster: View here
POEM Is An Ideal Symptom-based Severity Score For Childhood Eczema Research
Aim: It is important to objectively measure the severity of atopic dermatitis (AD). This study aims to
investigate correlations among various clinical severity scores and determine how a severity score
based on symptoms alone performs.
Methods: A modified symptom score based on Patient-Oriented Eczema Measure (POEM, a short-term
subjective-symptom score), Scoring Atopic Dermatitis (SCORAD, a short-term subjective-symptom and
objective-sign score), Nottingham Eczema Severity Score (NESS, a long-term subjective-symptom
score), Children Dermatology Life Quality Index (CDLQI, a short-term subjective-symptom score), skin
hydration (SH) and transepidermal water loss (TEWL) were compared and Spearman's rho correlations
evaluated.
Results: 126 sets of clinical scores from eczema patients (mean age: 11.4Â±5.6years; 34.7% male)
were evaluated. The modified-POEM, objective SCORAD, NESS, CDLQI correlated well with each
other. All round, best correlations were obtained with POEM: Objective SCORAD (rho=0.7, p<0.001),
NESS (rho=0.69, p<0.001), SCORAD symptom of itch (rho=0.75, p<0.01), SCORAD symptom of sleep
loss (rho=0.64, p<0.01), CDLQI (rho=0.77, p<0.001) and SH (rho= -0.043, p<0.05). Linear stepwise-
backward regression demonstrated that POEM was independently associated with CDLQI parameters
of pruritus (B: 2.16; p=0.018), activities (B: 1.80; p= 0.009), sleep disturbance (B: 2.78; p<0.001)], and
NESS parameter of sleep disturbance (B: 1.02; p=0.003).
Conclusion: Clinical scores for acute, chronic, subjective symptoms and objective signs correlated well
with each other. The symptom measures by modified POEM is easy to perform by parents or patients
and correlated better with most other clinical scores, making it an all-round ideal symptom-based
severity score for research.
Poster: ADE-10102
RSMPD 2018 Abstract ID: ADE-10102

POEM is an ideal symptom-based severity score for childhood eczema research
Theresa NH Leung1, Kam-lun Hon2

1 Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong
2 Department of Paediatrics, The Chinese University of Hong Kong
Background:
It is important to have objective measures of eczema outcome for clinical practice and research.
Aim:
To investigate correlations among various clinical severity scores and determine how a severity score
based on symptoms alone performs.
Methods:
Retrospective review of data for eczema children < 18 years attended the paediatric dermatology clinic of
Prince of Wales Hospital in Hong Kong
Clinical Scores listed below and physical assessment of skin hydration were compared and Spearman's rho
correlations evaluated
Table 1. Clinical Scores
Modified-POEM: modified Patient-Oriented Eczema Measure
N= 126 Number (%)
SCORAD : Scoring Atopic Dermatitis Gender M 43(34.7)
NESS : Nottingham Eczema Severity Score Mean (SD) age in years 11.4±5.6
Mean POEM (SD) 14.8±7.3
CDQLI : Children Dermatology Life Quality Index Clear 5 (4.8)
SH : Skin Hydration (Corneometer CM825) Mild 13 (12.5)
Moderate 34 (32.7)
Severe 34 (32.7)
Results: Very severe 13 (12.5)
Clinical scores for 126 patients as shown in Table 1 Mean SCORAD 38.1±18.2
Mild 18 (14.4)
All clinical scores include sleep loss and pruritus Moderate 39 (31.2)
The modified-POEM, objective SCORAD, NESS, Severe 18 (14.4)
CDLQI correlated well with each other. (Table 2) Mean NESS (SD) 7.6±3.4
Mild 44 (41.1)
Linear stepwise-backward regression demonstrated that Moderate 48 (44.9)
POEM was independently associated with Severe 15 (14.0)
CDLQI parameters of pruritus (B: 2.16; p=0.018), Mean (CDLQI) 9.8±7.3
No effect 27 (21.2)
activities (B: 1.80; p= 0.009), sleep disturbance (B: Small effect 33 (26.3)
2.78; p<0.001)], Moderate effect 29 (23.2)
Very large effect 18 (14.1)
NESS parameter of sleep disturbance (B: 1.02; Extremely large effect 19 (15.2)
p=0.003).
Table 2. Spearman’s correlations for components scores of SCORAD, NESS and CDLQI controlling for
gender, age and body-mass-index
Scores Types of assessement 1 2 3 4 5 6 7
1 SCORAD Objective short-term -
2 NESS Subjective long-term 0.66** -
3 POEM Subjective short-term 0.70*** 0.69*** -
4 CDLQI Subjective short term 0.36* 0.54* 0.77*** -
5 SH Objective physical 0.47 -0.41* -0.43* -0.46* -
6 Pruritus^ Subjective 0.50* 0.53* 0.75** 0.74** 0.03 -
7 Sleep loss^ Subjective 0.25 0.50* 0.64** 0.66* 0.00 0.72* -
^Pruritus and sleep loss components of SCORAD
*P<0.05, ** p<0.01, ***p<0.001
Conclusions:
Clinical scores for acute, chronic, subjective symptoms and objective signs correlated well with each other.
Modified POEM is an all-round ideal symptom-based severity score for clinical practice and research
Easy to perform by parents or patients
Correlated better with most other clinical scores
Contact Author: Nadia Wirantari
Country: Indonesia
Organization: Others
Dept of Dermatology and Venereology, Faculty of

Medicine,Universitas Airlangga/ Dr. Soetomo Hospital
Poster: View Here
COMPARISON OF TEWL VALUE BETWEEN DIAPER AREA AND NONDIAPER AREA OF

INFANT’S SKIN
Introduction and objectives: Diaper use has become an important factor influencing skin barrier function.
Prolonged use of diaper leads to exposure to irritants from feces and urine, concurrent mechanical
friction and occlusion, thus render the skin prone to inflammation and infection. Measurement of skin
TEWL may be used to document skin barrier function, and to our knowledge this has never been done
tropical country such as Indonesia. The objective of this study is to compare the difference of TEWL
value between baby’s diapered and nondiapered skin.
Materials and methods: in this cross sectional study, 43 healthy babies aged 1 to 6 months were
measured using open chamber instrument (Tewameter TM300) on diapered and nondiapered skin.
TEWL values were documented and compared.
Results: The mean TEWL value of diapered skin is 22,96 ± 12,42 g/m2/h (95% CI, 10,80 - 58,30) and
nondiapered skin is 18,49 ± 9,29 g/m2/h (95% CI, 10,60 - 51,80), with significant difference (p=0,006).
Conclusions: TEWL values increase significantly on diapered skin compared to nondiapered skin,
revealing impaired barrier function on diapered skin, despite no pathological skin changes.
Keywords: TEWL, infant’s skin, diaper area, nondiaper area.

Poster: ADE-10104
COMPARISON OF TEWL VALUE BETWEEN DIAPER AREA

AND NONDIAPER AREA OF INFANT'S SKIN
Nadia Wirantari, Linda Astari, Iskandar Zulkarnain
Department of Dermatology and Veneorology, Faculty of Medicine, Universitas Airlangga/
Dr. Soetomo Hospital, Surabaya, Indonesia
Telephone: (031) 5501609, email: nadia wirantari@yahoo.com
ABSTRACT
- Introduction and objectives -

Diaper use has become an important factor influencing skin barrier function. Prolonged use of diaper
leads to exposure to irritants from feces and urine, concurrent mechanical friction and occlusion, thus
render the skin prone to inflammation and infection. Measurement of skin TEWL may be used to
document skin barrier function, and to our knowledge this has never been done tropical country such as
Indonesia. The objective of this study is to compare the difference of TEWL value between baby's
diapered and nondiapered skin.
- Materials and Methods - The value of TEWL

In this cross sectional study, 43 healthy babies on diaper area and nondiaper area.
aged 1 to 6 months were measured using open
chamber instrument Tewameter TM300 on
6
diapered and nondiapered skin. TEWL values were 8.00
O
53
documented and compared. O
7.00
6.00
- Result -
The mean TEWL value of diapered skin is 22,96 ± 5.00
12,42 g/m2/h (95% CI, 10,80 - 58,30) and

nondiapered skin is 18,49 ± 9,29 g/m2/h (95% CI, diaper area nondiaper area
10,60 - 51,80), with significant difference

(p=0,006).
- Conclusions -
TEWL values increase significantly on diapered skin compared to nondiapered skin,
revealing impaired barrier function on diapered skin, despite no pathological skin changes
Keywords: TEWL, infant's skin, diaper area, nondiaper area

Skin Manifestations of Other Systemic
Track:
Disordersenodermatoses
Contact Author: Liau Mei Qi May
Country: Singapore
Poster: View Here
A Characterisation Of Juvenile Acanthosis Nigricans In A Singaporean Tertiary Dermatological

Centre
19 patients (12 males, 7 females) had a primary diagnosis of juvenile acanthosis nigricans in our centre
between 2014 - 2017. They presented with typical skin signs on the axilla (84%), neck (84%), groin
(42%), inner thigh (11%), antecubital or popliteal fossae (11%) and other regions (16%). They were
aged 5 to 19 and had a mean BMI of 28.3 (12 obese, 4 overweight, 1 within normal range, 2 not
documented). Hypertriglyceridemia was diagnosed in 3 patients - triglyceride levels (mmol/L) of 3.7, 2.9
and 3.07 respectively. 2 patients with diabetes mellitus (fasting glucose of 6.2 and 15.4 respectively)
were diagnosed after first presenting with acanthosis nigricans. Notably, both these patients were also
diagnosed with hidradenitis suppurativa.
There was a strong family history of metabolic syndrome. Of the 15 patients with a documented family
history, there was a positive association with the following: hypertension (58%), diabetes mellitus (53%),
ischaemic heart disease (15.8%), hyperlipidaemia (11%) and stroke (5%).
Juvenile acanthosis nigricans is an important cutaneous marker of insulin resistance, increasingly being
diagnosed in obese children and adolescents worldwide. Diagnosis provides a window of opportunity
for early intervention, which is vital to minimise the risk of future endocrinological and cardiovascular
complications.
Poster: SSD-10072
A char acter isation of

Juvenile Acanthosis nigr icans
in a Singapor ean ter tiar y der matological centr e
Liau MeiQi May, Nisha S. Chandr an
Intr oduction
Acanthosis nigr icans (AN) is a velvety thickening of the epider mis which pr imar ily affects the axillae,
poster ior neck fold and flexor skin sur faces. It is incr easingly seen in obese childr en and adolescents
and ser ves as a cutaneous mar ker of insulin r esistance.1
Results
19 patients (12 males, 7 females) had a pr imar y
diagnosis of juvenile AN in our centr e between
2014 - 2017. They pr esented with typical skin
signs on the axilla (84%), neck (84%), gr oin (42%),
inner thigh (11%), antecubital or popliteal fossae
(11%) and other r egions (16%). They wer e aged 5 to
19 and had a mean BMI of 28.3 (12 obese, 4
over weight, 1 within nor mal r ange, 2 not
documented).
Hyper tr iglycer idemia was diagnosed in 3 patients Fig 1. Acanthosis nigricans of the neck
folds
- tr iglycer ide levels (mmol/L) of 3.7, 2.9 and 3.07
r espectively. 2 patients with diabetes mellitus
(fasting glucose of 6.2 and 15.4 r espectively) wer e
diagnosed after fir st pr esenting with AN. Notably,
both these patients wer e also diagnosed with
hidr adenitis suppur ativa.
Ther e was a str ong family histor y of metabolic

syndr ome. Of the 15 patients with a documented
family histor y, ther e was a positive association
with the following: hyper tension (58%), diabetes
mellitus (53%), ischaemic hear t disease (15.8%),
Fig 2.Body mass index (BMI) of patients with primary diagnosis of
hyper lipidaemia (11%) and str oke (5%). Juvenile AN
Conclusion
The incidence of juvenile AN par allels the incr ease in childhood obesity and associated insulin r esistance.
Der matologists may ser ve an impor tant r ole in facilitating the pr oper wor kup and tr eatment of childr en with
acanthosis nigr icans. Diagnosis pr ovides a window of oppor tunity for ear ly inter vention, which is vital to
minimise the r isk of potentially devastating futur e complications including type 2 diabetes mellitus,
car diovascular disease, hyper tension, and liver and kidney dysfunction. 2
Refer ences
1. Sinha S, Schwar tz RA. Juvenile acanthosis nigr icans. Jour nal of the Amer ican Academy of Der matology. 2007 Sep 1; 57(3):502- 8.
2. Ten S, MacLar en N. Insulin r esistance syndr ome in childr en. J Clin Endocr inol Metab 2004;89:2526- 39
Abstract ID RSMPD23
Track Skin manifestations of other systemic disorders
Contact author R.M Rendy Ariezal Effendi
Country Indonesia
Faculty of Medicine Universitas Padjadjaran,

Organisation
Hasan Sadikin Hospital Bandung
Poster View Here
Langerhans Cell Histiocytosis: A Case Series
Background: Langerhans cell histiocytosis (LCH) is a disorder characterized by proliferation and

accumulation of Langerhans cells into various organs of the body. This disease may affect any age
group but mainly affects children between 1-15 years, with a peak incidence at 1-4 years of age. LCH
may involve multiple organ systems. The clinical manifestations depend on the site of the lesions and
the organ involved. Any organ can be involved in LCH, but skin and bone are most commonly affected.
Diagnosis of LCH is often delayed because of its varying clinical manifestations. Thus it can cause
delayed in treatment. The definitive diagnosis is established through skin biopsy.
Methods: We recorded the presenting complaints and dermatologic problems of patients with LCH who
were hospitalized in Hasan Sadikin Hospital from January to December 2017. Patients with LCH were
assessed based on age, physical and laboratory examination to establish diagnosis of LCH.
Result: Seven new patients with LCH have been consulted from paediatric department to our
department during this period. The youngest age is ten months old and the oldest is two years old. All
of these patients have erythematous macule, petechiae as a skin disorder and hepatosplenomegaly.
Histopathological results confirm the diagnosis of LCH. Four patients died during this period, three of
them died because of respiratory failure. All of LCH patient were given systemic therapy after diagnosis
of LCH is established
Conclusion: Delay in diagnosis can cause delay in therapy which can increases mortality in LCH
patients
Keywords: Langerhans cell histiocytosis, skin biopsy, systemic therapy

Poster RSMPD23
Abstract ID RSMPD11
Track Neonatal skin disorders
Contact author Triana Agustin

Country Indonesia
Organisation Faculty of Medicine, Universitas Indonesia-dr.

Cipto Mangunkusumo National General Hospital,
Jakarta, Indonesia
Poster View Here
Comparison of the Efficacy between Local Manufactured and Imported Moisturizers on The Skin
Barrier of Very Low Birth Weight Premature Neonates
Introduction and Objectives

Transepidermal Water Loss (TEWL) reflects the barrier integrity of the stratum corneum, which is
related to gestational age. Neonates delivered at 37 weeks gestation have completed development of
the epidermis, including stratum corneum, thus providing better skin barrier function in comparison to
neonates delivered at 32 weeks gestation. Premature neonates need 2 to 4 weeks of postnatal
epidermal maturing in order to develop epidermal structure and function equivalent to full-term
neonates. Topical application of moisturizers is evaluated as a strategy to enhance skin barrier function
in preterm neonates.
Materials and Methods

A pilot study for a prospective, randomized, double-blind, clinical trial was performed on preterm
neonates weighed 1500 grams or less at birth and/or delivered at 32 weeks gestation or less.
Moisturizer was applied on the right upper arm and thigh, twice daily for 14 days. TEWL measurement
(g/m2/hour) was done within 24 hours after delivery, and was continued on day 7 and 14 afterwards,
using tewameter TM300.
Results
The subjects included 54 premature infants, divided into two groups. The first group consisted of 28
infants (average gestational age 30.21 weeks), who received imported topical moisturizer. The second
group consisted of 26 infants (average gestational age 30.12 weeks), who received locally
manufactured topical moisturizer. The average TEWL measured before moisturizer application on the
right arm was 19.66 in the first group and 21.77 in the second group. While on the right leg, the average
TEWL was 21.54 and 27.12 in the first and second group, respectively.
On the 7th and 14th day after moisturizer application, the TEWL of the right limbs (received moisturizer
application) and the left limbs (did not receive treatment) were measured. In the first group, the TEWL
of the right limbs was lower compared to the left limbs. Similar results were observed in the second
group. This finding is caused by the improvement of epidermal barrier functions after moisturizer
application, which leads to a reduction of TEWL. No significant difference of TEWL between the two
groups after statistical analysis.
Conclusions
Moisturizer use, both locally manufactured and imported, reduces TEWL.
Poster RSMPD11
Contact Author: Srinivas Sahana
Country: India
Indira Gandhi Institute of Child Health, Bangalore,
Organisation:
Karnataka, India
Poster View Here
Silvery Grey Hair Syndromes: Experience From Tertiary Care Centre From South India
Introduction and Objectives

Silvery hair is a common presentation of rare group of autosomal recessive disorders called silvery hair
syndrome that includes Griscelli syndrome (GS), Chediak-Higashi syndrome (CHS) and Elejalde
syndrome. Characteristic features include silvery sheen to hair, variable cellular and humoral
immunodeficiency, systemic features and hair shaft anomalies. The main objective of this study is to
describe the epidemiology and clinical features of silvery hair syndrome in children presenting at a
tertiary care centre from South India.
Materials and Methods

This study is a descriptive study with retrospective chart review comprising 20 children with silvery hair
syndrome seen over a period of 6 years from January 2012 through December 2017. All children 18
years and below with characteristic features of silvery grey hair syndrome presenting to the department
of pediatric dermatology at authors institution were included in the study. The diagnosis was based on
clinical features and characteristic hair microscopy. Complete data was collected on a predesigned
proforma.
Result
Age of presentation in the study ranged from 15 days to 9 years. There were 12 (60%) boys and 8
(40%) girls. 19 (95%) children were born out of consanguineous marriage. Hyperpigmented and
hypopigmented mottled pigmentation was seen in 10 (50%) children on photo exposed areas. Systemic
features included recurrent fever (60%), recurrent respiratory tract infection (25%), failure to thrive
(20%), hepatosplenomegaly (55%), pneumonia (5%), epilepsy (5%), and hypokalemia (5%).
Pancytopenia was present in 15 (75%) and raised liver enzymes in 9 (45%) children. Hair microscopy
showed small and large clumps of melanin in irregular pattern in 17 (85%) children indicating the
diagnosis of GS and regularly arranged small melanin clumps in 3 (15%) children favoring diagnosis of
CHS. Based on the clinical and hair microscopic features diagnosis of GS type 1 was considered in 2
(10%), GS type 2 in 12 (60%) and GS type 3 in 3 (15%) children respectively.
Conclusions
GS type 2 is the most common type of silvery grey hair syndrome seen in our study. In a resource poor
setting early recognition of this condition is important to work up for immunodeficiency to prevent
morbidity.
Poster: GDT-10094
SILVERY GREY HAIR SYNDROMES: EXPERIENCE FROM

TERTIARY CARE CENTRE FROM SOUTH INDIA
Sahana M Srinivas, Vykunta Raju, Sanjeeva G.N, Asha Benakappa

Department of Pediatric Dermatology, Pediatric Neurology, Clinical Genetics & Pediatrics
Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
INTRODUCTION RESULTS
Silvery hair is a common presentation of rare group of Study children with silvery hair - 20
autosomal recessive disorders called silvery hair syndrome.
Age of presentation ranged - 15 days to 9years
Silvery hair syndromes includes 3 types: Griscelli syndrome
(GS), Chediak-Higashi syndrome (CHS) and Elejalde 19 (95%) children born of consanguineous marriage
syndrome. There are 3 types of GS.
Family history of silvery hair was seen in 3 (15%) children
Characteristic clinical features are silvery sheen to hair,
variable cellular and humoral immunodeficiency, systemic Delayed developmental milestones - 5 (25%)
abnormalities, hair shaft anomalies and rarely ocular
hypopigmentation. Mottled pigmentation on photoexposed areas - 10(50%)
AIM OF THE STUDY RESULTS
To study the epidemiological and clinical

features of silvery hair syndrome in children
METHODS
Descriptive study with retrospective chart
review of 20 children seen over a period of SEX DISTRIBUTION PROFILE OF SYSTEMIC FEATURES IN SILVERY HAIR
6 years from January 2011 through SYNDROME
December 2017.
All children 18 years and below with

silvery grey hair presenting to our SPECTRUM OF SILVERY
department of pediatric dermatology was GREY HAIR SYNDROME IN
included in the study. OUR STUDY BASED ON
CLINICAL FEATURES,
Diagnosis was based on clinical, LABORATORY AND HAIR
MICROSCOPY
laboratory features and characteristic hair
shaft microscopy. Data was collected on a
predesigned proforma
INVESTIGATIONS
Pancytopenia - 15 (75%) DISCUSSION
Increased liver enzymes in 9 (45%)
Peripheral smear: Giant granules in
leucocytes - 3 (12.5%) diagnostic of CHS GS are caused by mutation in
myosin Va (type 1) and RAB27A
(type 2). GS type 1 is associated with
neurological involvement like
HAIR MICROSCOPY developmental delay, mental
retardation, hypotonia, seizures. GS
Small and large clumps of melanin in type 2 is associated with
irregular pattern - 17 (85%) indicating immunodeficiency and GS type 3
diagnosis of GS involves only skin.
FIG 1: GRISCELLI FIG 2: CHEDIAK-
Regularly arranged small melanin clumps SYNDROME HIGASHI SYNDROME Prognosis of GS is grave than CHS
- 3 (15%) indicating diagnosis of CHS
Bone marrow transplantation or
CONCLUSION peripheral blood stem cell
In our study GS type 2 was the most commonest transplantation is only curative
silvery grey hair syndrome.
REFERENCES
In a resource poor setting where genetic analysis Sahana MS, Sacchidanand S, Hiremagalore R,
may not be feasible, early recognition of this entity Asha GS. Silvery grey hair syndrome:clue to
GS SHOWING SMALL CHS WITH SMALL diagnose immunodeficiency. Int J Trichol
CLUMPS OF by clinical features is important to work up for 2012;4:83-5.
AND LARGE CLUMPS
OF MELANIN IN MELANIN immunodeficiency which can not only predict the Rudramurthy P, Lokanatha H. Chediak-Higashi
IRREGULAR PATTERN ARRANGED prognosis but also aid in improving the course of syndrome: A case series from Karnataka, India. Ind
REGULARLY J Dermatol 2015;60:524.
the disease.
Abstract ID RSMPD21
Track Pigmentary disorders
Contact author Reiva Farah Dwiyana
Country Indonesia
Department of Dermatology and Venereology Faculty

Organisation of Medicine Universitas Padjadjaran / Dr. Hasan
Sadikin Hospital, Bandung Indonesia
Poster: View Here
THE COMBINATION THERAPY OF EXCIMER LIGHT AND VITAMIN D3 ORAL FOR CHILDHOOD
VITILIGO: A PROSPECTIVE STUDY IN INDONESIA
Background
Vitamin D deficiency is a condition often found in various autoimmune diseases, including vitiligo. There
were clinical improvements in autoimmune patients who had been given oral vitamin D
supplementation, as well as vitiligo patients. However, efficacy of vitamin D supplementation in
childhood vitiligo is still unknown. Therefore, a study of vitamin D supplementation efficacy in childhood
vitiligo is needed.
Aim
To know whether combination of excimer light phototherapy and oral vitamin D supplement gives better
result in improving vitiligo lesions and increased level of vitamin D levels in children compare to excimer
light monotherapy.
Methods
A controlled trial study was conducted on 16 childhood vitiligo patients (below 18 years old) who were
divided into two groups. The first group received combination of excimer light phototherapy and 5000
IU vitamin D3, while the second group received only excimer light therapy for 8 weeks. The serum
25(OH)-Vitamin D level and vitiligo area scoring index (VASI) were measured before and after therapy
to evaluate the vitamin D level status and improvement of lesions.
Results
Comparison between VASI score before and after therapy in Group 1 showed p value 0.012 (p<0.05)
and 0.028 (p<0.05) in Group 2, which indicated that both types of therapy gave good results in improving
vitiligo lesions in children. The average increase of 25-(OH)D level in group I was 324.00±119.06% and
29.84±36.10% in Group 2. However, comparison between VASI score changes between both groups
showed p value 0.681 (p>0.05), which meant there were no significant difference between VASI score
changes in both group, although 25-(OH)D level was increased significantly in both Group.
Conclusion
Combination of excimer light phototherapy and oral vitamin D supplement gave better effect than
monotherapy of excimer light phototherapy to increase 25-(OH)D levels, but not for improving vitiligo
lesions in children.
Keywords: Childhood vitiligo, excimer light, vitamin D

Poster: RSMPD21
THE COMBINATION THERAPY OF EXCIMER LIGHT

AND VITAMIN D3 ORAL FOR CHILDHOOD VITILIGO:
A PROSPECTIVE STUDY IN INDONESIA
Reiva Farah Dwiyana, DP Larasati, Pramita KC Nugrahaini , R.M Rendy A Effendi
Faculty of Medicine Universitas Padjadjaran / Dr. Hasan Sadikin Hospital, Bandung Indonesia
Email: reiva.farah@yahoo.co.id
INTRODUCTION
Vitamin D deficiency is a condition often found in various autoimmune disease, including vitiligo. There were clinical
improvements in autoimmune patients who had been given oral vitamin D supplementation, as well as vitiligo patients.
However, efficacy of vitamin D supplementation in childhood vitiligo is still unknown. Therefore, a study of vitamin D
supplementation efficacy in childhood vitiligo is needed. The ai m of this research was to know whether combination of
excimer light phototherapy and oral vitamin D supplement gives better result in improving vitiligo lesions and increased
level of vitamin D levels in children compare to excimer light monotherapy.
METHODS
A controlled trial study was conducted on 16 childhood vitiligo patients (age below 18 years old) which were divided into
two groups. The first group received combination of excimer light phototherapy and 5000 IU vitamin D3, while the second
group received only excimer light therapy for 8 weeks. The serum 25(OH)D level and vitiligo area scoring index (VASI) were
measured before and after therapy to evaluate the vitamin D level status and improvement of lesions
RESULTS
Comparison between VASI score before and after therapy in Group 1 showed p value 0.012 (p<0.05) and 0.028 (p<0.05)
in Group 2, which indicated that both types of therapy gave good results in improving vitiligo lesions in children. The
average increase of 25-(OH)D level in group I was 324.00±119.06% and 29.84±36.10% in Group 2. However, comparison
between VASI score changes between both groups showed p value 0.681 (p>0.05), which meant there were no significant
difference between VASI score changes in both group, although 25-(OH)D level was increased significantly in both Group.
DISCUSSION
The active form of vitamin D, 1,25-(OH)2D3, attached to VDR presented on most immune cells, such as monocytes,
macrophages, dendritic cells, natural killer cells, T cells, and B cells. The bond created supression of T cell activation and
changes cytokines secretion patterns which caused immunosuppression, which hoped to prevent melanocyte destruction in
vitiligo patients. However, in this study, addition of vitamin D did not show significant difference to excimer light
monotherapy. This showed minimal or lack of autoimmune process in children vitiligo pathogenesis.
CONCLUSION
Combination of excimer light phototherapy and oral vitamin D supplement gave better effect than monotherapy of
excimer light phototherapy to increase 25-(OH)D levels, but not for improving vitiligo lesions in children
References
1. Kruger C, Schallreuter KU. A review of the worldwide prevalence of vitiligo in children/adolescents and adults. Int J Dermatol. 2012: 51: 1206-1212.
2. Bilgic O, Bilgic A, Akis HK, Eskioglut F, Kilic EZ. Depression, anxiety and health-related quality of life in children and adolescents with vitiligo. Brit Assoc Dermatol. 2010: 36: 360-365.
3. Oh SH, Kim M. Vitamin D and vitiligo: InTech; 2011: 23-45.
4. Wang Y, Zhu J, DeLuca HF. Where is the vitamin D receptor? Arch Biochem Biophys. 2012: 523: 123–133.
5. AlGhamdi K, Kumar A, Moussa N. The role of vitamin D in melanogenesis with an emphasis on vitiligo. Indian J Dermatol Venereol Leprol. 2013: 79: 750-758
Abstract ID: HND-10044
Track: Hair and Nail Disorders
Contact Author: Colin Tan Wei Xuan
Country: Singapore
Poster View Here
Should We Biopsy Melanonychia Striata In Asian Children? A Retrospective Observational

Study.
Background and Objective:

Signs of a subungual melanoma are less reliable in children with melanonychia striata and the risk of
subungual melanoma in children is extremely rare. Nail biopsies in children is difficult and can result in
nail dystrophy. The aim of this study was to review the clinical and histological characteristics of
melanonychia striata in pediatric patients of Asian descent to aid the clinical decision for nail biopsy.
Materials and methods

62 patients presenting with melanonychia striata involving single or multiple nails at 2 tertiary pediatric
dermatology clinics were recruited between 2005 and 2015. Clinical and histopathological data were
reviewed, including: length of follow up, age at onset, age at presentation, location, color, width,
presence of Hutchinson’s/Pseudo-Hutchinson’s sign, nail dystrophy and clinical evolution.
Results
There were no cases of subungual melanoma in our series. Solitary lesions of the thumbnail, with a
single uniform color were the most common presentation. Pseudo-Hutchinson’s sign was commonly
observed. Majority of patients observed in the follow up period did not have worrisome features. There
was consensus in histological diagnosis between all 3 dermato-pathologists in only half of the cases
biopsied.
Conclusion:
We recommend a cautious approach and close monitoring of melanonychia in Asian children based on
our findings. A constellation of high risk features should be present before proceeding with a nail matrix
biopsy. Follow up of such patients should be till adulthood as there remains a theoretical risk of
malignant transformation.
Poster: HND-10044
Contact Author: Wai Mun Sean Leong
Country: Singapore
Poster View here
Use Of Diphenylcyclopropenone (DPCP) In The Treatment Of Paediatric Alopecia Areata (AA):

Experience From A Tertiary Healthcare Institution In Singapore
Introduction
Alopecia areata (AA) is chronic T cell-mediated disorder that targets anagen hair follicles and causes
non-scarring hair loss. It is a relatively common dermatological entity, accounting for approximately 2%
of dermatology new cases in the West[1]. Diphenylcyclopropenone (DPCP), first introduced by Happle
et al[2], induces an allergic contact dermatitis that modulates this T-cell-mediated reaction against the
hair bulb and induces hair regrowth. It is a suitable treatment modality in paediatric patients. We review
the use of DPCP in the treatment of paediatric AA in an Asian population over a 6 year period.
Material and Methods

We retrospectively reviewed all paediatric patients aged 16 and under who were diagnosed with AA
over a six year period (1st January 2012 to 31st December 2017) and who have received DPCP. We
evaluated the patient demographics, the extent and pattern of scalp involvement, any associated
presenting symptoms, comorbidities, previous treatments, number of sessions of DPCP before hair
regrowth was noted, follow-up duration and also whether any recurrence was noted in these patients.
Clinical response rate was determined from the percentage regrowth of hair.
Results
Nine patients were studied in total. The mean age of the patients was 11 years old. The male:female
ratio was 1.25:1. None of the patients had previous autoimmune or atopic disease. All patients had
scalp involvement, except one patient who only had involvement of the eyebrows. The mean duration
of lesions prior to presentation was 825 days. 66.7% (n=6) of the patients had received prior treatment
before DPCP, the most common being intralesional triamcinolone. The mean duration of onset of AA
prior to the start of DPCP therapy was 313 days. Improvement was seen after 1 and 2 sessions of
DPCP in 33.3% (n=3) and 66.7% (n=6) patients respectively. The mean duration of follow up was 205
days. 1 patient progressed to alopecia totalis requiring oral prednisolone. No significant side effects
were noted during treatment.
Conclusion
DPCP is an effective and safe treatment for paediatric AA patients with a short duration of alopecia.
Poster: HND-10088
USE OF DIPHENYLCYCLOPROPENONE (DPCP) IN THE

TREATMENT OF PAEDIATRIC ALOPECIA AREATA (AA):
EXPERIENCE FROM A TERTIARY HEALTHCARE INSTITUTION
IN SINGAPORE
LEONG WMS1, CHANDRAN NS1
1 Division of Dermatology, University Medicine Cluster, National University Hospital
Introduction
Alopecia areata (AA) is chronic T cell-mediated disorder that targets anagen hair follicles and causes non-scarring hair loss. It
is a relatively common dermatological entity, accounting for approximately 2% of dermatology new cases in the West[1].
Diphenylcyclopropenone (DPCP), first introduced by Happle et al[2], induces an allergic contact dermatitis that modulates
this T-cell-mediated reaction against the hair bulb and induces hair regrowth. It is a suitable treatment modality in
paediatric patients. We review the use of DPCP in the treatment of paediatric AA in an Asian population over a 6-year period.
Aim
To evaluate the safety, efficacy, tolerability of DPCP in paediatric patients aged 16 years old and below with alopecia areata.
Methodology
We retrospectively reviewed all paediatric patients who were diagnosed with AA over a 6-year period (1st January 2012 to 31st
December 2017) and who have received DPCP. Patients were first sensitised to a 2% solution of DPCP over a small area of
the scalp. Subsequently, further concentrations of DPCP were applied at increasing concentrations starting from 0.001% until
a mild dermatitis was observed. We evaluated the patient demographics, the extent and pattern of scalp involvement, any
associated presenting symptoms, comorbidities, previous treatments, number of sessions of DPCP before hair regrowth was
noted, follow-up duration and also whether any recurrence was noted in these patients. Clinical response rate was determined
from the percentage regrowth of hair. Ethics approval was obtained from the National Healthcare Group Institutional Review
Board.
Results
Variable N (Percentage Variable Mean (±Range/Percentage)
%)
Mean age of Presentation 11 (4 – 16)
Gender Male 5 (55.6)
(Years)
Female 4 (44.4)
Duration of Disease Prior 313 (60 – 1400)
Ethnicity Chinese 5 (55.6)
to Starting Therapy (Days)
Malay 1 (11.1)
Indian 1 (11.1)
Percentage of Patients 6 (66.7%)
Others 2 (22.2)
who Received Prior
Presence of Atopy 0 (0)
Family History of 1 (11.1) Treatment Before DPCP
Associated
Factors Alopecia Areata Patients who Improved 3 (33.3%)
after 1 session of DPCP
Autoimmune/End 1 (11.1) Patients who Improved 6 (66.7%)

ocrinological after 2 sessions of DPCP
Disorders Mean number of DPCP 14.4 (1 – 51)
sessions received
Specific Oophiatic 0 (0)
Pattern of Hair Eyebrow/eyelash 1 (11.1) Duration of Follow Up 205 (71 – 500)
Loss involvement only (Days)
Alopecia Totalis 1 (11.1) Recurrence Rate at Last 0
Follow up (Number of
Patients)
Nine patients were studied in total. The two tables above summarise the most pertinent findings. The most common treatment
prior to DPCP was intralesional triamcinolone. One patient developed lymphadenopathy without fever during sensitization and
decided not to proceed with DPCP. One patient with alopecia totalis had minimal improvement with 14 sessions of DPCP and
required further immunosuppression subsequently.
Conclusions
DPCP is a well tolerated treatment modality in paediatric patients. Recurrence rates are low. Patients with more extensive
alopecia at presentation may not have optimal response to DPCP.
¢ 1.Safavi KH, Muller SA, Suman VJ, Moshell AN, Melton LJ, 3rd. Incidence of alopecia areata in Olmsted County, Minnesota, 1975 through 1989. Mayo Clin Proc. 1995;70(7):628-33.
¢2 Happle R, Hausen BM, Wiesner-Menzel L. Diphencyprone in the treatment of alopecia areata. Acta Derm Venereol. 1983;63(1):49-52.
Country: Malaysia
Institute Of Paediatrics Hospital Kuala Lumpur
Poster View Here
Pattern Of Paediatric Dermatology Inpatient Consultations In A Tertiary Care Hospital In

Malaysia
Introduction and Background:

Although Paediatric Dermatology practice remains mainly outpatient, inpatient consultations are
frequently requested. These referrals provide valuable diagnostic and management information to
improve patient care.
Objectives:
This study endeavours to describe the pattern of dermatological referrals and the major Paediatric
subspecialties req uesting them.
Methods:
A retrospective review of referral data from January 2012 to December 2016 was carried out in the
paediatric dermatology department of Hospital Kuala Lumpur. The patientsâ_™ demographic data,
consultation diagnoses and information on the requesting subspecialties were collected.
Results:
A total of 688 inpatient referrals were received during the study period of 5 years. Fifty eight percent
(n=403) of patients were males and they range in age from newborn to 18.5 years old. Seventy eight
percent (n=536) of patients were from the Malay ethnic group, followed by Chinese, 8.2 % ( n=57) and
Indians, 7.9% (n=55). The commonest reason for Paediatric dermatology referral was skin infections,
which made up 36.5% (n=215) followed by atopic dermatitis, 16.1% (n=95) and autoimmune causes
and other dermatitis at 8.6% (n=51) respectively . Genodermatoses and vascular anomalies comprise
28% each of neonatology referrals. Half of the consultations requested (50%, n=345) were made by
the general Paediatrician, followed by the neonatal team (15%, n=105)and the oncology team (13.9%,
n=96).
Conclusion:
Our study revealed that the common dermatoses account for a majority of dermatologic inpatient
referrals in our centre. Skin infection remains the most frequently encountered reason for referral. There
is a need for further training of general Paediatricians in identifying and managing common skin
infections to improve the care of patients.
Poster: BCI-10061
Pattern of Paediatric Dermatology Inpatient

Consultations in a Tertiary care hospital in Malaysia.
WONG HL, LEONG KF, SABEERA BKI
PAEDIATRIC DERMATOLOGY DEPARTMENT, INSTITUT PEDIATRIK HOSPITAL KUALA LUMPUR
Introduction
Although Paediatric Dermatology practice remains mainly outpatient, inpatient consultations are
frequently requested. These referrals provide valuable diagnostic and management information to improve patient care.
Objective Methods
A retrospective review of referral data from January 2012 to
This study endeavours to describe the pattern of December 2016 was carried out in the paediatric
dermatological referrals and the major Paediatric dermatology department of Hospital Kuala Lumpur. The
subspecialties requesting them. patients’ demographic data, consultation diagnoses and
information on the requesting subspecialties were
collected.
Results
2012 2013 2014 2015 2016 Total

General Paeds 98 72 31 64 80 345
NICU/SCN 16 25 6 28 30 105
Oncology 21 23 10 19 23 96
PICU/PHDW 13 10 2 10 7 42
Orthopedic 5 3 2 5 7 22
Respiratory 4 4 1 7 3 19
Nephrology 8 2 0 3 5 18
Paeds Surgery 2 4 3 2 4 15
Nephrology 2 2 1 0 2 7
Others 9 2 1 4 3 20
A total of 688 inpatient referrals were received during the study period of 5 years. Fifty
eight percent (n=403) of patients were males and they range in age from newborn to
18.5 years old. The commonest reason for Paediatric dermatology referral was skin
infections, which made up 36.5% (n=215) followed by atopic dermatitis, 16.1% (n=95)
and autoimmune causes and other dermatitis at 8.6% (n=51) respectively .
Genodermatoses and vascular anomalies comprise 28% each of neonatology referrals.
Half of the consultations requested (50%, n=345) were made by the general
Paediatrician, followed by the neonatal team (15%, n=102)and the oncology team
(13.9%, n=96).
Discussions
The spectrum of Paediatric skin dermatoses in hospital settings has yet to be studied before in Malaysia. Our study not only reports the
common dermatoses amongst hospitalized patients but also the common specialties and subspecialties that request for Paediatric Dermatology
consultations in our centre. It is interesting to find that infections topped the list of referrals for the Paediatric age group whereas Genodermatoses
amd vascular anomalies were the most frequent referral by the neonatal team. The commonest infections referred were fungal infections ( Tinea
corporis, Tinea Capitis, Pityriasis Vesicolor etc) which contributes to 20%, followed by Bullous Impetigo of 17.6% then by scabies 12.5%. Atopic
dermatitis remained to be the second most often referred Paediatric dermatological cases in our settings. Most of the time, the referred Atopic
Dermatitis patients were complicated by secondary bacterial or viral infections e.g. Eczema Herpeticum or Coxsakium etc. In neonatal unit,
Epidermolysis Bullosa has been the most frequent genodermatosis encountered. For surgical specialties, the commonest conditions which prompted
the surgeons to make a dermatological referral were vascular anomalies which included venolymphatic malformations, lymphatic malformations and
birthmarks. In the Oncology setting, fungal infections, graft versus host disease and leukemic cutis are the dermatoses referred . In this study, the
common reasons for PICU and PHDW referrals were acute cutaneous drug reaction and purpura fulminans.
Conclusion
Our study revealed that the common dermatoses account for a majority of dermatologic inpatient referrals in our centre. Infection remains the
most frequently encountered reason for referral. There is a need for further training of general Paediatricians in identifying and managing common
skiOur study revealed that the common dermatoses account for a majority of dermatologic inpatient referrals in our centre. Skin infection remains
the most frequently encountered reason for referral. There is a need for further training of general Paediatricians in identifying and managing
common skin infections to improve the care of patients.
References
1. Eoin, et al. Experience of a year of adult hospital dermatology consultations. International Journal of Dermatology. Volume 54, Issue 10 October 2015. 1150-1156.
2. Abilasha et al. Pattern of Inpatient Dermatology Consultations in a Tertiary Care Centre from Northern India. Journal of Clinical and Diagnostic Research, 2016.Vol-10(12): WC07-WC10.
3. Afsar FS, Analysis of Pediatric Dermatology in patient consultations in a pediatric teaching hospital. Arch Argent Pediatr. 2017 Dec 1;115(6):e377-e384.
Track: Fungal infections
Contact Author: Cheryl JL Lie
Country: Singapore
Organization: KK Women's and Children's Hospital
Poster View Here
Retrospective review of paediatric dermatology patients with dermatophytosis
Introduction/Objectives:
Dermatophytes are a group of fungi capable of invading and growing in keratinized structures of the
skin. They comprise of three genera- Microsporum, Epidermophyton and Trichophyton. Topical therapy
is used for most infections, and can be used in combination with systemic antifungals. Treatment with
systemic antifungals often poses concerns regarding duration and safety.
Our study aims to review the characteristics, management and outcomes of paediatric patients
diagnosed with dermatophytosis in a tertiary hospital.
Materials/Methods:
We conducted a 12-year retrospective study of children with skin lesions and positive cultures for
dermatophytes. Cases were identified through microbiological laboratory records.
Results:
34 patients were identified, of which 25 were females and 9 were males. The mean age of presentation
was 7.24 years, ranging from 2 months to 16 years. Racial distribution showed a preponderance of
Malays(53%), Chinese(41%) and Indians(6%).
The most commonly affected site was the scalp(38.2%), followed by the groin/buttocks(20.6%),
feet(11.8%), trunk/limbs(11.8%), face(8.8%) and nail(2.9%). 2 patients(5.9%) had involvement of
multiple sites.
Fungal cultures were performed for all patients while fungal microscopy was performed in all but 2
patients. While 100% of the cultures were positive, only 50% of the microscopies yielded positive
results. Trichophyton was the most commonly identified species(64.7%), followed by
Microsporum(29.4%) and Epidermophyton(5.9%).
14 patients were treated with topical monotherapy, while 19 were treated with combination topical and
oral therapy. Mean duration of therapy was 5 months. Most cases of tinea capitis were treated with
combination therapy(86%). Among those who reported complete resolution/improvement, 53.3%
received combination therapy. No side effects were reported.
8(23.5%) patients reported complete resolution, while 7(20.6%) had partial resolution. 19(55.9%)
patients defaulted follow-up.
Conclusions:
Dermatophytosis is rare in our Asian paediatric population. Topical or systemic antifungals with
antidermatophyte activity are effective with minimal/no side effects. Combination therapy should be
considered in extensive disease or tinea capitis to avoid complications such as kerion formation and
scarring alopecia.
Poster: RSMPD26
Contact Author: Yustian DevikaRakhmawati
Country: Indonesia
dr. Saiful Anwar Regional General Hospital,

Malang, East Java Indonesia
COLLODION BABY WITH VITAMIN D DEFICIENCY AND ORAL CANDIDIASIS
Abstract
Background: Collodion baby is a rare congenital disorder characterized by parchment-like taught

membrane covering the whole body. The thickened skin of collodion baby acts as a physical block for
UV penetration that suppress cutaneous vitamin D synthesis. Vitamin D has an immune-modulatory
effects and a low level of vitamin D may influence host susceptibility to infections, especially candida
infections.
Case Presentation: A newborn, male, presented with plastic-like membrane encasing the entire body.
The patient was born at 39 weeks of gestation with birth weight 2900 grams and Apgar score 7-8. There
was no history of cosanguinity in the parents. Dermatological examination showed parchment-like
membrane covering the whole body, accompanied by fissure, ectropion, eclabium, auriculla deformity,
and sausage-shaped fingers. In the oral mucosa, a white pseudo-membrane was obtained and Gram
staining showed pseudohyphae and budding yeast. Culture examination showed the presence of
Candida albicans. Examination of vitamin D levels showed vitamin D deficiency (<5 ng/ml). The patients
was diagnosed with collodion baby, vitamin D deficiency and oral candidiasis. The treatment was
carried out in NICU with 40-60% incubator humidity, intravenous fluid, emollient petrolatum, eye care,
topical antibiotics in fissure lesions, systemic antibiotics, nystatin drop, and vitamin D supplement.
During treatment, the lesions were contaminated by MRSA and Klebsiella ESBL bacteria. Evaluation
at age 11 weeks showed completely a normal skin. White pseudo-membrane was no longer found in
the patient’s oral mucosa and Gram staining did not show any pseudohyphae or budding yeast.
Examination of vitamin D level showed a significant increment (37.10 ng/mL).
Discussion: In only approximately 10% of collodion baby, the skin will eventually spontaneously
improve. Oral candidiasis frequently found in up to 80% of newborn. Vitamin D can increase the
secretion of cathelicidin which will disrupt the sterol cell membrane of fungi. This will cause fungal cell
lysis. Clinical improvement occurs in concordance with the level of vitamin D and the improvement of
oral candidiasis, in which the defence against candida infection increased when the activity of antifungal
is improved.
Keywords: collodion baby, vitamin D deficiency, oral candidiasis

Contact Author: Lynette Wee
Country: Singapore
Compound Heterozygosity Of ITGB4 Mutations With A Splicing Error In Junctional

Epidermolysis Bullosa With Pyloric Atresia And Severe Gastrointestional And Tracheolaryngeal
Manifestations.
Inherited epidermolysis bullosa (EB) is a rare genodermatosis characterised by mechanical fragility of

epithelial tissues in the skin and mucous membranes, resulting in recurrent blistering and erosions
which are poorly- healing. There are four major groups based on the ultrastructural level within which
blisters develop in the affected tissues (EB simplex, junctional EB, dominant dystrophic EB and
recessive dystrophic EB). Junctional epidermolysis bullosa with pyloric atresia (JEB-PA), a rare
subgroup, is an autosomal recessive disease caused by mutations in ITGB4 or ITGA6, which encodes
the integrin protein that plays a critical role in maintaining the integrity of skin and epithelial linings.
We present an interesting case of JEB-PA that had mainly affected her gastrointestinal and
tracheolaryngeal mucosa. Immunofluorescent mapping of the patient’s gut showed absent staining for
ITGB4. She also had complications that involved her eye, urogenital system and development.
Expertise from more than 10 different disciplines were required to diagnose and manage this complex
and challenging case. The patient required chronic invasive ventilatory support and long- term
parenteral nutrition to support her growth which was failing secondary to severe gastrointestinal
disease.
The patient’s phenotype is caused by two variants- a heterozygous frameshift variant (c.794dupC) that
is classified as pathogenic according to the Human Gene Mutation Database (HGMD), and a
heterozygous synonymous variant (c.1608C>T/p.Cys536Cys) that may cause abnormal splicing. The
latter variant is a common polymorphism in East Asians but has not been reported previously in ITGB4.
Currently, there are 11 splicing variants listed in the HGMD with JEB-PA and this case further expands
the genotype-phenotype correlation for this rare genodermatosis.
Contact Author: Hanifati Sonia
Country: Indonesia
Faculty of Medicine Universitas Indonesia/Dr. Cipto

Organisation:
Congenital And Infantile Erythrodermic Psoriasis: Two Case Reports
Background
Erythroderma in infant is a rare condition that may be caused by ichthyosis and inflammatory disorders,
such as seborrheic dermatitis, atopic dermatitis and psoriasis. Etiological diagnosis is challenging due
to similar clinical findings. Congenital and infantile erythrodermic psoriasis are very rare. In 1995, a
study stated that there were 14 cases of congenital erythrodermic psoriasis from 1976, while another
study published in 2008 stated only 9 cases of congenital psoriasis reported worldwide.
Histopathological examination may aid and still becomes the cornerstone of the diagnosis. Thus, we
reported 2 cases of congenital and infantile psoriasis.
Case Presentation
The first case was a 2-month-old baby boy, who came to our outpatient clinic with erythroderma since
birth. The second case was an 11-month-old baby boy presented with generalized erythema since 2
months ago. Both babies were otherwise healthy, full term, born from healthy mothers, and had no
history of collodion. Histopathological findings were consistent with psoriasis i.e. regular acanthosis,
parakeratosis, suprapapillary thinning, hypogranulosis, and dilated papillary capillaries. Munro
microabscess was found in second case. Clinical improvement was achieved by emollients and topical
steroid, further proving that both cases were psoriasis.
Discussion
The etiological diagnosis of erythroderma in infant is difficult. Consistent histological findings of
erythrodermic psoriasis in infant and children are regular acanthosis and dilated papillary capillaries,
while Munro microabscess and spongiform pustule of Kogoj are not mandatory to establish the
diagnosis. Early diagnosis is important to perform adequate treatment because the disorder tends to
persist and some complications may arise such as dehydration, hypoalbuminemia, electrolyte
imbalance, infection, and failure to thrive.
Keywords: psoriasis, erythroderma, congenital, infantile

Country: Malaysia
Blue Rubber Bleb Naevus Syndrome Presenting As An In Utero Facial Tumour- A Case Report
BACKGROUND
Blue rubber bleb naevus syndrome (BRBNS) is a rare multifocal venous malformation syndrome
involving predominantly the skin and gastrointestinal tract. We report a case of BRBNS presenting as
a facial tumour in utero for its rarity and atypical presentation which casts a diagnostic dilemma.
CASE PRESENTATION
This is a 9- month-old Malay girl who presented with a facial mass during routine antenatal scan at 35
weeks of gestation. A fetal MRI done showed an exophytic mass with features suggestive of vascular
tumour. At birth, there was a pedunculated violaceous tumour found at the right forehead extending to
the right upper eyelid, measuring 7x8cm. It was compressible, soft in consistency and non tender. The
clinical impression was haemangioma and oral propranolol was started at day 4 of life.
However, the tumour increased in size disproportionately to growth and she developed one episode of
fresh per rectal bleed despite treatment with propranolol for 2 months and hence it was stopped. MRI
of brain, orbit and face was repeated at 4 months of age and there was evidence of slow flow vascular
malformation which was confirmed by angiogram. In view of the development of new bluish naevi over
other parts of the body and further episode of bleeding from the tumour, the diagnosis was revised to
Blue Rubber Bleb Naevus Syndrome which was supported by HPE findings.
DISCUSSION
BRBNS is a rare vascular anomaly syndrome consisting of multifocal venous malformations . The
cutaneous lesions of BRBNS are generally multiple, small (measuring less than 2 cm), and blue to
violaceous in color. In our case, a large facial tumour as the sole presentation during antenatal period
is the first case ever reported in history and hence a diagnostic challenge. The site of this lesion (
extending from the right forehead to the upper eyelid) makes the biopsy or excision difficult. The biopsy
was only done at 6 months of age when she started to develop other cutaneous lesions around her
body.The HPE results finally helped in cinching the diagnosis of venous malformation. Sirolimus was
started while awaiting surgical excision.
Contact Author: Diane Tantia Sari
Country: Indonesia
dr. Saiful Anwar Regional General Hospital, Malang,
East Java Indonesia
A CASE OF JUVENILE DERMATOMYOSITIS COMPLICATING CALCINOSIS AND

HYPERIMMUNOGLOBULINEMIA E SYNDROME
Abstract
Background: Juvenile dermatomyositis (JDM) is a unique idiopathic autoimmune disorder resulting in

chronic inflammation which manifests as muscle weakness and pathognomonic skin rash. The
estimated annual prevalence is 0,19 in 100,000 children. Calcinosis may provoke significant morbidities
in JDM, such as joint contractures, cutaneous ulcerations and susceptibility to Staphylococcus aureus
infections. It contributes to elevated IgE serum levels, which is amongst the classic signs of
hyperimmunoglobulinemia E syndrome (HIES).
Case Presentation: A 5-year-old girl had suffered from recurrent itching and multiple skin bumps,
marasmus, and symmetrical joint contractures since three years prior to admission. Multiple hyper- and
hypopigmented patches, pustules, cold abscesses, erosions, and ulcers were dispersed over the whole
body, and some skin hardening was found on palpation. Heliotrope rash and Gottron papules were also
found. Manual muscle testing (MMT) on upper and lower extremities was 3/3. There was limited range
of motion (ROM) on proximal and distal joints. Staphylococcus aureus grew positive on pustule lesion
culture. Laboratory investigations revealed elevated muscle enzymes and extremely high levels of
serum IgE. Radiograph examination showed soft tissue calcification and pneumonia, whilst EMG
suggested myopathy. Dermal calcinosis found on biopsy confirmed that it could be a part of
dermatomyositis. We established the diagnosis of JDM with HIES, with comorbidities of tinea capitis
and scabies. We prescribed systemic corticosteroids, physiotherapy, and treatment for the
comorbidities as per guideline. A five-month follow-up showed improvements in muscle weakness and
mobility, healthy levels of muscle enzymes, and decreased serum IgE levels.
Discussion: We established the diagnosis of JDM in this patient based on the presence of typical rash
and three signs within the modified Bohan-Peter criteria. The basis of HIES diagnosis was recurrent
pruritic skin infection, pulmonary infection, and high serum IgE levels. Generalised calcinosis
suppresses granulocyte chemotaxis, leading to susceptibility to staphylococci, which raises serum IgE
concentrations in JDM patients. Thus, the case reflected a pathophysiological association between JDM
and HIES.
Keywords : juvenile dermatomyositis, calcinosis, hyperimmunoglobulinemia E syndrome.

Contact Author: Heah Sheau Szu
Country: Malaysia
Organisation: Paediatric Institute, Hospital Kuala Lumpur
MANAGEMENT OF RECURRENT ERYTHEMA NODOSUM LEPROSUM IN A CHILD
The occurrence of erythema nodosum leprosum (ENL) is a type 2 reaction in a lepromatous or

borderline lepromatous leprosy patient. ENL presents as painful cutaneous nodules with multiple
systemic inflammation and can mimic a sepsis picture. It usually runs a recurrent and chronic course,
hence poses a challenge in the long term management.
We describe a 12 year-old girl who was diagnosed with lepromatous leprosy since the age of 6. She
developed multiple episodes of ENL for the past 2 years. In the beginning, she failed to comply to the
standard WHO MDT (Multibaciliary) treatment due to side effects of treatment which led to prolonged
courses of MDT. ENL episodes started after a few years after, presented as multiple painful skin
nodules, neuritis and high fever with lethargy. Treatment courses include high dose of clofazimine and
prednisolone, NSAIDS for symptomatic relief, a trial of Azathioprine and lastly Thalidomide. Though
prednisolone rapidly controlled her symptoms but she became cushingnoid with other steroid toxicity
complications. Unfortunately ENL relapsed upon tapering or stopping prednisolone. Thalidomide seems
to control the disease better with longer remission though this drug needs special approval on case
basis due to its teratogenicity side effect. Besides the drug choices, there are many factors which
contribute to the difficulty in this patient’s management especially the poor socio-economic
circumstances, poor drug adherence and lack of understandingof this chronic disease in the community.
Contact Author: Benny Loo
Country: Singapore
Organisation: KK Women’s and Children’s Hospital
Identification Of Compound Heterozygous ABCA12 Mutations In A Patient With Harlequin

Ichthyosis
Background:
ABCA12 (ATP binding cassette subfamily A member 12) encodes a keratinocyte lipid transporter which
is needed to maintain the layers of lipids within the epidermis to prevent dehydration and for normal
development of the skin. Mutations in the gene are known to cause autosomal recessive congenital
ichthyoses including harlequin ichthyosis, congenital ichthyosiform erythroderma (CIE), and lamellar
ichthyosis depending on the type and location of the mutations.
Case presentation:
The female infant was born to a non-consanguineous couple of Chinese ancestry at 40 weeks gestation
via Caesarean section for breech presentation. She weighed 3080 grams and had APGAR scores of 5
at one minute and 8 at five minutes. Antenatal ultrasonographic assessments did not detect any foetal
abnormalities. Her two older siblings were normal.
At birth, her scalp, face and entire body surface was covered with a thick, plate-like collodion membrane;
her eyelids and lips were everted, and her hands and feet were small and constricted by a wax-liked
cast of extremely tight skin. No respiratory support was required. She was placed in a high humidity
incubator with frequent application of white soft paraffin over her entire skin surface.
Genetic analysis identified a heterozygous single nucleotide deletion (c.6858delT (p.Phe2286fs)) and
a heterozygous single nucleotide substitution (c.5884G>A (p.Gly1962Ser)) in the ABCA12 gene (NM
173076.2). Parental studies showed that the frameshift variant is paternally inherited while the missense
variant is maternally inherited.
Abstract ID: ACN-10005
Track: Acne and Acneiform Disorders
Contact Author: Marius Rademaker
University of Auckland Medical School, Waikato
Clinical Campus
Isotretinoin In Young Children. Very Effective And Safe, But Relapse Common.
Introduction: Isotretinoin is the most effective treatment of acne, but data on its use in young children is
limited.
Methods: Retrospective review of children aged 12 years and under, started on isotretinoin for acne
vulgaris over a 5 year period.
Results: 36 children [17 girls, mean age 10.9 (range 4-12 years); weight 40.8kg (range 23-53 kg)] were
started on isotretinoin. Average length of treatment was 26 weeks (range 13–52 weeks), average daily
dose 25.8mg (range 10-40 mg), mean dose/kg body weight was 0.60 mg/kg/day (range 0.1-0.95
mg/kg/day), and mean cumulative dose was 108mg/kg (range 27-162 mg/kg). Additional antibiotics
were prescribed in 10 children (trimethoprim -7, erythromycin -3).
Outcome: 33 (92%) children’s acne cleared completely, two had moderate improvement (both stopped
early because of adverse effects), and one child’s acne was controlled, but only whilst on medication.
Five (14%) children reported no adverse effects; mild in 26 (72%) and moderate in five (14%). Adverse
effects included cheilitis (31 children), nose bleeds (5), mood change (5), tiredness (4), skin fragility (3),
muscle aches (2), eczema/dry skin (2), and x1 each of staph infection, asymptomatic abnormal liver
function tests, periungual granuloma, gingival hyperplasia, faint, with one child doing a temporary run
away from home.
Over an 18 year follow-up period, 17 (47%) had a subsequent course of isotretinoin: 8 (22%) a 3rd
course, 4 (11%) a 4th course and 2 (6%) had 5 courses.
Comparing the 17 children who required a 2nd course, on average they had received a larger and
longer initial course of isotretinoin (120.4 mg/kg cumulative dose vs 96.6 mg/kg; 27.2weeks vs 25.6
weeks). Only three (18%) of the children receiving a 2nd course had received additional antibiotics
compared to 7 (37%) of those who only had one course. Other characteristics including age, weight
and sex were similar.
Conclusion: Isotretinoin is effective and well tolerated but relapse over a 5 year follow-up period was
common. This relapse is a reflection of acne vulgaris in young children, rather than any specific dose
of isotretinoin.
Atopic Dermatitis and Other Eczematous
Track:
Conditions
Contact Author: Nirmala Ponnuthurai
Country: Malaysia
NUTRITIONAL ASSESMENT OF INFANTS WITH SEVERE ATOPIC DERMATITIS: A

PROSPECTIVE OBSERVATIONAL STUDY
Nirmala Ponnuthurai, Sabeera B
Department Of Paediatric Dermatology, Hospital Kuala Lumpur, Malaysia
Background
Atopic Dermatitis (AD) is one of the most common skin disorders in children. This study was conducted
to observe the demographic background in infants with severe AD, their nutrition, and associated micro
and macronutrient deficiencies together with the infant dermatological quality of life (IDQOL).
Methods
A prospective observational study was done on infants with severe AD less than 18 months old admitted
to the Paediatric ward Hospital Kuala Lumpur for 12 months duration. Data was collected using Data
Collection sheet which recorded the demography, feeding pattern, growth, blood investigations for
micro and macronutrients including RAST for common food allergens. Caretakers were requested to fill
IDQOL questionnaire.
Results
A total of 35 infants were recruited in the study. M:F ratio was 1.9:1 aged from 3 months till 16 months.
All the infants were born term with no neonatal problems. 77% babies were breast fed while 17 % were
on mixed feeding. 34% had not weaned to solids while 60% started weaning at 6 months. 63% of
parents practiced food restriction, of these commonest food restricted were seafood, egg, chicken and
dairy products. 34% infants had normal weight and Z score, 23% were moderately malnourished and
43% were severely malnourished. 43% infants had complications of neurodevelopmental regression
which was proportional to the severity of malnutrition. 82% had normal haemoglobin levels. 66 % had
low urea levels however serum creatinine levels were normal in all. Majority had low phosphate and
albumin levels while all infants had low serum zinc and Vit D levels. High total Ig E levels of > 5000ku/L
were seen in 47% .The common allergens tested by RAST with levels >100 ku/L in descending order
are hen’s egg white, peanut, cow’ milk and wheat. The IDOL index showed that 100% of the infants
have a moderately severe impaired quality of life.
Conclusion
In the studied sample we found a high prevalence of malnutrition with corresponding poor z-score
values in infants with severe AD. Food avoidance practice further aggravated this condition. Majority of
the infants had low albumin and phosphate levels while all had low serum vitamin D and Zinc levels .
Track:
Conditions
Contact Author: Jenny Yen LingTan
Country: Malaysia
Poster View here
Contact Sensitization In Children With Atopic Dermatitis Presented To Paediatric Dermatology

Clinic, Institute Of Paediatric, Hospital Kuala Lumpur
Introduction
Atopic dermatitis is a common skin disease that mainly affects children, with prevalence rates of up to
20%. Many factors influence this disease in a negative way and contact allergy is one such factor. There
is only scarce literature data on contact allergy in children with atopic dermatitis.
The diagnosis of contact allergy requires a careful evaluation of the patients’ clinical history, physical
examination and skin testing. Patch testing is the gold standard diagnostic test.
Objective
To assess the pattern of contact sensitization in children with atopic dermatitis
Methods
A total of 40 patients aged 7-18 years old with atopic dermatitis underwent patch testing on European
Standard Series, Shoes Series and Rubber Chemicals Series.
Results
26(65%) patients had at least one positive patch test reactions. The most common allergens yielding
positive results were nickel sulfate, potassium dichromate and 4-phenylenediamine base.
Conclusion
Patients with atopic dermatitis should be patch tested when indicated because they also develop
contact allergic sensitization to a significant degree. Our observations indicate that patch testing with
standard allergens adds valuable information about contact sensitivity in these patients.
Poster: ADE-10051
Contact sensi za on in children with atopic derma s, presented

to Paediatric Dermatology Clinic, Hospital Kuala Lumpur
Jenny TYL1, Leong KF1, Sabeera BKI1
1Department of Paediatric Dermatology, Institute Pediatric Hospital Kuala Lumpur
Introduction
Atopic dermatitis (AD) is the most common inflammatory skin disease in childhood, affecting 15-30% of children. Many
factors influence this disease in a negative way, and contact allergy is one such factor. Children with atopic dermatitis may have
unacknowledged contact allergies contributing to their skin symptoms.
The diagnosis of contact allergy requires a careful evaluation of a patient’s clinical history, physical examination and skin
testing. Patch testing is the gold standard diagnostic test.
Objective Table 1: Demographic characteris c of
To assess the pattern of contact sensitization in children with atopic dermatitis the study popula on
Methods
This prospective, observational study was performed from July 2017 until October
2017 at Paediatric Dermatology Clinic of Hospital Kuala Lumpur.
A total of 46 patients aged 6-18 years old with atopic dermatitis underwent patch
testing on European Standard Series, Shoes Series and Rubber Chemicals Series. Study
population was divided into two age groups, reflecting the different exposures during
primary and secondary school.
Patch tests were performed using Finn Chambers on scanpore tape. All
substances were applied on the upper back and removed 48 hours after application. The
sites were examined at day 2 and day 4 according to the International Contact Dermatitis
Research Group guidelines.
Atopic dermatitis was diagnosed using criteria by Hanafin and Rajka. The severity
was assessed using Eczema Area and Severity Index (EASI); Mild <6, Moderate 6-17 and
Table 2: Frequency of posi ve patch tests
Severe >18. according to age group
Those with skin lesions on the upper back, erythrodermic, infected or severe
Age 6 - Age 13 -
eczema which does not allow discontinuation of oral antihistamine and/ or systemic 12 18
corticosteroid for at least 2 weeks before the scheduled examination were excluded. Total patch tested 24 16
Results One or more posi ve
Forty six patients with atopic dermatitis were enrolled in the study. Patch test was patch tests 16 10
not successfully performed in 6 patients as the patch test came off or patient bathed and Potassium Dichromate 8 5
wet the upper back before reading of result. 40 patients were evaluated. Nickel sulfate 6 3
Median age of participant was 11 years old. 26 (65%) patients had at least one Phenylenediamine Base 4 5
positive patch test reactions. 18 (69%) of them are boy. Most of them have mild eczema Formaldehyde 1 3
20(77%), 5 have moderate and only 1 with severe eczema. Cobalt chloride 1 2
The most common allergens yielding positive results were potassium dichromate 4-ter-butylphenol
13 (50%), nickel sulfate 9 (35%) and 4-phenylenediamine base 6 (23%). Formaldehyde Resin 2 1
Epoxy resin 2 0
Discussion Hydroquinone
Contact allergy is a common problem in children with AD. Overall prevalence of a Monobenzylether 2 0
positive reaction to at least one allergen in our study population was high (69%). The Fragrance mix 2 0
impaired skin barrier in AD facilitates the penetration of potential allergens. The dry,
inflamed skin of people with AD requires frequent application of emollients, corticosteroids Diaminodiphenylmethane 1 0
and antibacterial creams that may result in contact sensitization to those allergens. Diphenylthiourea 1 0
Our study also show AD is associated with contact sensitization to metal allergens Glutaraldehyde 1 0
like chromium and nickel. Potassium dichromate contact sensitization in AD is associated Dibutylthiourea 0 1
with footwear contact dermatitis. Contact sensitization to nickel in children may result not 2-n-Octyl-4-isothiazolin-3-
only from jewelery, belt buckles and clothing fasteners, but also from toys and technological one 0 1
devices such as laptops and mobile phone. Phenylenediamine is found in hair dye, henna, Thiuram mix 1 0
textile dye, photocopy and printing inks. Clioquinol 1 0
We found that patch testing in children can be challenging, especially for the Balsam Peru 1 0
younger ones. The test requires cooperation from the child to keep the patch test in place Mercaptobenzothiazole 1 0
for 48 hours, to not wet the test area nor to conduct activities which would result in Colophony 0 1
excessive sweating. References
Limitation of this study is in the small sample size and the fact that it only involved 1. Wahlberg JE, Lindeberg M. Patch
1 study center which is a tertiary referral hospital. testing. In: Frosch PJ, Menne T,
Lepoittevin JP, eds. Contact dermatitis,
Conclusion 4 t h e d . B e r l i n : S p r i n g e r - Ve r l a g ,
Patients with atopic dermatitis should be patch tested when indicated, as they also 2006:365-390.
developed contact allergic sensitization to a significant degree. Our observations indicate 2. Simonsen AB. Contact allergy in children
with atopic dermatitis: a systematic
that patch testing with standard allergens often adds valuable information about contact review. British Journal of Dermatology
sensitivity in these patients. 2017; 177: 395-405
Abstract ID: GDT–10103
Contact Author: Bishnoi Priya
Country: Singapore
Organisation: KK Women’s and Children’s Hospital
Has this been published before: No
EB In Singapore - Brief Overview
Epidermolysis Bullosa (EB) is a genetic skin condition which is further subdivided in 4 subtypes based
on the level of cleavage. Making an early diagnosis helps in better management, prognostication and
for prenatal diagnosis in future. EB diagnosis is based on antigen-mapping, genetic analysis and
electron microscopy.
We received samples from 23 patients with a diagnosis of EB which were further investigated by
immunofluorescence and/or DNA sequencing based on the sample provided. Out of 23 only 7 patients
gave both blood and tissue samples and the remaining just blood. Antigen-mapping was helpful in
predicting the level of cleavage in 6 out of 7 cases (85.7%). Four out of 5 (57.14%) cases showed weak
to complete absence of protein based on antigen mapping. Molecular diagnosis was made in 80% of
the cases based on DNA sequencing out of which 10 were EBS, 3 JEB and 2 DEB. Out of 23 samples
4 still remain inconclusive and 4 are awaiting sequencing. Amongst the simplex subtype KRT5 and
KRT14 mutations were found in 6 and 4 patients respectively with KRT14p.R125C being the most
common mutation.
Antigen mapping is a useful tool for early diagnosis of EB and usage of an extended panel of antibodies
can further help in detecting the affected protein. This study also underscores the importance of
molecular diagnosis in the genetic characterization of this disease in the region.
Track:
Conditions
Contact Author: Purvis Diana
Organisation: Starship Children's Hospital
Poster View here
Longterm Effect Of Methotrexate For Childhood Atopic Dermatitis
INTRODUCTION
Methotrexate is increasingly used for treatment of severe paediatric atopic dermatitis (AD). This study
aimed to look at the effect of methotrexate on AD severity both while on treatment and afterwards.
METHODS
Medical records of all children treated with methotrexate for AD at Starship Children’s Hospital,
Auckland, New Zealand between 2011-16 were reviewed. Demographics, previous treatment, length
and dose of methotrexate treatment, hospitalisations and use of other systemic treatment were
recorded. Parents were subsequently contacted by phone to assess response to treatment, AD
relapses, use of additional systemic treatment, and completed a patient oriented eczema measure
(POEM).
RESULTS
Forty-three patients aged 2-16 years (median 10) were included. Four (9%) had previous phototherapy
or other systemic immunosuppressives, and 14 (33%) previous hospitalisation for AD (28 admissions).
Methotrexate was given at a median dose of 0.33mg/kg (IQR 0.26-0.40mg/kg) for a median duration of
17 months (2-53 months). Adverse effects were recorded for 4 patients, all gastrointestinal upset. No
blood monitoring abnormalities occurred. Post methotrexate initiation only 6 (14%) were subsequently
hospitalised (9 admissions).
Telephone follow-up occurred in 30 patients, median 29 months post-methotrexate (IQR 14-45months).
Seven (23%) reported a change or a slight improvement on methotrexate, and 23 (77%) reported that
AD was a lot better. After stopping methotrexate only 15 (50%) reported AD recurrence, 10 (33%)
reporting the recurrence to be as severe as the pre-methotrexate condition, 8 (27%) had recommenced
systemic treatment (6 methotrexate, 2 cyclosporin). Median POEM at follow-up was 6 (IQR1-17).
Twenty-two (73%) had a POEM score <17 indicating mild-moderate AD, with only 8 (27%) having a
POEM of 17 or greater, indicating severe disease and 11 (37%) having a score <3 indicating they were
clear or almost clear. Two of the 13 children not available for telephone follow-up were known to have
had further systemic treatment.
CONCLUSION
Methotrexate treatment for severe AD appeared to be effective in three quarters of patients treated,
with both decreased severity scores and reduced hospital admissions. These improvements were
evident at a median of more than two years follow-up, suggesting persistance of treatment effect
following discontinuation of treatment.
Poster: ADE-10092
Long term effect of methotrexate

for childhood atopic dermatitis
M Lee1, D Purvis2,3, K Agnew2, N Birchall2, S Dalziel2,3
1 Auckland City Hospital , 2 Starship Children’s Health, 3 University of Auckland, New Zealand
Introduction Figure 1 Patient flow Figure 2

• The use of systemic immunosuppressants such as
methotrexate (MTX) for severe atopic dermatitis (AD) in
POEM score at a median
childhood is included in treatment guidelines but there is follow-up of 29 months
limited data. Although benefit on MTX has been reported, 43 patients after stopping MTX n=30
long-term outcome after stopping MTX is unknown.
• We have used MTX as a firstline systemic agent for the
treatment of children with severe AD for over 7 years in
38 completed a course of 5 patients still on
tertiary paediatric dermatology clinics. MTX treatment
• The aims of this study were to assess the effect of MTX

while on treatment, and to report long term outcome after
stopping MTX treatment.
8 patients unable to be
30 patients contacted
contacted
Methods
• Medical records of all children treated with MTX for AD at
Starship Children’s Hospital, Auckland, New Zealand 5 – ‘no change’ at end 2 – ‘slight improvement’ 23 – ‘a lot better’ at end
of MTX treatment at end of MTX treatment of MTX treatment
between 2011-16 were reviewed.
• Demographics, previous systemic treatments, duration
and dose of MTX treatment, adverse events and 1 restarted MTX
hospitalisations were recorded. 2 ciclosporin
1 azathioprine No recurrence of AD 10 AD returned 13 no recurrence of AD
• MTX (once weekly oral dose) was offered if severe AD was 1 no further systemic
treatment
Clear 0-2 33%
not controlled with topical corticosteroids and emollients.
Mild 3-7 23%
• Topical therapy was continued as needed.
• Laboratory monitoring of full blood count, liver and renal 4 restarted MTX
Moderate 8-16 13%
function was performed initially weekly then 1-3 monthly.
Severe 17-24 23%
• Telephone follow-up of parents in 2017 recorded child’s
response to MTX treatment, AD relapses, additional Very severe 25-28 3%
systemic treatment and current Patient-Oriented Eczema 6 no further systemic
treatment
Measure (POEM) score over the last week (0-28).1
• Response to MTX was graded as:

(i) a lot worse
Table 1 Table 2
(ii) slightly worse
(iii) no change Demographics and MTX treatment Telephone follow-up
(iv) slightly better Demographics N=43 N (%) Parent reported outcome N=30 N (%)
(v) a lot better or minimal/no AD Gender Male:Female 21:22 (Male 49%) Time to telephone follow- Median (months) 29 (IQR 14-45)
Ethnicity European 19 (44%) up after stopping MTX
AD at end of MTX ‘A lot better’
• POEM score: Maori/Pacific 12 (28%)
treatment
23 (77%)
‘Slightly better’ 2 (7%)
0-2 clear or almost clear of AD Chinese/SE Asian 7 (16%)
‘No change’ 5 (17%)
Indian 5 (12%)
3-7 mild AD ‘Slightly worse’ 0
Previous systemic Phototherapy 2 (5%)
8-16 moderate AD treatments
Cyclosporin 2 (5%)
‘A lot worse’ 0
AD recurrence in those Recurrence (any) 10 (40%)
17-24 severe AD Prednisone 1 (2%) responding to MTX n=25 ‘As bad as before MTX’ 6 (24%)
25-28 very severe AD Previous Children admitted 14 (33%)
Mild recurrence 4 (16%)
hospitalisations
Number of hospitalisations 28
No recurrence 15 (60%)
Age at starting MTX Median 10 Years (IQR 7-12)
Median time to recurrence 24 (IQR 10.5-28.5)
Results MTX dose Median weekly oral dose
(mg/kg)
0.33 (IQR 0.26-0.40) (months)
Systemic treatment
4 (16%)
• 43 patients were identified (Figure 1). Duration of treatment Median (months) 17 (IQR 7.5-20)
restarted
Systemic treatment after Cyclosporin 2 (7%)
• Demographic details are shown in Table 1. MTX Azathioprine 1 (3%)
• MTX treatment was started at a median age of 10 years, Adverse effects Gastrointestinal upset 4 (9%)
Restarted MTX 6 (20%)
Cataracts 1 (3%)
at median dose of 0.33mg/kg, for a median duration of POEM score at time of Median 6 (IQR 1-17)
Laboratory monitoring 0 follow up
17 months. abnormalities <3 ‘Clear or near clear’ 11 (37%)
• Hospitalisation for AD was required by 33% of children Hospitalisation after Children admitted 6 (14%) 3-16 ‘Mild-moderate AD’ 11 (37%)
starting MTX >17 ‘Severe AD’ 8 (27%)
prior to starting MTX, but only 14% after MTX (p=0.07). Number of hospitalisations 9
• MTX was generally well tolerated:

• 1 patient stopped due to GI side effects.
• 1 patient was diagnosed with cataracts, but had Discussion
prior treatment with periorbital topical • This is the first study to report long term outcome • A study of 15 children treated with cyclosporin found
corticosteroids and oral prednisone which were after stopping MTX treatment. that 12 (80%) responded to treatment, of whom 5
thought to be causative. • MTX treatment for severe AD was effective (42%) required further systemic treatment at around
• No other serious adverse events were reported. • improved AD in more than 80% of children two years follow up. 4
• Telephone follow-up occurred with 30 parents (70%) at a • half the number of children hospitalised for AD • Limitations of our study include retrospective design,
median of 29 months after stopping MTX (Table 2). • Of those who responded to MTX, 40% had a vulnerability to recall bias, lack of use of a
• No improvement on MTX was reported by 5 (17%). recurrence of AD and only 16% required further standardised AD severity measure while on MTX.
• A positive response on MTX was reported by 25 (83%), systemic treatment. • It is also possible that the long term improvement
with 23 (77%) ‘a lot better’ and 2 (7 %) ‘slightly better’. • Importantly, MTX was associated with ongoing found in our study represents a natural history of
• AD recurred in 10 (40%) of responders, at a improvement in AD to a median of 29 months after improvement of AD through childhood.
median of 24 months after stopping MTX. MTX was stopped. • Ideally, future studies of MTX for AD would gather
• Severe AD recurred in 6 (24%). • 73% had no, mild or moderate AD data prospectively using both objective and subjective
• Systemic treatment was restarted in 4 (16%). • A third remained clear/near clear of AD measures. Long term outcome should be included in
• Median POEM at follow-up was 6 (mild AD), with 22 • These findings agree with other studies of MTX for studies of systemic therapies, as AD is a chronic
(73%) having no, mild or moderate AD (Figure 2). childhood AD which report good efficacy and disease and long term outcomes are important for
tolerability of MTX while on treatment.2,3 families and clinicians in making treatment decisions.
Conclusion
MTX was effective in over 80% of children with severe AD, with decreased severity and reduced hospital admissions.
73% reported no, mild or moderate AD at a median follow up of 29 months after stopping MTX,
suggesting long lasting treatment benefit following discontinuation.
REFERENCES: 1 Spuls et al The Patient-Oriented Eczema Measure: development and initial validation of a new tool for measuring atopic eczema severity from the patient’s perspective. Arch Dermatol 2004; 140:1513-19. 2 Deo et al. Methotrexate for the treatment of atopic dermatitis in children and adolescents. Int J Dermatol
2014;53(8):1037. 3 Dvorakova et al Methotrexate for severe childhood atopic dermatitis, clinical experience in a tertiary centre. Pediatr Dermatol 2017;34(5):528. 4 Sibbald et al Retrospective review of relapse after systemic cyclosporine in children with atopic dermatitis Pediatr Dermatol 2015;32(1):36-40.
Contact Author: Jagadeesan Soumya
Country: India
Amrita Institute of Medical Sciences, Kochi,

Organisation:
Kerala,India
Topical Immunotherapy With Diphencyprone In Extensive Alopecia Areata In Children: A 3 Year

Experience From A Tertiary Centre In South India.
Background: Alopecia areata is a chronic inflammatory non-scarring alopecia directed against the
anagen hair follicle and can occur at any age. Even though majority of the cases in children are self-
limiting and localized, a sub-set of pediatric alopecia areata exists, which is extensive, progressive and
chronic, often requiring systemic immunomodulatory drugs. Topical diphencyprone(DPCP)
immunotherapy is used to treat refractory and advanced alopecia areata, which has been evaluated in
several studies. However, use of DPCP in children has been the focus of only a limited number of
studies.
Objective: To study the efficacy and safety profile of topical immunotherapy with DPCP in the treatment
of moderate to severe pediatric alopecia areata.
Methods: Children less than 18 years of age suffering from moderate-severe alopecia areata (>50%
scalp involvement), refractory to other modalities of treatment for at least 1 year, were recruited in the
study. The study received ethical committee approval. All children followed the same immunotherapy
protocol beginning with sensitization with DPCP2% followed by a treatment with DPCP0.0001%, 2
weeks later. Thereafter, treatment continued on a weekly basis with increasing concentrations of DPCP
depending on the response. A complete response was defined as full re-growth of scalp hair, and a
partial response was defined as any hair re-growth other than full re-growth. The data was entered and
statistically analyzed. Results: A total of 33 patients were studied with a male: female ratio of 1:1.25.
The average duration of disease was 11.2 months, average duration of treatment was 9.2 months;
average onset of response was at 5 weeks. 2 patients discontinued treatment mid-way. Complete
response was seen in 14(42 %) patients, partial in 16(48%) and 3 were non-responders. 13 patients
(39%) developed relapses, of which 4 developed during treatment, 6 during the tapering phase and 4
after stopping treatment. Adverse effects were minor including eczematous reactions, occipital
lymphadenopathy, depigmentation, urticarial or id eruptions and pigmented contact dermatitis. Only
one patient had a wide-spread eczematous reaction meriting discontinuation of the treatment.
Conclusions: Topical immunotherapy seems an effective and fairly safe treatment modality for
extensive alopecia areata in children. Limitations: Lack of long-term follow-up.

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