Neonatal

Dermatology
Marely Santiago Vázquez, MD (PGY3)
Department of Dermatology
University of Puerto Rico
Outline
• Neonatal Rashes • Infantile Hemangiomas and
• Neonatal Cephalic Pustulosis Vascular Malformations
• Erythema toxicum neonatorum • Infantile Hemangioma
• Transcient Neonatal Pustular • Port Wine Stains
Melanosis
• Seborrheic Dermatitis
• Neonatal Birthmarks • Others
• Dermal Melanosis • Aplasia Cutis Congenita
• Congenital Melanocytic Nevus • Accessory Tragus
• Neurocutaneous Melanosis • Focal Dermal Dysplasias
• Nevus Sebaceous
2 wk old healthy bg presents for evaluation of a 5 day
history of facial red bumps. Pmhx: full term vaginal birth
with no complications. Which of the following best
describes the cause of her rash?
A. Congenital viral infection transmitted from her
mother during delivery

B. Inflammatory reaction to Pityrosporum(Malassezia)

species

C. Allergic reaction to soap

D. Immaturity of adnexal structures

2 wk old healthy bg presents for evaluation of a 5 day
history of facial red bumps. Pmhx: full term vaginal birth
with no complications. Which of the following best
describes the cause of her rash?
A. Congenital viral infection transmitted from her
mother during delivery

B. Inflammatory reaction to Pityrosporum(Malassezia)

species

C. Allergic reaction to soap

D. Immaturity of adnexal structures

Neonatal Cephalic
Pustulosis
Neonatal Cephalic Pustulosis
• ~20% of newborns (ages 0-6 wks)

• Onset: First 2-3 weeks of life

• Inflammatory non comedonal pustular

eruption

• Pathogenesis: Inflammatory response to

Malassezia (Pityrosporum) species

• Prognosis: Resolves spontaneously by 3-6

months of age
Neonatal Cephalic Pustulosis
• Clinical Characteristics:
• Inflammatory papules and pustules
(papulopustules) over background
erythema

• No true comedomes (blackheads or

whiteheads)

• Most commonly seen on the face

(cheeks[most common]>forehead, chin,
eyelids) > >neck, chin, upper chest, and
scalp
Neonatal Cephalic Pustulosis
• DDXà Infanile Acne
• Lack of comedomes and absence of prolongued course (typically
characteristic of infantile acne)
• Treatment:
• Reassurance. Self limited.
• Not required. Topical ketoconazole cream
3 day old healthy male presents with 1 day history of
white/yellow papules and pustules surrounded by blotchy
erythema in face,trunk and extremities. Swab stains from
one of the pustules reveals eosinophils.
Which of the following is the most
likely diagnosis?
A. Acrodermatitis enteropathica
B. Transient neonatal pustular
melanosis
C. Erythema toxicum neonatorum
D. Seborrheic dermatitis
E. Miliaria crystallina
3 day old healthy male presents with 1 day history of
white/yellow papules and pustules surrounded by blotchy
erythema in face,trunk and extremities. Swab stains from
one of the pustules reveals eosinophils.
Which of the following is the most
likely diagnosis?
A. Acrodermatitis enteropathica
B. Transient neonatal pustular
melanosis
C. Erythema toxicum neonatorum
D. Seborrheic dermatitis
E. Miliaria crystallina
Erythema Toxicum
Neonatorum
 Erythema Toxicum Neonatorum
• Common, benign vesiculopustular eruption

• Affects ~ 20-70% of full-term babies

• Onset: 24-48 hours after delivery.

• Prognosis: Resolves spontaneously ~5-7 days.

No treatment is needed. Recurrence rarely
seen.

• Wright stain of pustulesà accumulation of

eosinophils
 Erythema Toxicum Neonatorum
• Clinical characteristics:
• Large erythematous macules and papulesàrapidly
progress to flaccid pustules w/ surrounding blotchy
erythema.“flea bitten appearance”
• Occurs in any part of the body except palms and
soles
• Onset within 24-48 hours. Resolves within 5-7 days
• After 2 weeks of age, all lesions should resolve
without hyperpigmentation

• Treatment: Self limiting. Not required

1 day old healthy bg presents with widespread eruption.
Lesions consist of pustules and hyperpigmented
macules, some with discrete scaling. No erythema
visible around lesions.
Which of the following is the most appropriate step
in the continued treatment of this girl?

A. No additional evaluation of the lesions is required

B. Obtain RPR on both mother and child
C. Obtain ANA and dsDNA
D.Bacterial culture of vesicular fluid
E. Tzanck smear
1 day old healthy bg presents with widespread eruption.
Lesions consist of pustules and hyperpigmented
macules, some with discrete scaling. No erythema
visible around lesions.
Which of the following is the most appropriate step
in the continued treatment of this girl?

A. No additional evaluation of the lesions is required

B. Obtain RPR on both mother and child
C. Obtain ANA and dsDNA
D.Bacterial culture of vesicular fluid
E. Tzanck smear
Transient Neonatal Pustular Melanosis
Transient Neonatal Pustular Melanosis
• ~5% of newborns with darkly pigmented skin.
May also be seen in Caucasian infants

• Onset: Present at birth

• Pathogenesis: Idiopathic

• Prognosis: Benign, asymptomatic, non-scarring,

self-limiting disease.

• Clinical Diagnosis. Wright stain of pustuleà

accumulation of neutrophils with no organisms
Transient Neonatal Pustular Melanosis
• Clinical characteristics:
• 3 different morphologies (can co-exist)
• Small pustules on a non erythematous base
(vs ETN)
• Erythematous macules with surrouding fine
colarette of scales (2ry to rupture of
previous pustules)
• Hyperpigmented macules
• Chin, forehead, nape of the neck, lower
back, buttocks and shins>> remainder of
the face, trunk, palms, and soles.
• Treatment: Self limiting. Not required
4 wk old male infant that comes for evaluation of skin
rash present on scalp. Pmhx remarkable for full term,
vaginal birth with no complications.
Name the diagnosis.
Seborrheic
Dermatitis
Seborrheic Dermatitis
• Common, benign eruption. Affects ~ 10%
newborns

• Pathogenesis: Exact etiology unknown but can be

due to hormones, proliferation of Malasezzia
species

• Onset: 3 weeks-12 months

• Prognosis: Benign, usually self resolves. 8% may

have persistent SD

• Generalized SD is uncommon in healthy children.

Usually associated with immunodeficiencies
Seborrheic Dermatitis
• Clinical Characteristics:
• Erythema, greasy scales + salmon colored oval scaly
patches

• Scalp (“craddle cap”), face (Forehead, eyebrows,

nasolabial folds, postauricular folds), flexural areas
including diaper area

• areas of ↑ sebaceous gland activity (auricles, beard

area, eyebrows, trunk & inframammary areas

• Treatment:
• Low potency steroids + topical antifungals
Dermal
Melanosis
Aka Mongolian Spots
Dermal Melanosis
Aka Mongolian Spots
• More common in Black, Native Americans and Hispanic
populations

• Onset: Usually present at birth or within first weeks of life.

• Clinical characteristics: flat bluish-gray/ brown macules/patches on the back or buttocks

and shoulders. Rarely occurs in face, head or flexural regions

• Pathogenesis: Caused by melanocytes trapped in the dermis due to arrest in migration from
the neural crest into the epidermis

• Prognosis: Benign. Most lesions fade by age 2. No treatment is needed.

• The most common lysosomal storage disease associated with generalized MS is Hurler
syndrome followed by gangliosiodosis type 1
Dermal Melanocytosis
Patient’s mother wants know what to expect and if
any treatment is needed. Which of the following
should you tell her?
A. The patient needs to be referred to plastics for
removal of lesions
B. The patient should be monitored as it confers
higher risk of developing melanoma
C. Lesions usually fade during adulthood and no
treatment is required.
D. Recommend to begin treatment with
hydroquinone cream
E. None of the above
Dermal Melanocytosis
Patient’s mother wants know what to expect and if
any treatment is needed. Which of the following
should you tell her?
A. The patient needs to be referred to plastics for
removal of lesions
B. The patient should be monitored as it confers
higher risk of developing melanoma
C. Lesions usually fade during adulthood and no
treatment is required.
D. Recommend to begin treatment with
hydroquinone cream
E. None of the above
A 3-month-old infant with a GCMN involving the back
with at least 30 satellite nevi presents for initial
evaluation.
Which of the following should be recommended as the next
step in management?
A. Skin biopsy
B. MRI of brain and spine
C. Surgical excision of the nevus
D. Clinical monitoring
E. Genetic testing of child and parents
A 3-month-old infant with a GCMN involving the back
with at least 30 satellite nevi presents for initial
evaluation.
Which of the following should be recommended as the next
step in management?
A. Skin biopsy
B. MRI of brain and spine
C. Surgical excision of the nevus
D. Clinical monitoring
E. Genetic testing of child and parents
Congenital
Melanocytic
Nevi
Congenital Melanocytic Nevus
• 1-3% of newborn infants affected regress
• Pathogenesis: Melanocytic nevi 2ry • They are classified* as:
to proliferation of benign • Small (most common)à <1.5cm
melanocytes • Medium à >1.5-20cm
• Onset: Present at birth or within • Large à >20-40cm
the first few months • Giant à > 40cm
• * based on projected adult size of the CMN (calculated)

• Characterized by round symmetric

lesions associated with
hypertrichosis
• Rugose or pebbly surface and with
age can become elevated, darken or
have a verrucous appearance or
Neurocutaneous Melanosis
• Proliferation of melanocytes in the • Asymptomatic
skin and CNS (leptomeninges or • Risk factors for NCM
brain parenchyma) • Multiple satellite lesions (>20)
• Congenital syndrome characterized • Large/giant CMN
by the presence of either a • Posterior axial location (neck, back,
large/giant (>20cm) CMN or head, buttocks)
multiple smaller CMN a/w • MRI should be considered in any
meningeal melanosis or melanoma pt with a large/giant CMN with
• Classified as: multiple satellite lesions or
• Symptomatic – presents with signs multiple medium-size CMN in
of ICP due to hydrocephalus or mass order to screen for NCM
effect
Congenital Melanocytic Nevi
• Classification correlates with malignancy potential or risk of
melanoma development (usually before 5 yrs of age)
• Small and medium sized CMN:
• <1% lifetime risk.
• Monitor regularly for changes in size, color, texture or shape.
• Large, giant or multiple CMN:
• ~3-10% of higher lifetime risk
• Regular skin exam to r/o melanoma.
• Screen pts at risk for Neurocutaneous melanosis with MRI of brain and spine during
first 6 months of life
1 y/o Bb comes to your clinic for evaluation of the
following lesion. Mother refers lesion has been present
since birth and has remained stable in growth.
Name the diagnosis.
Nevus Sebaceus
Nevus Sebaceus
• ~ 0.3% of newborns
• Pathogenesis: Hamartoma involving the epidermis, hair follicles and
sebaceous and apocrine glands
• Onset: Birth
• Clinical characteristics: solitary, smooth, yellow-orange, hairless raised
patch/plaque +/-fine papules on the surface
• Often oval or linear in shape; Scalp and face
• Prognosis. Remains constant through childhood
• androgen stimulation during puberty can cause the lesion to become larger, more
pronounced (bumpy, warty or scaly).
• They do not regress with age
Nevus Sebaceus
• Clinical diagnosis
• Can be an isolated finding but can be associated with neurocutaneous
disorders
• Linear nevus sebaceus syndrome – characterized by the triad
• Midline nevus sebaceous
• Seizures
• Intellectual disability
• Recent studies indicate a small percentage of lesions turning into
tumor
• BCC is the most common malignancy associated with NS
• TX à surgical excision
Port-Wine Stain
Aka Nevus Flammeus
Port-Wine Stains
Aka Nevus Flammeus
• Pathogenesis: vascular birthmark. Vascular malformation of
dermal capillaries
• Onset: Present at birthà persist into adulthood
• Characterized by a blanchable pale pink/red macules/patches
which with time progress to become dark red-to-purple
• Usually follows the distribution of the trigeminal nerve branches (V1,
V2, V3); often in unilateral/segmental distribution
• Prognosis:
• Do not regress over time. Grow proportionally with the child’s
growth: darker, thicker, more popular
• Isolated or a/w syndrome
Port Wine Stain Distribution
• Can be found anywhere
• Certain distributions are associated with complications:
• Trigeminal Branch V1(or upper/lower eyelid)--> Sturge Weber
Syndrome
• Eyelid: congenital glaucoma. Urgent opthalmologic evaluation
• Large/widespread: Overgrowth syndromes
Which of the following is the most important next step
in the evaluation of this patient?

A. CMP
B. Renal ultrasound
C. CBC
D. Opthalmologic examination
E. None of the above
Which of the following is the most important next step
in the evaluation of this patient?

A. CMP
B. Renal ultrasound
C. CBC
D. Opthalmologic examination
E. None of the above
Sturge Weber Syndrome
• Characterized by the triad of vascular
malformations :
• Cutaneous: Facial PWS( V1)
• CNS: ipsilateral leptomeningeal
angiomatosisà ↑ risk of seizures,
neurologic delay, hemiplegia
• Ocular: Ipsilateral choroidal vascular
malformationà glaucoma
• Seizures + PWS = Sturge Weber
syndrome
Port-Wine Stains
Aka Nevus Flammeus
• Work up:
• MRI to exclude SWS with facial PWS involving more than one
trigeminal nerve branch or with neurologic symptoms
• MRI of the spine to r/o spinal dysraphism in patients with midline
lumbosacral capillary malformation
• Treatment
• Conservative- No treatment.
• Pulsed Dye Laser – irreversibly damages the capillary vessels
Cutis Marmorata
Cutis Marmorata
Cutis Marmorata
• Physiologic. More common in premature infants
• Pathogenesis: The ability of neonate to adjust to extrauterine
surroundings is immature, resulting in cutaneous blood flow
abnormalities
• When neonates are cold the constricted capillaries and venules
produce a reticulated, blanchable, violaceous pattern
• Prognosis: Transient condition rapidly improving with rewarming
and tends to diminish with age
• Should not be confused with cutis marmorata telangiectatica
congenita, which is a vascular malformation that persists for
several years.
Infantile
Hemangiomas
Infantile Hemangioma
• 1.2-5% present aMost common benign vascular
tumor of infancy
• t birth; 10% infancy proliferation
• Risk factors: low birth weight, female gender,
multiple gestation, prematurity
• Prognosis:
• Proliferation during first 2-3 months (rapid
proliferation b/e 5-7 wks)
• Growth stablilizes ~4-6 months à involutes over years
• Cutaneous (head and neck>ext), mucosal or
visceral involvement
Infantile Hemangioma
• Clinical characteristics:
• Superficial: Upper dermis. Bright red papule, nodule, plaque
• Deep: Extends to adipose tissue. Flesh colored nodule w/ blue hue
• Combined: Mix of the above
Infantile Hemangioma

• Complications:
• Ulceration (MC)
• Visual impairment
• Airway imparement
• High output cardiac failure
• Aesthethic changes
Infantile Hemangioma
• The patient in the accompanying
photograph presents with his parents.
They say that this “birthmark” was not
present at birth and that it started
growing rapidly when the patient was 3-
4 weeks old. How many hemangiomas
on exam would prompt you to look for
internal involvement?
• 1. two or more
• 2. Three or more
• 3. Four or more
• 4. Five or more
• 5. Six or more
Infantile Hemangioma
The patient in the accompanying
photograph presents with his parents.
They say that these “birthmarks” were not
present at birth and started growing
rapidly when the patient was 3-4 weeks
old. What is your next step in
management?
A. Start the patient on timolol
B. Start the patient on propranolol
C. Order a liver US
D. Order a head and chest MRI
E. Reassure the parents that these are
benign and will go away with time
Infantile Hemangioma
• Number, conformation and distribution can vary.
• 3 types: focal, segmental and inderterminate/diffuse
• Focal/Localized:
• Associated with extracutaneous complications(MC: Liver) when they are multifocal (≥5)
• Hepatic ultrasound should be performed in patients with >5 cutaneous IH
• Periorbital: Periorbital hermangiomas have ~80% risk of ocular complications
including astigmatism, amblyopia, refractive errors and occasionally blindness (visual
axis obstruction)
• Segmental: greater risk of extracutaneous complications
• Head/neck à PHACE syndrome (30% risk).
• Lumbosacral and perineal lesions should raise suspicion for associated spinal cord, renal
and genital anomalies.
• Mandibular/beard distributionà airway involvement
Infantile Hemangioma

• Which of the following additional studies

do you perform in this patient?
A. Air-contrast barium enema
B. Direct laryngoscopy
C. Echocardiogram
D. Brainstem auditory evoked response
test
E. Renal US
PHACES syndrome
• You are seeing an infant in your office for her 2-month
health supervision visit. The parents are concerned
about a red bump that first appeared on her face at 2
weeks of age. The lesion has since grown to cover a
large portion of her cheek, chin, and infraorbital
area. The infant is growing and developing normally,
and her physical examination findings are otherwise
unremarkable. Of the following, the BEST treatment
option for this lesion is
A. Intralesional corticosteroid injection
B. Observation
C. Oral propranolol
D. Parenteral α-interferon
E. Surgical excision
Infantile Hemangiomas
• Treatment:
• Majority proliferate and involute w/o functional impairment
• Beta-blockers (propranolol): systemic and topical (Timolol)
• Steroids (clobetasol)
• Pediatric Laser therapy: Pulse Dye Laser
Infantile Hemangiomas
Aplasia Cutis
Congenita
Aplasia Cutis Congenita
• Condition in which localized or widespread areas of skin are absent
or scarred at birth
• May be an isolated defect or can occur in association with other
developmental anomalies or a feature of several syndromes
• Possible etiologies:
• Genetic factors
• Vascular compromise
• Trauma
• Teratogens
• Intrauterine infection
Aplasia Cutis Congenita
• Can present as an erosion or deep ulceration to
a scar due to healing in utero, or an ovoid defect
covered by a membrane
• Membranous aplasia cutis – most common
form, occurring primarily in the scalp
surrounded by a “hair collar”
• Characterized by a punched-out, oval/round defect
covered by a thin, translucent, glisetining epithelial
membrane
• Sometimes can contain serous fluid resulting in a
bullous appearance
• They tend to flatten and transform into an atrophic
scar
Aplasia Cutis Congenita
• In patients with large, deep, irregular, or membranous lesions of the
scalp(hair collar sign), imaging studies (MRI) is indicated to assess for
underlying bone defects, vascular anomalies, or brain malformations
• Most lesions heal within the first few months leaving an atrophic scar
• Treatment
• Gentle cleansing
• Topical antibiotic ointment
• Petrolatum ointment
Accessory
Tragus
Accessory Tragus
• Common congenital anomaly of the 1st brachial arch
found in ~5/1000 newborns
• Soft or firm (due to a cartilaginous core) skin-colored
papules or nodules covered by vellus hairs
• Located in preauricular area > mandibular cheek or
anterolateral neck (congenital cartilaginous rests of
the neck or ‘wattles’).
• Usually an isolated finding; occasionally associated
with hearing impairment or malformation syndromes
(e.g. hemifacial microsomia).
• Rx: assessment of hearing; excision, with care to
remove any cartilaginous component.
 Focal Facial Dermal
Dysplasia
• Rare genetically heterogenous group of disorders
• 4 subtypes:
• Type 1: Brauer Type 2: Brauer-Setleis
• Type 3: Setleis Type 4: Preauricular
• Clinical characteristics: congenital bilateral (bitemporal)
cutis aplasia +/- facial anomalies
• Round/oval atrophic hypopigmented
macules/patches +/- hair collar

• Bilateral temples from lateral eyebrow to anterior

hairline (Types 1-3)/preauricular cheek to angle of
mouth (Type 4)

• +/- absent eyelashes on both lids or multiple rows on

the upper lids, absent Meibomian glands, slanted
eyebrows, chin clefting, and others
Setleis syndrome
• Mutation in TWIST2

• First described in patients from

consanguineous Puerto Rican (PR) families

• Characterized by:
• Bilateral temporal marks, an aged-
leonine appearance, absent eyelashes
on both lids or multiple rows on the
upper lids, absent Meibomian glands,
slanted eyebrows, and chin clefting.
 Thank you !
Questions?
marely.santiago@upr.edu