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Review

A global consensus statement on ashy dermatosis, erythema


dyschromicum perstans, lichen planus pigmentosus,
idiopathic eruptive macular pigmentation, and Riehl’s
melanosis
Sujith Prasad W. Kumarasinghe1, MBBS, MD, FACD, Amit Pandya2, MD, Veena
Chandran3, MD, DNB Dermatology, Michelle Rodrigues4, MBBS, FACD, Ncoza C. Dlova5,
6 7 8
MBChB, FCDerm, PhD, Hee Young Kang , MD, M. Ramam , MBBS, MD, Johannes F. Dayrit ,
9 10
MD, Boon Kee Goh , BSc, MBChB, FRCP, and Davinder Parsad , MBBS, MD

1
Department of Dermatology, Fiona Stanley Abstract
Hospital, Murdoch, WA, Australia, Ashy dermatosis (AD), lichen planus pigmentosus (LPP), erythema dyschromicum perstans
2
Department of Dermatology, University of
(EDP), and idiopathic eruptive macular pigmentation are several acquired macular
Texas Southwestern Medical Center,
hyperpigmentation disorders of uncertain etiology described in literature. Most of the
Dallas, TX, USA, 3Royal Perth Hospital,
Perth, WA, Australia, 4Department of published studies on these disorders are not exactly comparable, as there are no clear
Dermatology, St Vincent’s Hospital, Fitzroy, definitions and different regions in the world describe similar conditions under different
Vic, Australia, 5Department of Dermatology, names. A consensus on the terminology of various morphologies of acquired macular
College of Health Sciences, Durban, South
pigmentation of uncertain etiology was a long-felt need. Several meetings of pigmentary
Africa, 6Department of Dermatology, Ajou
University, Suwon, South Korea,
disorders experts were held to address this problem. A consensus was reached after
7
Department of Dermatology, All India several meetings and collation of e-mailed questionnaire responses and e-mail
Institute of Medical Sciences, New Delhi, communications among the authors of publications on the above conditions. This was
India, 8Department of Dermatology, achieved by a global consensus forum on AD, LPP, and EDP, established after the 22nd
Research Institute for Tropical Medicine,
International Pigment Cell Conference held in Singapore in 2014. Thirty-nine experts
Metro Manila, Philippines, 9Mount Elizabeth
Medical Centre, Singapore, and
representing 18 countries participated in the deliberations. The main focus of the
10
Department of Dermatology, deliberations was terminology of the conditions; as such, we present here the consensus
Postgraduate Institute of Medical Education statement of the forum and briefly review the available literature on the subject. We have
and Research, Chandigarh, India not attempted to discuss treatment modalities in detail.

Correspondence
Sujith Prasad W. Kumarasinghe, MBBS, MD,

FACD

Department of Dermatology
Fiona Stanley Hospital, 11 Robin Warren
Drive, Murdoch, WA 6150
Australia
E-mail: prasadkumarasinghe@yahoo.com

Conflicts of interest: None declared.

doi: 10.1111/ijd.14189

small or large macules of hyperpigmentation which have a gray


Background and Review of Literature
appearance and are deeper than epidermal pigmentary disor-
Thousands of patients world over suffer from different clinical ders such as melasma and lentigines.1–3 Some authors con-
and morphological types of patchy hyperpigmentation. These sider AD, LPP, and EDP as the same condition, whereas
disorders are of a greater clinical and psychological concern others consider them to be different.4–9 Furthermore, a similar
among dark-skinned populations (Fitzpatrick’s type III–VI). Ashy condition but with smaller macules of acquired hyperpigmenta-
dermatosis (AD), lichen planus pigmentosus (LPP), and ery- tion termed idiopathic eruptive macular pigmentation (IEMP)
thema dyschromicum perstans (EDP) present with acquired and an additional condition known as IEMP with papillomatosis 1

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2 Review Global consensus statement Kumarasinghe et al.

have also been described, and their relationship with AD, LPP, Blashkoid distribution whereas others were not. However, in our
and EDP is unclear.10,11 opinion, acquired macular hyperpigmentation in unilateral or
Blashkoid distribution should not be labeled as AD just because
EDP and AD of the color of the lesions.
Ramirez first described AD in 1957 in El Salvador.1 The name Various authors have elucidated the ultrastructural and
was coined because of the ashy blue-gray color of the lesions. immunopathologic features in patients with EDP/AD.35,39,40
The term EDP was used by Convit and colleagues in 1961 in a Ashy dermatosis lacks a well-defined histopathological picture
report of five cases in which the disease was characterized by and can be similar to other inflammatory disorders including
numerous small and large macules of a grayish color of varying lichenoid reactions.4
intensity, sometimes becoming confluent covering extensive Vasquez-Ochoa et al.39 in a study of their 43 patients
areas of the body.2 When the lesions were evolving, the mac- reported the presence of dermal lymphocytic infiltrate in all of
ules had a slightly raised, firm, erythematous border that felt like their cases, which was of perivascular distribution in 86%. Mela-
a thin piece of string. In 1957, Ramirez had reported 139 similar nophages were demonstrated in all the cases with vacuolization
cases, some of which had an easily observable nonelevated of basement membrane zone in 58% and exocytosis of lympho-
erythematous border.12 cytes in 53.5%. There was predominance of CD8+ T lympho-
The above two morphological types were generally thought to cytes (cytotoxic) in the dermis and HLA–DR+, ICAM-1+
be characterized by acquired small and large macules with keratinocytes in the epidermis. Exocytosis of cutaneous lympho-
ashy/gray discoloration and on histopathology by a lichenoid cyte antigen+ cells were observed in areas of BMZ zone dam-
perivascular infiltrate and pigmentary incontinence (me- age suggesting that antigenic stimulation may play a role in the
lanophages). The major difference between the above two is development of EDP.
the presence of an erythematous border in the early active Baranda et al.35 found that the expression of ICAM-1 and
phase of EDP. HLA–DR positivity in their patients disappeared after treatment
The etiology mostly remains unknown.1,2,13 There were with clofazimine. They found that the dermal cells expressed
attempts to identify the etiology of these conditions. Stevenson CD69 and the cytotoxic cell marker CD94.
and Miura reported a patient who had similar skin lesions with Tienthavorn et al.41 patch tested 36 patients with a provi-
associated intestinal parasitism and showed a therapeutic sional diagnosis of AD and LPP with histological findings of pig-
response to anti-parasite therapy.14 Chua et al., attributed the mentary alteration with lichenoid infiltration. Patients with a
pigmentation in their three patients to omeprazole.15 This was provisional diagnosis of AD and LPP had positive patch tests in
questioned by Chandran and Kumarasinghe who argued that 40% and 36% of cases, respectively. The common allergens
these cases should be labeled as drug-induced hyperpigmenta- implicated were nickel sulphate hexahydrate, fragrance mix,
tion rather than ashy dermatoses.16 EDP has also been associ- and cobalt. They highlighted the importance of getting a thor-
ated with enteroviral, hepatitis C infections and HIV ough history about systemic medications and topical contac-
seroconversion.17–19 There were reports associating EDP/AD tants and recommended performing patch testing in all cases
with intake of ethambutol, chlorothalonil, cobalt allergy, ammo- that present like AD or LPP. However, as nickel, cobalt, and fra-
nium nitrate, Chinese herbal extracts, fluoxetine, etc.20–25 A grances are common allergens, it is not clear whether these
study done on Mexican patients suggested genetic susceptibility positive patch tests are really relevant in the pathogenesis of
conferred by genes located within the major histocompatibility pigmented macules, as usually there is no history of preceding
complex region.26 pruritus or typical contact dermatitis. Furthermore, proven cases
The age range is from childhood through maturity, and both of clinically relevant typical contact dermatitis due to nickel,
sexes are affected equally.13,27–30 Unlike adult patients who are cobalt, or fragrances have not been reported to evolve into AD,
mostly Hispanic in origin, children are usually Caucasian. Other EDP, or LPP.
important factors in children are absence of consistent trigger Some authors have reported cases as EDP with pruritus prior
factors and eventual improvement or resolution in 50–69% of to the onset of lesions as well as scaling.42,43 In one case
prepubertal patients.27,29 The youngest pediatric patient reported as EDP, the patient had a pruritic rash which resem-
reported was 2 years old.30 bled pityriasis rosea and completely resolved.42 In our view, if
Different types of therapies have been employed with little or there is a definite history of pruritic pityriasis rosea-like eruption,
no benefit.1,2,13 it should not be categorized as AD but rather as postinflamma-
However, several authors have subsequently trialed various tory hyperpigmentation due to pityriasis rosea.
treatment modalities and reported success in some cases. Iso-
tretinoin,31 topical tacrolimus,32,33 dapsone,34 clofazimine,35 and The entity “LPP” and the continuing nosological
NBUVB36 have been reported to be successful in some cases. controversies
Some cases of “unilateral AD” have been reported mainly Although similar dermatoses had been reported in Japanese,
from Japan.37,38 Some of them were reported to have a French, and Italian literature, it was Bhutani and colleagues

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Kumarasinghe et al. Global consensus statement Review 3

who clearly described 40 Indian patients with acquired macular 3 Simulators: (i) LPP and actinic LP (ii) postinflammatory hyper-
pigmentation and coined the term LPP.3,6 These patients had pigmentation (iii) drug-induced melanodermas (iv) mastocytosis.
clinical and histological features similar to those of AD/ED. They
categorized LPP as a variant of lichen planus due to the pres- According to this classification, LPP also falls under ashy der-
ence of a lichenoid tissue reaction, the finding of colloid bodies, matoses.
and clinical association with different forms of lichen planus in
one-third of their patients. Naidorf and Cohen also proposed
Idiopathic Eruptive Macular Pigmentation
that EDP-like conditions should be labeled as erythema dys-
chromicum variant of lichen planus.5 Tschen et al. also sup- IEMP was initially described by Dego et al.53 and Sanz de
ported the view that LPP and EDP are the same entity.44 Galdeano proposed the diagnostic criteria.10 IEMP is charac-
Vega and colleagues4 observed some clinical differences terized by asymptomatic, nonconfluent brown macules involv-
between AD and LPP though their histopathological study did ing the neck, proximal extremities, and trunk with no preceding
not reveal any significant differences between them. inflammation or history of drug exposure.10 Histology is char-
Kanwar, Dawn, and Dhar in 1996 reported 55 patients with acterized by hyperpigmentation of the basal layer of the epi-
bilaterally symmetrical gray, blue, and brown to black persistent dermis and prominent dermal melanophages without visible
pigmentation of the face, neck, and upper trunk with tissue evi- basal layer damage or lichenoid inflammatory infiltrate and nor-
dence of lichenoid reaction and no typical clinical features of mal mast cell counts. Stinco and colleagues reviewed 26
lichen planus. They preferred to categorize this as an entity dis- cases and in the majority of them, the condition typically
tinct from lichen planus as none of the patients showed evi- regressed in several months or in a few years with no treat-
dence of lichen planus at any stage of the disease and ment required.54 The pigmented macules in most reports were
45
proposed the term “lichen dyschromicum perstans.” smaller in size (ranging from 5 to 25 mm) compared to the
Later in 2003, Kanwar and coworkers46 reported 124 Indian typical larger macules of AD. Joshi et al. reported 10 cases of
patients with slate gray to brownish-black pigmentation of the IEMP from India with features of acanthosis nigricans of some
face and neck. The pattern of pigmentation was mostly diffuse of the lesions and histology showing pigmented papillomatosis.
(77.4%), followed by reticular (9.7%), blotchy (7.3%), and peri- They proposed to classify IEMP to an eruptive form of acan-
follicular (5.6%). Lichen planus was noted in 19 patients with thosis nigricans.11,55
typical histopathological changes of this disorder. They pro-
posed that LPP is a distinct clinical entity commonly encoun-
Riehl’s Melanosis
tered in the Indian population and should be considered in the
spectrum of lichenoid disorders as a variant of lichen planus. This condition, first described by Riehl in 1917, is characterized
There have been reports of LPP in linear and segmental pat- by facial hyperpigmentation, most pronounced on the forehead
terns.47,48 A variant of LPP with associated annular patches and in the zygomatic and/or temporal region. Riehl suggested
with central pigmentation has been described in one report.49 In that certain foods used during World War I could be responsi-
2013, Dlova published for the first time 24 South African cases ble. Some authors consider Riehl’s melanosis almost synony-
of LPP associated with lichen planopilaris, in the frontal fibros- mous with pigmented contact dermatitis of the face. The
ing pattern.50 In this report, it was suggested that LPP could be commonest cause has been sensitizing chemicals in cosmet-
a harbinger of frontal fibrosing alopecia. Lichen planus pigmen- ics.56 In our view, if a definite relevant contact allergy is demon-
tosus inversus has been described as a variant of lichen planus strated in patients with finely reticulated pigmented lesions on
with mildly pruritic hyperpigmented macules and/or patches the face and neck region, the disorder may be labeled as pig-
involving intertriginous and flexural skin folds in light skinned mented contact dermatitis. In a study of 14 Korean women with
individuals.51 acquired bilateral melanosis of the neck consistent with Riehl’s
Subsequently, more cases with the above morphological melanosis, Park et al. have noted that although some cases
types have been reported in the literature, and the controversies showed positive photopatch tests, they were of doubtful rele-
continued.15,51,52 vance.57 Various treatments, individually as well as in combina-
7
Zaynoun et al. in 2008 attempted to alleviate confusion by tions, have been attempted with variable success in Riehl’s
their classification of these disorders. They proposed “ashy der- melanosis.58
matoses” as a broad category, which can be divided into:

The Global Consensus Forum and the


1 AD: Patients with idiopathic eruptive hyperpigmented mac-
Consensus Statement
ules, irrespective of the presence or absence of interface der-
matitis histologically at the time of examination. A consensus on the terminology of various morphologies of
2 EDP: Patients with lesions similar to those of AD, but who acquired macular pigmentation of uncertain etiology (MPUE)
have or have had lesions with erythematous borders was a long-felt need.

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4 Review Global consensus statement Kumarasinghe et al.

Objectives Table 2 List participants and the countries of the ashy


dermatosis, erythema dyschromicum perstans, and lichen
To arrive at a consensus on the terminology of various mor- planus pigmentosus global forum
phologies of acquired MPUE.
1. Australia: S. Prasad W. Kumarasinghe, Michelle Rodrigues, Adrian
Methods Mar, Veena Chandran
2. Brazil: Marcia Ramos-e-Silva
Establishment of the consensus forum and the forum 3. China: Flora Xiang
meetings 4. France: Michelle Verschore
5. India: Davinder Parsad, Rashmi Sarkar, M. Ramam, Binod Khaitan,
The consensus was planned and proposed by a forum headed
Deepti Ghia, Saumya Panda, Siddharth Sonthalia, Mala Bhalla
by coconveners Professor Prasad Kumarasinghe (SPK) and
6. Italy: Torrello Lotti
Professor Davinder Parsad with the support of the Asian Soci- 7. Japan: Chika Ohata
ety for Pigment Cell Research. 8. Kuwait: Nawaf Al-Mutairi
The consensus forum was established after a panel discus- 9. Mexico: Judith Dominguez-Cherit, Jorge Ocampo, Maira Herz
Ruelas, Zulema Olazaran Medrano
sion on the same subject which was held during the Interna-
10. Saudi Arabia: Sanjeev Mulekar
tional Pigment Cell conference (IPCC) held in Singapore in
11. Singapore: CheeLeok Goh, Boon Kee Goh, Steven Thng
2014.59 After this first meeting, authors of publications on AD, 12. South Africa: Ncoza Dlova
EDP, and LPP were contacted by the conveners if an e-mail 13. South Korea: Hee Young Kang, Kyoung Chan Park
address could be obtained. Several rounds of questionnaires 14. Thailand: Pailin Puangpet
15. Taiwan: Eric Lan
were circulated to understand the views of clinicians, authors of
16. The Philippines: Johannes Dayrit, Evangeline Handog
papers, and researchers from different regions of the world. Dr.
17. The Netherlands: Marcel Bekkenk
Veena Chandran collated the questionnaire responses. E-mail 18. United States of America: Amit Pandya, Robert Schwartz, Seemal
communications were very useful, as all the members of the Desai, Iltefat Hamzavi, Marsha Henderson
group could not attend all the meetings.
The second meeting was held during the World Congress of
Dermatology (WCD), Vancouver, Canada, in June 2015, and Results
the third meeting was held during the American Academy of
Having the consensus deliberations during three different but
Dermatology (AAD) Meeting in Washington, DC, in March 2016.
relevant conferences (IPCC, WCD, and AAD) gave a wide
After each meeting, detailed minutes were circulated to all
exposure to the consensus effort. Experts from many countries
participants.
across the world participated in the deliberations and the e-mail
Both the conveners were involved in another panel discus-
discussions. Interesting and fruitful discussions ensued at the
sion on the topic at the Indian National Dermatology Congress
meetings. Experts from all over the world agreed on several
(Dermacon) in Kolkata in January 2017. The Final Consensus
important aspects of the vexing problems of acquired MPUE.
Statement was presented by one of the conveners (SPK) at the
This forum has also led to planning of a global prospective mul-
23rd IPCC held in Denver Colorado, USA in August 2017.
ticenter study on these conditions and is spearheaded by Prof
The methods used in arriving at the consensus are summa-
Amit Pandya.
rized in Table 1. Thirty-nine experts on pigmentary disorders
representing 18 countries took part in the meetings and/or e-
mail discussions (Table 2). Conclusions

Ashy dermatosis/EDP/LPP Global Consensus Forum reached


a consensus on the following:
Table 1 Methods used in arriving at a consensus
1 Morphologies typical of AD, EDP, and LPP are in the spec-
Analysis of responses to two rounds of questionnaires sent by e-mail trum of acquired MPUE.
Panel discussions on the subject with audience participation at
2 Many etiologies can cause acquired macular hyperpigmen-
international dermatology meetings/pigment cell research meetings in
Singapore, Canada, USA
tation as a sequel. Examples of these include, but are not
Face-to-face Discussions of the core group of experts at international limited to, lichen planus and drug eruptions.
meetings in Singapore, Canada, USA 3 Only a minority of cases of LPP have past or current evi-
Small group meetings, telephone discussions by the conveners dence of typical lichen planus.
Broad agreement of the final consensus report circulated by e-mail to
4 There should be an erythematous border, past or current; to
the full group
definitively label acquired MPUE, as EDP.
a
The Final Consensus Statement was presented by one of the con- 5 If there is a history of pruritus, and clinical features of
veners (SPK) at the International Pigment Cell Conference held in papules and plaques associated with the pigmented lesions,
Denver, Colorado, USA in August 2017. the condition is unlikely to be AD.

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Kumarasinghe et al. Global consensus statement Review 5

6 Due to usage of previous publications by numerous authors, 19 Considering that “ashy dermatosis” has negative social con-
acquired large (e.g., >5 cm) and small (e.g., 0.5–5 cm) mac- notations among the dark-skinned ethnic groups in the North
ules of pigmentation of uncertain etiology involving predomi- American Continent, the term EDP was favored over AD.
nantly the head and neck region, with or without present or 20 Melanophages in the dermis cause the ashy pigmentation in
past evidence of lichen planus elsewhere, predominantly AD, LPP, and EDP. It is not clear why the melanophages in
occurring in the people of South Asian extraction (e.g., India, LPP/AD/EDP do not clear as rapidly as in cases of simple
Pakistan, Sri Lanka) and the African Continent, may be postinflammatory hyperpigmentation.
termed LPP. However, the working group understands that 21 Interface dermatitis is not necessarily a feature required to
most such cases do not ever develop typical lichen planus diagnose LPP/AD/EDP/RM or IEMP.
and that LPP may not be etiopathologically related to lichen 22 Adding new, contradictory subgroups to describe entities such
planus. If the hyperpigmentation is limited to known areas of as “AD with plaque lesions,” “EDP with hyper and hypopig-
previous lichen planus lesions, or current visible and palpa- mented lesions,” “EDP with pruritic and scaly lesions,” “LPP
ble lesions of lichen planus, it is best to avoid labelling such with a Zosteriform pattern” etc., should be discouraged as pig-
cases as LPP. It is preferable to categorize such cases as mentation can be due to varied causes in these cases.
simply lichen planus with postinflammatory hyperpigmenta- 23 Small macules (0.5–2 cm) of acquired pigmentation, occur-
tion (the analogy for this is when a patient’s pigmented ring predominantly in adolescents and young adults with or
macules are secondary to a known fixed drug eruption or without raised velvety pigmented lesions, which resolve
eczema, we label the condition by the original diagnosis). within a few months or a few years could be termed IEMP,
7 LPP involves the head and neck region in most cases. provided that the pigmented macules did not appear follow-
8 In LPP, temple, forehead, preauricular region, ears, mental ing a known disease episode such as a viral exanthem, a
and submental region, and neck are the more common lichenoid drug eruption, or pityriasis rosea, etc.
areas of involvement in the head and neck region. 24 The condition described as IEMP with papillomatosis
9 In LPP, after the head and neck region, the next most com- appears to be a different entity to typical IEMP as the mor-
mon area of involvement is the flexures, particularly axillae. phology involves patchy, velvety thickening of skin as
10 LPP lesions are found on sun-exposed areas as well as opposed to macular lesions, with the histopathological char-
non-sun-exposed areas. acteristic of papillomatosis.
11 LPP-like pigmentation has been associated with lichen 25 The term Riehl’s Melanosis should only be used to describe
planopilaris of the scalp in some cases. numerous fine (i.e., few millimeters in size) or reticulate,
12 Diffuse (not patchy or discrete macules) acquired hyperpig- acquired macules of pigmentation of uncertain etiology
mentation as in Addison’s disease should not be labeled as occurring on the face, neck, and upper chest.
LPP, EDP, or AD. 26 If a diagnosis of a relevant contact dermatitis can be estab-
13 Histopathology of LPP/AD/EDP can appear similar, at least lished; numerous fine (e.g., few millimeters in size) acquired
during some periods of the disease. macules of pigmentation of uncertain etiology occurring on
14 Acquired large (>5 cm) hyperpigmented macules are char- the face, neck, and upper chest, should be termed pig-
acteristic in AD and EDP. In typical cases of AD and EDP, mented contact dermatitis
the trunk is a common area of involvement. 27 If a definite etiology (e.g., drug eruption, postinflammatory)
15 Cases described as EDP without a history or current evi- or a definite morphological pattern (LPP, EDP/AD, IEMP,
dence of erythematous border can be considered synony- Riehl’s melanosis, etc.) cannot be established at the time of
mous with AD. presentation, any other form of acquired macular hyperpig-
16 Other causes of pigmentation such as medicinal drugs, food mentation may be termed as “MPUE” or “dyschromia of
additives, and food coloring should be carefully excluded as uncertain etiology.” The clinician should then try to work out
the pigmentation can be insidious. Pigmentation identical to the diagnosis by carefully monitoring the evolution of the dis-
the morphology of AD/EDP has been reported due to some ease while carefully analyzing contributory factors in a given
drugs. patient. Of the two terms, “MPUE” implies hyperpigmenta-
17 LPP/EDP/AD morphological patterns occur in different tion (as in the case of IEMP), whereas “dyschromia” may
regions in communities with different eating habits and differ- imply hypo- or hyperpigmentation.
ent sociocultural practices. These conditions are unlikely to 28 Simple postinflammatory hyperpigmentation due to known
be due to a particular oil applied on the skin or a particular conditions (e.g., dermatitis, graft vs. host disease, lichenoid
dietary ingredient (i.e., cultural practices and dietary habits drug eruption) should not be labeled as LPP, EDP, or AD.
are diverse in countries such as Brazil, Mexico, India, Saudi 29 More research should be conducted on these conditions to
Arabia, USA, South Africa, and the Philippines). characterize the conditions, identify etiology, discover effec-
18 There is no uniformly effective therapy for LPP/AD/EDP. tive treatments, and prevent progression.

ª 2018 The International Society of Dermatology International Journal of Dermatology 2018


6 Review Global consensus statement Kumarasinghe et al.

It is hoped that more emphasis will be now be given for Some examples of the above conditions are depicted in
identifying various causes of acquired macular pigmentation Figs. 1–7.
including AD, EDP, and LP and researching into the ways and Tables 3–6 summarize the salient features of the above con-
means of expediting elimination of dermal pigment in these ditions.
conditions.

(a) (b)

Figure 1 (a) Forty-year-old man of Indian descent with hyperpigmented macules with erythematous borders on the abdomen. Inset:
Histopathology of a biopsy from an erythematous area depicting a florid lichenoid reaction pattern. (Hematoxylin and eosin, 91,000).
(b) Same patient 12 months later without any visible erythema but with only hyperpigmented macules. Inset: Histopathology of an older
hyperpigmented lesion without any erythema showing dermal melanophages and some dermal cellular infiltrate. (Hematoxylin and eosin,
91,000)

Figure 2 A patient with lesions of ashy dermatosis on the


abdomen (Photo courtesy: Dr. Jose  Eleuterio Gonzalez,
Dermatology Service, Hospital Universitario, Monterrey, Mexico.).
After the erythema disappears, lesions of erythema dyschromicum
perstans can also look identical to this appearance

Figure 3 Hyperpigmented, ill-defined blotchy, pigmentation on the


neck and upper back consistent with lichen planus pigmentosus

International Journal of Dermatology 2018 ª 2018 The International Society of Dermatology


Kumarasinghe et al. Global consensus statement Review 7

Figure 4 Macular pigmentation on the


forehead in a 30-year-old Indian man
consistent with lichen planus pigmentosus.
Inset: Skin biopsy showing prominent
dermal melanophages (Hematoxylin and
eosin, 91,000)

Figure 5 Riehl’s melanosis. An 83-year-old


Eurasian woman with reticulate pigmentation
of the neck and the face

Figure 6 Idiopathic eruptive macular


pigmentation in a 7-year-old Filipino boy.
Inset: Biopsy shows hyperpigmentation of
the basal cell layer and few pigment-laden
macrophages (Hematoxylin and eosin,
91,000) (Photograph courtesy of Dr
Johannes F. Dayrit of De La Salle University
Health Sciences Institute, Philippines)

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8 Review Global consensus statement Kumarasinghe et al.

Table 5 Idiopathic eruptive macular pigmentation

Characterized by asymptomatic, nonconfluent small brown macules


(0.5–2 cm) involving the neck, proximal extremities, and trunk
No preceding inflammation or history of drug exposure
Histology is characterized by hyperpigmentation of the basal layer of
the epidermis and prominent dermal melanophages without visible
basal layer damage or lichenoid inflammatory infiltrate
Resolves in few months to years
Predominantly involves adolescents and young adults

Table 6 Reihl’s melanosis and pigmented contact dermatitis

Characterized by numerous fine (e.g., few millimeters in size) or


reticulate, acquired macules of pigmentation of uncertain etiology
Occurs on the face, neck, and upper chest
If a definite relevant contact allergy is demonstrated in patients with
finely reticulated pigmented lesions on the face and neck region, the
disorder may be labeled as pigmented contact dermatitis

Questions (answers provided after


Figure 7 An 11-year-old Indian patient with idiopathic eruptive references)
macular pigmentation (Photograph courtesy of Dr Vinay
Keshavmurthy of Post Graduate Institute of Medical Education and For questions 1–4, choose the correct response; there may be
Research, Chandigarh, India). Inset: Closer view of periumbilical more than one correct response
lesions showing velvety thickening of the pigmented lesions 1. The major difference/s between EDP and AD is/are:
(a) The presence of an erythematous border in EDP
(b) The presence of a surrounding area of depigmentation
on AD
Table 3 Ashy dermatosis (AD)/erythema dyschromicum
(c) Presence of elevated plaque lesions in AD
perstans (EDP)
(d) None of the above
Characterized by acquired large hyperpigmented macules > 5 cm
Etiology unknown 2. The characteristic/s of idiopathic eruptive macular pigmen-
Commonly involves trunk tation is/are:
AD and EDP can be considered to be synonymous except for the
(a) Asymptomatic lesions
presence of erythematous border in the active stage of EDP
(b) Typically regresses over several months or a few years
Melanophages in dermis cause the ashy gray pigmentation
Interface dermatitis may be present but is not a feature necessary to (c) The lesions are mostly large macules >5 cm in size
diagnose these conditions (d) Most cases have an associated history of drug exposure

3. Which of the following is/are false about LPP:


Table 4 Lichen planus pigmentosus (LPP) (a) Trunk is the commonest site of involvement
(b) Appearance of lesions is not related to sun exposure
Acquired large (e.g., >5 cm) and small macules (e.g., 0.5–5 cm) of (c) Histopathology LPP can be similar to AD
pigmentation of uncertain etiology, with or without present or past
evidence of lichen planus elsewhere
4. Which of the following statement/s is/are false:
Most such cases do not ever develop typical lichen planus and that
LPP may not be etiopathologically related to lichen planus (a) Lesions of Reihl’s melanosis are very fine macules,
The commonest area of involvement is head and neck region followed usually 1–2 mm in size, predominantly involving the
by flexures head and neck region
If the hyperpigmentation is limited to known areas of previous lichen
(b) If a diagnosis of relevant contact dermatitis can be
planus lesions, or current visible and palpable lesions of lichen
established, numerous fine macular pigmented lesions
planus, such cases should be labeled as lichen planus with
postinflammatory hyperpigmentation and not LPP of uncertain etiology occurring on the face, neck, and
Histology may be similar to EDP/AD with presence of dermal upper chest should be termed pigmented cosmetic
melanophages with or without interface dermatitis at some stage dermatitis

International Journal of Dermatology 2018 ª 2018 The International Society of Dermatology


Kumarasinghe et al. Global consensus statement Review 9

(c) Melanophages in the dermis in patients with AD and 6 Bhutani LK. Ashy dermatosis or lichen planus pigmentosus:
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uncertain aetiology revisited: two case reports and a proposed
5. MPUE includes postinflammatory hyperpigmentation fol- algorithm for clinical classification. Australas J Dermatol 2017;
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lowing lichen planus.
9 Anderson A, Wood B, Kumarasinghe SP. Defining erythema
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Idiopathic eruptive macular pigmentation: report of 5 patients.
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14 Stevenson JR, Miura M. Erythema dyschromicum perstans
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ª 2018 The International Society of Dermatology International Journal of Dermatology 2018


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Answers to Questions
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