Skin lesion in neonate

Pediatr Clin N Am 51 (2004) 863 – 888

Skin lesions in the neonate
Joseph D. Conlon, MD, Beth A. Drolet, MD*
Departments of Dermatology and Pediatrics, Medical College of Wisconsin,
Children’s Hospital of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, USA

Neonatal skin plays a pivotal role in the transition from an aqueous to an air-
dominant environment, because it provides mechanical protection and assists in
thermoregulation, immunosurveillance, and fluid balance. Worried parents often
seek medical attention from their child’s physician regarding skin lesions. Thus, a
working knowledge of both normal and abnormal cutaneous lesions of the
neonate is required to properly address these issues. In this article, the authors
briefly discuss transient benign lesions, pustular and vesicular infections, ‘‘birth-
marks’’ (vascular and pigmentary lesions), common congenital abnormalities,
select blistering disorders, and various other skin conditions.

Transient benign cutaneous lesions
At birth, infants near complete gestational maturity have varying degrees of
vernix caseosa. This moist, greasy, gray-white coating composed of sebum,
keratin, and hair becomes thicker with advancing gestational age and provides
protection in utero and perinatally. It may be stained yellow with meconium
signaling possible fetal distress [1].
Fine desquamation occurs in most infants. Timing is dependent on gestational
age. Preterm and full-term infants undergo this process at 2 to 3 weeks and 1 to
2 day of age, respectively. Post-term infants often experience thicker peeling
and cracking.
Additional benign skin lesions are often discovered during the infant’s first
few weeks of life. Milia are 1- to 2-mm, white, smooth papules most often
located on the forehead, cheeks, and nose (Fig. 1). They are the result of tiny
inclusion cysts in the epidermis. These lesions are discrete and resolve sponta-
neously as they naturally express their keratin contents. When located within the
oral mucosa, they are called Epstein pearls. Milia may be easily confused with

* Corresponding author.
E-mail address: drolet@mew.edu (B.A. Drolet).

0031-3955/04/$ – see front matter D 2004 Elsevier Inc. All rights reserved.
doi:10.1016/j.pcl.2004.03.015

864 J.D. Conlon, B.A. Drolet / Pediatr Clin N Am 51 (2004) 863–888

Fig. 1. Milia on the forehead.

sebaceous hyperplasia, which appears as smooth, yellow, follicular-based papules
on or around the nose. These are found in about one-half of term infants, rarely in
preterm infants, and also resolve fairly rapidly [2].
Milaria is a general term used to describe occlusion of the eccrine duct [3].
This occurs more frequently with infants who are in warm environments,
excessively bundled, or with fevers. The cutaneous findings associated with
these phenomena vary depending on the level of obstruction. Obstruction at
higher levels in the epidermis results in superficial trapping of sweat under the
stratum corneum, producing the small clear vesicles typical of miliaria crystallina.
The vesicles, which resemble water droplets on the skin, appear within the first
few days of life, are extremely fragile, and wipe away easily [1]. Miliaria rubra or
‘‘prickly heat,’’ presents as 1- to 3-mm, erythematous, nonfollicular-based
papules and papulopustules, usually on the forehead, upper trunk, and flexural
or occluded areas. Deeper intraepidermal occlusion of the eccrine duct causes
mild inflammation, producing the erythema associated with miliaria rubra. It is
also generally found in overheated and febrile infants. Clinical diagnosis is
sufficient, but if confusion with neonatal infection arises secondary to its
inflammatory characteristics, a smear of the vesicle contents reveals an absence
of cellular contents. Relief and avoidance of overheating can hasten resolution of
both types of miliaria.
Erythema toxicum is the most well recognized, frequently occurring, benign
eruption of the newborn period, occurring in approximately half of all term
infants [4]. Its presences correlate with gestational age and birth weight, because
it is rarely seen in very premature infants [5]. Presenting as blotchy, 1- to 3-cm,
erythematous macules, sometimes with a central 2- to 4-mm vesicle or pustule,
the lesions of erythema toxicum wax and wane, lasting about 1 week (Fig. 2).
They typically occur within the first 1 to 2 days of life on the face, with
subsequent spread to the trunk and extremities. Rarely, lesions may present at
birth or after 1 to 2 weeks. The diagnosis is again clinical; however, a Wright
stain may be performed to confirm the presence of eosinophils and relative lack
of neutrophils. The etiology of this common eruption is unclear, and no treatment
is required because individual lesions clear in about a week.
Transient neonatal pustular melanosis (TNPM) is an eruption that may be
confused with erythema toxicum. In fact, some have proposed that TNPM is a
precocious form of erythema toxicum [6,7]. Most common in African Americans,

J.D. Conlon, B.A. Drolet / Pediatr Clin N Am 51 (2004) 863–888 865

Fig. 2. Individual lesion of erythema toxicum.

TNPM occurs predominantly on the forehead, back, posterior neck, and shins. It
has three distinct phases. First, superficial, 2- to 10-mm vesicopustules without
inflammation are present at or near birth. The contents are sterile, but filled
predominantly with neutrophils. These fragile lesions usually clear within the
first week, and the second phase becomes apparent as a collarette of fine scale
develops around the resolving vesiculopustules. Last, macules of brown hyper-
pigmentation develop at the sites and eventually fade over the first few weeks
to months.
Another papulopustular eruption that may occur in the newborn period is
benign cephalic pustulosis, commonly referred to as neonatal acne. It is
characterized by small, inflammatory, erythematous papules and pustules found
on the cheeks, forehead, and scalp. They may be present at birth, but more
frequently appear at 2 to 3 weeks of age and resolve spontaneously. Benign
cephalic pustulosis is differentiated from infantile acne by its lack of comedonal
lesions and earlier onset [8].
Sucking blisters, erosions, or calluses are usually solitary, tense, fluid-filled
blisters found on an extremity [9]. They are thought to be related to vigorous in
utero sucking by the fetus on the site of involvement. Erosions or calluses may
also develop, especially if the neonate continues to suck at the site.

Infectious vesiculopustular eruptions of the newborn
Familiarity with the transient benign lesions of the newborn period is
mandatory for all health care professionals taking care of neonates. Without this
knowledge, one is less able to differentiate between the aforementioned entities
and potential worrisome vesicular or pustular lesions indicative of infection. The
list of neonatal infections with cutaneous clues is extensive. The following
section provides only a limited review of more commonly encountered eruptions
associated with specific infectious etiologies: herpes simplex, group B strepto-


Box 1. Most causes of neonatal pustules or vesicles
Superficial staphylococcal pustulosis
Erythema toxicum neonatorum
Transient neonatal pustular melanosis
Miliaria (cystallina or rubra)
Benign cephalic pustulosis
Neonatal candidiasis
Congenital candidiasis
Herpes simplex infections
Scabies
Acropustulosis of infancy
Incontinentia pigmenti

Adapted from Frieden IJ, Howard R. Vesicles, pustules, bullae,
and erosions. In: Eichenfield LF, Frieden IJ, Esterly NB, editors.
Textbook of neonatal dermatology. Philadelphia: WB Saunders;
2001. p. 138; with permission.

coccal infection, staphylococcal pustulosis, and candidiasis. Box 1 lists causes of
neonatal pustules or vesicles [10].
Neonatal herpes simplex (HSV) infection remains one of the most feared
etiologies of vesicles and pustules in the newborn period, because significant
systemic involvement is possible. It may result in several different clinical
patterns: localized, disseminated (visceral involvement, usually lungs or liver),
and central nervous system disease. All three may have cutaneous findings.
Classically, lesions are grouped, 2- to 4-mm, clear vesicles on an erythematous
base on the scalp or face (Fig. 3); however, these may be located anywhere, and
can evolve onto pustules, crusts, or erosions. Although they may be the most
characteristic findings of HSV infection, they may be subtle, inconspicuous,

Fig. 3. Lesions on scalp indicative of HSV infection.


and lag behind other signs and symptoms. Lethargy or poor feeding may be the
first, albeit nonspecific, clues to disease. Seizures may indicate central nervous
system involvement.
Neonatal herpes is usually caused by infection with herpes simplex virus type
2 and is acquired as the infant passes through the vaginal canal at birth. A
maternal history of a primary herpes infection may provide important diagnostic
information; however, the majority of neonatal herpes cases occur in infants born
to women who remain asymptomatic or do not recognize symptoms. A secondary
outbreak of maternal herpes infection rarely leads to neonatal infection.
If skin lesions suspicious of HSV infection are present, early diagnostic and
therapeutic intervention is crucial. Representative lesions should be scraped for
Tzanck smear and culture. Viral cultures of the blood, cerebrospinal fluid, and
urine should be obtained. Intravenous antiviral therapy, close monitoring, and
appropriate isolation protocols should be initiated.
The most common cause of sepsis in the newborn period is group B
streptococcal infection. A maternal history of infection may heighten awareness
for signs or symptoms of disease. These infants may present with temperature
instability, lethargy, irritability, or poor feeding. They may also present with
vesicles, bullae, erosions, or honey-crusted lesions at any site. Gram stain of the
lesion demonstrates gram-positive cocci in chains. A full septic work-up is
warranted and antibiotic therapy is required because bacteremia, pneumonia, and
meningitis are potential complications.
When multiple small vesicles and pustules develop in the diaper area and
lower abdomen of neonate, a gram stain and culture should be performed, even in
a well-appearing child. The presence of gram-positive cocci in clusters indicates
the possibility of staphylococcal pustulosis (Fig. 4). These infants are usually
asymptomatic, but lesions may evolve into bullae heralding the progression to
staphylococcal scalded-skin syndrome. The causative strains of S aureus (phage
group 2) produce epidermolytic toxins that target cells in the superficial
epidermis, causing erythema and loss of cell-to-cell adhesion. Staphylococcal
scalded-skin syndrome has a low, but real associated risk of mortality in
children and the majority of these cases are neonates. Local and hematogenous

Fig. 4. Staphylococcal pustulosis on the lower abdomen.


spread may also occur, leading to cellulitis, osteomyelitis, pneumonia, or
septicemia [11].
These potential morbidities necessitate prompt diagnosis and initiation of
treatment. Culture of lesion contents confirms the diagnosis and sensitivity
profile of the particular strain of S aureus. In an infant who has localized
nonbullous disease and no signs of systemic disease, oral antibiotic therapy with
very close follow-up may be warranted. Cephalexin or dicloxacillin are appro-
priate choices. When evidence of bullous disease is present, however, admission
with appropriate isolation of the patient and use of intravenous antibiotic
therapeutics is recommended because of the potential for rapid progression to
more severe disease. The need for a full septic work-up is controversial. Parental
therapy should be continued until clearance of active lesions and systemic
symptoms. Oral antibiotics may be used to complete a full 7- to 10-day course.
The clinical presentation of cutaneous infection with Candida species is
variable, depending on the age of patient, the route of infection, and the patient’s
immune status. Candida prefers a warm, moist environment, which explains
its predilection for the mouth and diaper area of infants. With oral candidiasis,
involvement of the tongue and buccal mucosa is most common. Candidal
diaper dermatitis is frequently associated with oral infection, and presents with
bright red papules and pustules that coalesce into plaques. Satellite lesions are
apparent as red papules at the border of the large plaques and facilitate the
diagnosis. If necessary, potassium hydroxide (KOH) examination confirms the
presence of budding yeast or pseudohyphae.
Congenital candidiasis is acquired in utero as a result of an ascending infection
from a maternal candidal vulvovaginal infection. It is surprisingly uncommon,
given the high prevalence of vaginal colonization in pregnant women [12]. This
eruption is present at birth as small red papules and pustules, followed by crusting
and desquamation (Fig. 5). These can be located virtually anywhere, including
the palms and soles (Fig. 6). Rarely, there will be generalized erythema and
erosions. Ironically, the mouth and diaper area are spared. Systemic dissemina-
tion is rare in term infants. They typically respond well to topical antifungal
therapy. Candidiasis with systemic involvement is more common in premature,

Fig. 5. Erythema and desquamation characteristic of cutaneous candidiasis.


Fig. 6. Cutaneous candidiasis on the plantar surface.

low birth-weight infants. These infants and those with evidence of systemic
signs of illness may have disseminated infection, and thus require aggressive
parental therapy.

Birthmarks
‘‘Birthmark’’ is a general term used to describe congenital anomalies of the
skin. It should not be used as a diagnosis, because congenital skin lesions vary
greatly in their appearance and prognosis. Many congenital skin anomalies exist
and often present with associated signs and symptoms (Table 1).

Hemangiomas and vascular malformations
The understanding of vascular lesions has evolved significantly over the past
30 years. They can be divided into two major categories: hemangiomas and
vascular malformations. Hemangiomas are vascular tumors characterized by clear
proliferative and involutional phases. Malformations are developmental defects
derived from the capillary, venous, arterial, or lymphatic vessels. These lesions
remain relatively static, and growth is commensurate with growth of the child. It
is important to differentiate these two entities because they have very different
prognoses and clinical implications.
Hemangiomas are the most common soft-tissue tumors of infancy, occurring
in approximately 5% to 10% of 1-year-olds [13]. True hemangiomas are
characterized by a growth phase, marked by endothelial cell proliferation and
hypercellularity, and by an involution phase. Hemangiomas are clinically
heterogeneous; their appearance is dictated by the depth and location in the
skin, as well as by the stage of evolution. In the newborn, hemangiomas may
originate as a pale white macule or patch with threadlike telangiectasia. As the
tumor proliferates, it assumes its most recognizable form: a bright red, slightly
elevated, noncompressible plaque. Hemangiomas that lie deeper in the skin are
soft, warm masses with a slightly bluish discoloration. Frequently, hemangiomas
have both a superficial and deep component. They range from a few millimeters
to several centimeters in diameter and usually are solitary; however, up to 20% of
infants will have multiple lesions. A female predominance (3:1), and an increased

870
Table 1
Common birthmarks
Color/1 lesion Birthmark Location Other

J.D. Conlon, B.A. Drolet / Pediatr Clin N Am 51 (2004) 863–888
Brown/macule or patch ´
Cafe-au-lait macule Variable, trunk Associated with neurofibromatosis
Brown (<20 cm)/plaque Congenital melanocytic nevus Scalp, trunk Possible increased risk of melanoma
Brown ( > 20 cm)/plaque Giant melanocytic nevus Trunk most common 3% – 7% risk of melanoma, neurocutaneous
melanosis
Brown-flesh colored/plaque Epidermal nevus Variable: trunk/ neck
Red/patch Port-wine stain Variable, face most common
Red/papule or plaque Hemangioma Variable, head and neck most common
Red-purple/plaque Lymphatic malformation Variable, trunk, proximal leg Often have a vesicular appearance
Gray -blue/patch Mongolian spot (dermal melanosis) Buttocks, lower trunk Usually resolve spontaneously
Gray-blue/patch Nevus of Ota Forehead and eyelids Ocular pigmentation
Gray-blue/patch Nevus of Ito Posterior shoulder
Blue/nodule Dermoid cyst Scalp, face, neck May connect to CNS if midline
Blue-purple/nodule Cephalohematoma Scalp
Blue-purple/plaque Venous malformation Variable Enlarge slowly over time
Yellow-orange/plaque Nevus sebaceus Scalp, face, neck Basal cell carcinoma may arise with
in lesion
Yellow-orange/nodule Congenital juvenile xanthogranuloma Trunk
Yellow-brown/papule or nodule Mastocytoma Variable May urticate or blister
Hypertrichosis/plaque Congenital melanocytic nevus Scalp, trunk
Hypertrichosis/tumor Plexiform neurofibroma Trunk most common Associated with neurofibromatosis
Hypertrichosis/plaque Smooth muscle hamartoma Trunk
White/patch Nevus anemicus Variable
White/patch Nevus depigmentosus Variable


incidence in premature infants have been documented. Approximately 55% of
these tumors are present at birth, and the remainder develop in the first weeks of
life. Generally, superficial hemangiomas have reached their maximal size by 6 to
8 months, but deep hemangiomas may proliferate for 12 to 14 months.
Hemangiomas then undergo slow, spontaneous resolution, which generally takes
3 to 10 years. Most hemangiomas do not require medical intervention and will
involute spontaneously; however; if complications arise and treatment is war-
ranted, oral systemic corticosteroids are the mainstay of therapy.
Despite the benign nature of most cutaneous hemangiomas, a small number
will cause functional compromise or permanent disfigurement. The risk of
permanent disfigurement is much higher if the hemangioma is located on the
face. The nose, lip, and forehead are the most vulnerable regions, and heman-
gioma in these areas must be monitored closely (Fig. 7).
Ulceration, the most frequent complication, can be excruciatingly painful and
carries the risk of infection, hemorrhage, and scarring. Hemangiomas located in
the diaper region are at the greatest risk of ulceration. Rarely, hemangiomas may
present as a congenital ulceration in the diaper region, and careful examination of
the ulceration will reveal a small rim of typical superficial hemangioma.
Periorbital hemangiomas pose considerable risk to vision and should be
carefully monitored. Hemangiomas involving the ear may decrease auditory
conduction, which ultimately may cause speech delay. Multiple cutaneous
(diffuse hemangiomatosis) (Fig. 8) and large facial hemangiomas may be asso-
ciated with visceral hemangiomas. Subglottic hemangiomas can manifest with
hoarseness and stridor, and progression to respiratory failure may be rapid.
Approximately 50% of these infants will have associated cutaneous heman-
giomas; thus noisy breathing in an infant who has a cutaneous hemangioma
involving the chin, lips, mandibular region, and neck warrants direct visualiza-
tion of the airway. Approximately 60% of young infants who have extensive
facial hemangiomas in the ‘‘beard’’ distribution will develop symptomatic air-
way hemangiomas.

Fig. 7. Nasal tip hemangiomas are at increased risk for permanent deformity.


Fig. 8. Multiple hemangiomas raise suspicion of visceral involvement.

Extensive cervicofacial hemangiomas may be associated with multiple
congenital anomalies (PHACES syndrome) (Fig. 9). The acronym PHACES
syndrome describes a constellation of findings including Posterior fossa malfor-
mations, large cervicofacial Hemangioma, Arterial anomalies of the head and
neck, Coarctation of the aorta and cardiac defects, Eye anomalies, and Sternal
cleft. This syndrome has a marked female predominance (9:1), and is thought to
represent a developmental field defect that occurs during the 8th through
10th weeks of gestation. Lumbosacral hemangiomas may also be markers for
occult spinal dysraphism (Fig. 10) and anorectal and urogenital anomalies.

Fig. 9. Large, flat facial hemangiomas can be associated with PHACE syndrome.


Fig. 10. Sacral hemangioma. Patient had underlying lipomyelomeningocele.

Imaging of the spine is indicated in all patients who have midline hemangiomas
in this region.
The Kasabach-Merritt phenomenon (KMP), a complication of rapidly enlarg-
ing vascular lesion, is characterized by hemolytic anemia, thrombocytopenia, and
coagulopathy. Most reported cases present in the first weeks of life. Recently, the
differences between the vascular lesions that induce this phenomenon and classic
hemangiomas of infancy have been emphasized. The vascular tumors that cause
KMP are large, deep red-blue color, firm, grow rapidly, have no sex predilection,
tend to proliferate for a longer period (2– 5 years), and have a different histologic
pattern. It is now clear that most patients who have true Kasabach-Merritt
phenomenon do not have hemangiomas of infancy, but rather other more
aggressive proliferative vascular tumors. Kaposiform hemangioendotheliomas
and tufted angiomas are the tumors most often associated with this phenomenon,
and they require aggressive, (often multi-modality) treatment and carry a
significant mortality rate.
Vascular malformations are congenital abnormalities that are composed of
anomalous capillaries, veins, lymphatics, or a combination thereof [14]. It is
important to differentiate these lesions from proliferative vascular tumors during
infancy, because they have a very different natural history. Precise use of accurate
terminology is essential to determine potential complications, associations, and
treatment options.
Transient macular stains (stork bite, salmon patches, or angel kiss) are present
in up to 70% of normal newborns. They are usually found on the nape of the
neck, the eyelids, the glabelar region of the forehead, and less commonly on the
upper lip. In a prospective study of affected infants, most of the facial lesions had
faded by 1 year of age, but those on the neck were more persistent. Surveys of
adult populations confirm the persistence of the nuchal lesions in approximately
one fourth of the population. The terms ‘‘butterfly-shaped mark’’ or ‘‘sacral
medial telangiectatic vascular nevi’’ are often used to describe similar vascular
stains located on the midline sacral region of the back.
Port wine stains (nevus flammeus) are malformations of the superficial
capillaries of the skin. The term capillary malformation is more accurate and
should be used, especially if the lesion is associated with additional vascular


malformations. Capillary malformations are present at birth and should be con-
sidered stable developmental defects. They may be only a few millimeters in
diameter or may cover extensive areas, occasionally involving up to half the
body surface. They do not proliferate after birth; any apparent increase in size is
caused by growth of the child. A capillary malformation may be localized to any
body surface, but facial lesions are the most common. Lesions are pink-red,
sharply demarcated macules and patches in infancy. With time they darken to a
purple or port wine color and may develop a pebbly or slightly thickened surface.
Capillary malformations are often dermatomal in their distribution. Currently, the
most successful treatment modality in use for superficial capillary malformations
is the pulsed dye laser, which is usually quite effective in fading these lesions.
The efficacy of laser treatment is further increased if undertaken in infancy.
Most capillary malformations occur as isolated defects and do not indicate
systemic malformations. Rarely, they may be a clue to the presence of defects in
the eye or certain vascular syndromes. Children who have facial port wine stains
involving skin innervated by the V1 or V2 branches of the trigeminal nerve
should have a thorough ophthalmologic examination in infancy, because these
children are at risk for glaucoma or Sturge-Weber syndrome [15]. Sturge-Weber
syndrome, or encephalofacial angiomatosis, consists of a facial capillary malfor-
mation (usually in the cutaneous distribution of the first branch of the trigeminal
nerve), a leptomeningeal venous malformation, mental retardation, seizures,
hemiparesis contralateral to the facial lesions, and ipsilateral intracortical calci-
fication. Ocular manifestations are frequent and include buphthalmos, glaucoma,
angioma of the choroid, hemianoptic defects, and optic atrophy. Roentgenograms
of the skull of the older child show pathognomonic ‘‘tramline,’’ double-contoured
calcifications in the cerebral cortex on the same side as the capillary malforma-
tion. CT may detect the calcifications in the younger child before they are
apparent on roentgenograms; however, MRI has replaced CT as the diagnostic
modality of choice. The prognosis depends on the extent of cerebral involvement,
rapidity of progression, and response to treatment. Anticonvulsant therapy and
neurosurgical procedures have been of value in some patients.
´
Klippel-Trenaunay syndrome is a rare entity characterized by a capillary
malformation, venous varicosities (venous malformation), and overgrowth of the
bony structures and soft tissues of the involved extremity. Complications include
severe edema, phlebitis, thrombosis, ulceration of the affected area, and vascular
malformations involving the viscera. Port wine stains also occur with moderate
frequency in Beckwith-Wiedemann syndrome (macroglossia, omphalocele, mac-
rosomia, and cytomegaly of the fetal adrenal gland), Roberts syndrome, and
Cobb syndrome (cutaneomeningospinal angiomatosis).
Venous malformations present as soft, blue compressible plaques and nodules
that may occur on any skin surface. They usually appear at birth and enlarge
slowly over time secondary to engorgement of the anomalous vessels. Venous
malformations may be very small and of minimal concern, whereas very large
lesions can be severely disfiguring and may be complicated by thrombosis,
infection, and edema of surrounding tissue.


Lymphatic malformations
Lymphatic malformations (lymphangiomas) are composed of dilated lymph
channels that are lined by normal lymphatic endothelium. They may be
superficial or deep, and often are associated with anomalies of the regional
lymphatic vessels.
The term ‘‘lymphangioma circumscriptum’’ is used to describe the most
common type of lymphatic malformation, and may be present at birth or appear
in early childhood. Areas of predilection are the oral mucosa, the proximal limbs,
and the flexures. These lesions consists of 2- to 5-mm, red-to-purple, gelatinous
papules clustered on a red-to-brown patch or plaque.
Cystic hygroma is a benign, congenital multilocular mass of anomalies and
cystic lymph vessels that is usually found in the neck region. Surgical excision or
sclerotherapy are the available treatment options for venous and lymphatic
malformations. The tumors tend to increase in size and should be treated by
surgical excision.
Epidermal nevi are a group of lesions that are found commonly in the neonatal
period. Most of them consist of an overgrowth of keratinocytes and often have an
identifiable differentiation toward one of the appendages normally found in skin.
They vary considerably in their size, clinical appearance, histologic character-
istics, and evolution, depending on their topographic location. Lesions occurring
in sites normally rich in sebaceous glands (eg, the scalp) may look like sebaceous
nevi, whereas others, found in areas where the epidermis is thick (eg, the elbow),
look primarily warty in nature.
The most common type of epidermal nevus in the newborn infant is the
sebaceus nevus (of Jadassohn), a yellow or pink, hairless, slightly elevated plaque
on the scalp, forehead, or face (Fig. 11). The surface is velvety and often
papillated. These lesions have a characteristic shape, oval or lancet-shaped on the
scalp and more linear on the face. This lesion is usually an isolated finding
without extracutaneous manifestations. The nevus sebaceus syndrome is a very
rare entity in which multiple neurologic, ocular, and skeletal anomalies are
associated with a very large, often facial, nevus sebaceus. Because of a signifi-

Fig. 11. Nevus sebaceus on forehead.


cant incidence of neoplastic growths within these lesions, surgical excision is
usually recommended.

Pigmented lesions
The most frequently encountered pigmented lesion is the Mongolian spot
(dermal melanosis), which occurs in 70% to 96% of black, Oriental, and Native
American infants, and in approximately 5% of white infants. Although most of
these lesions are found in the lumbosacral area, occurrence at other sites is not
uncommon. The pigmentation is gray-blue in color and completely macular, with
ill-defined borders. It may cover an area 10 cm or larger in diameter. Most of
these lesions gradually disappear during the first few years of life, but aberrant
lesions in unusual sites are more likely to persist.
´
Cafe-au-lait spots are pigmented macules that may be present in the newborn
infant but tend to develop during childhood. They range in color from very light
´
brown to a chocolate brown and are usually oval in shape. Classically, cafe-au-
´
lait macules are associated with neurofibromatosis. As many as five cafe-au-lait
macules are found in 1.8% of newborns and 25% to 40% of normal children and
´
have no significance [16]. Children who have multiple cafe-au-lait macules
should be evaluated carefully for additional stigmata of neurofibromatosis type 1.
Six or more lesions measuring greater than 0.5 cm in length are suggestive of the
diagnosis, especially when accompanied by freckling in the flexures. The axillary
´ ´
and inguinal freckles represent tiny cafe-au-lait macules. Cafe-au-lait spots are
usually the first cutaneous lesions to appear in a patient who has neurofibroma-
tosis, but additional genetic and clinical investigations may be required to
establish a diagnosis. National Institutes of Health consensus criteria for neu-
rofibromatosis type 1 are listed below; definitive diagnosis requires meeting at
least two of the criteria:

´
Six or more cafe-au-lait macules 0.5 cm (children) or 1.5 cm (adults)
Two or more neurofibromas or one plexiform neurofibroma
Axillary or inguinal freckling
Optic glioma
Two or more Lisch nodules
Distinctive bone changes
First-degree relative with neurofibromatosis type 1

The pigmented patches of McCune-Albright syndrome (polyostotic bone
´
dysplasia, cafe-au-lait spots, and multiple endocrine disorders) are also referred
´
to as cafe-au-lait macules; however, they are usually unilateral, elongated, and
large (more than 10 cm), and often have a ragged, irregular border.
Approximately 1% to 2% of newborn infants are born with congenital
melanocytic nevi. Small lesions (as opposed to giant pigmented nevi) are slightly
elevated plaques, often with an oval or round configuration. Most lesions are dark
brown in color; however, in the neonatal period these nevi may appear red or


Fig. 12. Congenital melanocytic nevus on scalp this lesion presented with a red-brown color.

light brown in color, especially when located on the scalp (Fig. 12). The
pigmentation within an individual lesion is often variegated or speckled, with
an accentuated epidermal surface ridge pattern. Textural changes, deeper pig-
´
mentation, and elevation help to differentiate these lesions from cafe-au-lait
macules. Thick dark, coarse hair is frequently associated with congenital
melanocytic nevi. These lesions are variable in size, but most are solitary and
not associated with extracutaneous manifestations. Histologically, they are
characterized by the presence of nevus cells in the dermis, and most will have
nevus cells extending into the deeper dermis and fat. Although there is no clear-
cut evidence that the small congenital nevus is a premalignant lesion, it is the
prevailing opinion that these lesions pose a small increased risk for development
of malignant melanoma, mostly during adult life. Although controversial, many
dermatologists advise removal of these lesions before or near the time of puberty.
Should the family elect to observe rather than excise the nevus, periodic
evaluation of the lesion for surface changes and associated symptoms should
be performed. An excisional biopsy is indicated in instances in which malignant
change is suspected.
Giant congenital nevi are defined as nevi that will be 20 cm or greater in
adulthood. These lesions can be one of the most dramatic birth defects observed.
These nevi may occupy 15% to 35% of the body surface, most commonly
involving the trunk or head and neck region (Fig. 13). The pigmentation often is
variegated from light brown to black. The affected skin may be smooth, nodular,
or leathery in consistency. Prominent dark hypertrichosis is often present. Almost
invariably, numerous smaller, 1 to 5-cm, light brown patches, the so-called
‘‘satellite nevi,’’ are distributed diffusely. Approximately 6% to 8% of these
patients develop malignant melanoma in the nevus over a lifetime [17]. Rarely,
other malignant tumors have been reported in these lesions (rhabdomyosarcoma,
neuroblastoma, liposarcoma). Neurocutaneous melanosis is rarely associated with
giant congenital melanocytic nevi in an axial distribution. These patients may
present with hydrocephalus, seizures, and death from an intracranial melanoma in
early childhood. Because of the significant incidence of malignant degeneration,


Fig. 13. Giant congenital melanocytic nevus on upper back.

the hideous deformity, and the intense pruritus that may accompany these lesions,
surgical excision is usually attempted. This process is usually done in stages, and
the use of tissue expansion techniques has greatly enhanced the ability to obtain
acceptable cosmetic results after surgical removal of large lesions.

Developmental anomalies
Developmental abnormalities of the skin are a diverse group of anomalies that
represent errors in morphogenesis. By definition, they are present at birth, and
most are diagnosed in infancy. They may range in severity from the incon-
sequential to the serious, and in some instances they represent a marker for more
significant extracutaneous developmental abnormalities [18].
Supernumerary digits may be small pedunculated papules to normal-sized and
appearing digits containing cartilage, bone, and nail. These anomalies may be
bilateral or multiple; however, they are likely to be solitary and on the ulnar side
of the fifth digit (Fig. 14). Ligation with suture should not be attempted. This may
result in skin necrosis, infections, and painful neuromas, even outside the

Fig. 14. Patient with supernumerary digit.


Fig. 15. Hair collar sign. This patient had an underlying dermal sinus.

newborn period. Instead, supernumerary digits should be surgically excised with
careful dissection of the associated nerve if present [19].
Cranial dysraphism is a general term for defects in cranial closure. More
specific terms, such as cephalocele and meningocele, represent the herniation of
intracranial structures, tissue, and fluid or the meninges and cerebrospinal fluid
through a defect in the cranium, respectively. Large lesions rarely present
diagnostic challenges; however, smaller lesions are often missed or improperly
diagnosed. All exophytic scalp nodules should be extensively evaluated, because
up to one third of all congenital, nontraumatic scalp nodules have connection to
the underlying central nervous system [20].
The clinical appearance of cephaloceles is variable. They are usually soft,
compressible, round nodules that appear in or near the midline of the frontal,
parietal, and occipital regions. They may be covered with blue translucent film
or normal skin. Other cutaneous clues may raise the suspicion of cranial dys-
raphism. These include the ‘‘hair collar sign,’’ capillary malformations, cutaneous
dimples, or sinuses. The hair collar sign (Fig. 15) is visible as ring of more dense,

Fig. 16. Human tail with underlying lipoma in an infant with a lipomyelomeningocele.


Table 2
Cutaneous lesions associated with spinal dysraphism
High index of suspicion Low index of suspicion
Hypertrichosis Telangiectasia
Dimple (large, 2.5 cm from anal verge) Capillary malformation (port wine stain)
Acrochordans/pseudotails/true tails Hyperpigmentation
Lipomas Melanocytic nevi
Hemangiomas Small sacral dimples ( 2.5 cm from anal verge)
Aplasia cutis or scar Teratomas
Dermoid cyst or sinus
Adapted from Drolet BA, Conlon JD. Developmental abnormalities. In: Eichenfield LF, Frieden IF,
Esterly NB, editors. Textbook of neonatal dermatology. Philadelphia: WB Saunders; 2001. p. 123.

darker, and course hair (versus the normal scalp hair) surrounding the defect [21].
This finding may also be associated with aplasia cutis [22].
Incomplete fusion of the spinal axis, or spinal dysraphism, may be clinically
obvious, or more frequently subtle to imperceptible. The small, often, occult
anomalies, such as a tethered cord, may be asymptomatic until later in life. Early
diagnosis of these malformations may lead to interventions that might prevent the
development of permanent neurologic consequences. Cutaneous lesions may be
the first clue to these underlying defects. Knowledge of these skin findings allows
a health care provider to determine the necessity of further evaluation and
imaging to rule out spinal dysraphism.
As always, a thorough history and physical examination should be performed,
because it may provide valuable additional insight. Frequent fevers, urinary tract
infections, recurrent meningitis, or a positive triple screen can raise suspicion in
the neonatal period. The entire vertebrae should be visualized and palpated.
Furthermore, careful inspection of the urogenital system and rectum may reveal
abnormalities associated with incomplete closure of the spinal axis [23].
The cutaneous markers that are most likely to signal the presence of
underlying occult spinal dysraphism include hypertrichosis, large dimples, true
or pseudotails (Fig. 16), lipomas, hemangiomas, absence of skin, and cystic or
sinus tract lesions (Table 2) [24 – 26]. These lesions are typically found in the
midline lumbosacral region, but may be found anywhere along the vertebral
column. Their presence should prompt ultrasonographic or magnetic resonance
imaging of the spine.

Selected inherited blistering diseases
Epidermolysis bullosa (EB) is a group of rare inherited blistering disorders
characterized by blister formation caused by minor frictional trauma [27]. These
diseases are the result of various genetic defects in essential dermal-epidermal
adhesion molecules, which anchor the basal keratinocytes to the underlying


papillary dermis. Over 20 subtypes of EB are described, and they have been
divided into three major forms (simplex, junctional, and dystrophic), based on the
level of skin separation, the pattern of inheritance, and the presence or absence of
scarring. In several variants, internal involvement may occur, and may be life-
threatening. Because blister formation is due to defective adhesion components
and not antibody-mediated, inflammation is not found in EB.
Six subtypes of epidermolysis bullosa simplex (EBS) have been described,
with all but one being inherited in an autosomal dominant fashion. A very rare
form of EBS associated with muscular dystrophy is inherited in an autosomal
recessive pattern. EBS is the clinical manifestation of defective keratin filaments,
which result in fracturing of the keratinocytes within the epidermis. The
subsequent blister is superficial and heals without scarring. The blistering is
often congenital from passage through the birth canal. In the neonate, blistering
and large erosions primarily affect the feet, hands, neck, and lower legs. As
the child begins to crawl and walk, blister formation is predominantly seen on
the knees, feet, buttocks, elbows, and hands. The severity of each phenotype
varies greatly.
Junctional epidermolysis bullosa (JEB) is an autosomal recessive disorder
with subepidermal blistering occurring within the dermal-epidermal junction.
Several key molecules that help form the hemidesmosomes of the skin are
defective or absent in JEB. Several subtypes of JEB have been described,
encompassing a spectrum from life-threatening disease to relatively minor
involvement. Herlitz-JEB is the most severe variant of all EB patients, and is
associated with a 50% to 80% mortality in the first 2 years of life. These
infants have massive erosions that heal very slowly, often forming thick
granulation tissue. The nail plates are often absent at birth, replaced by exuberant
granulation tissue. Because of the large erosions, these infants are prone to
infection, electrolyte imbalance, temperature instability, and high metabolic
demands. Herlitz-JEB is associated with airway disease as well as numerous
systemic complications, including growth retardation, ocular problems, and
several gastrointestinal diseases. One subset of JEB is associated with pyloric
atresia [28].
In dystrophic epidermolysis bullosa (DEB), blister formation occurs below the
dermal-epidermal junction in the superficial papillary dermis. It is inherited in
both autosomal dominant and recessive patterns, with the dominant variant being
mild and the recessive form much more severe. In recessive dystrophic-EB,
widespread blistering begins at birth and results in contractures with marked
scarring and milia formation. Fusion of the digits with encasement of the fingers
and toes and subsequent autoamputation leads to the classic ‘‘mitten deformities’’
of recessive dystrophic-EB. Systemic involvement may be severe, with gastro-
intestinal complications being the most common. During infancy and early child-
hood, septicemia, pneumonia, and overwhelming secondary cutaneous infections
may arise. Patients who have recessive dystrophic-EB are also at increased risk
for the development of nonmelanoma skin cancers. As a rule, nail changes are
seen in all forms of DEB.


As a general rule, the management of all forms of EB is symptomatic and
palliative, consisting of good wound care, prevention of trauma, and treatment
of infection.
Incontinentia pigmenti is a rare, X-linked dominant disorder that affects the
skin, bones, eyes, and central nervous system. Almost all patients are female, but
affected males have been reported and are thought to represent genetic mosaicism
or Klinefelter syndrome.
The cutaneous lesions usually are present at birth and have three distinct
morphologic stages. Initially there are 2- to 4-mm, clear-to-yellow vesicles,
which are clustered in a linear configuration. These vesicular lesions appear in
crops during the first few weeks of life and most commonly occur on the trunk
and limbs. At times they appear distinctly pustular or crusted. Gray to brown,
warty, linear and swirled plaques develop next; these are most prominent on the
distal limbs. The third stage consists of patterned, macular brown hyperpigmen-
tation in streaks and whorls on the trunk and extremities. Occasionally, pigmen-
tation may accompany some of the early lesions, and all three more morphologies
may be present at the same time. A fourth stage, consisting of atrophic
hypopigmented streaks, has been described during adulthood in some individu-
als. Nail hypoplasia, areas of alopecia, and ocular and skeletal abnormalities also
may be detected. Delayed dentition, partial anodontia, and abnormalities of the
central nervous system (CNS) can occur, but may not be apparent during the
neonatal period. There is often peripheral eosinophilia during the vesicular
phase of the disease. The diagnosis should be considered when inflammatory
vesicles arranged in lines are seen in a newborn female infant. Biopsy of a small
blister demonstrates a subcorneal vesicle filled with eosinophils. No specific
therapy is required for the skin lesions; if inflammation becomes excessive during
the vesicular phase, treatment with compresses and topical steroids may be
helpful. An ophthalmologic examination is indicated for all infants who have
this disorder.

Miscellaneous skin disorders

Mastocytosis
The abnormal proliferation of mast cells in the skin is referred to as
mastocytosis, and, depending on the extent of involvement, can lead to various
manifestations, including mastocytoma, urticaria pigmentosa, and diffuse cuta-
neous mastocytosis. Mastocytoma appears as one or several, isolated, skin-
colored to light yellow-brown, 1 to 5-cm macules, nodules, or plaques, typically
on the trunk or extremities. Mastocytomas are the most common presentation of
mastocytosis in the neonate. They are often transient and frequently exhibit a
wheal and flare phenomenom with stroking, a positive Darier’s sign. Bullous
lesion may develop. Most lesions of mastocytoma resolve spontaneously before
adolescence. Mastocytosis may also present as urticaria pigmentosa (UP) or


diffuse cutaneous mastocytosis (DCM). These entities represent progressively
more extensive infiltration of the skin with mast cells, with potential involvement
of the viscera, albeit rare in both. The lesions tend to be smaller, darker, and
yellow-red pigmented, with more diffuse involvement. DCM may present as
generalized thickening of the skin, often being described as ‘‘doughy.’’ The
predominant symptom in all is pruritus. Additional symptoms, depending the on
extent of involvement, may be present, including flushing, hypotension, diarrhea,
vomiting, or abdominal pain. These may be controlled by the use of hydroxyzine,
long-acting antihistamines, H2 blockers, or oral cromolyn sodium, if warranted.
Agents that trigger histamine release (eg, narcotics, aspirin, or alcohol) should be
avoided. Resolution in the second decade is the typical course; however, some
may persist or even progress with advancing age.

Zinc deficiency and acrodermatitis enteropathica
Zinc is an essential elemental nutrient required for normal growth and de-
velopment [29]. Low levels of zinc may result from malabsorption or inadequate
intake. In acrodermatitis enteropathica, a rare, autosomal recessive disorder, the
ability to absorb zinc is impaired. The child presents with eczematous erosions
and plaques located in the periorificial, acral, and diaper areas; creating possible
confusion with atopic and seborrheic dermatitis. Diarrhea and alopecia may
also be present. An infant may also develop similar findings secondary to
inadequate intake of zinc. This zinc deficiency dermatitis occurs most commonly
in premature infants who have high nutritional demands, or in infants being
breast fed by mothers who have low or absent levels of zinc in their breast milk
[29]. The diagnosis in both acrodermatitis enteropathica and zinc deficiency
dermatitis is made by measurement of serum zinc levels. Supplementation with
zinc sulfate leads to rapid resolution, and lifelong treatment may be required for
acrodermatitis enteropathica.

Ichthyotic disorders
Ichthyosis is a term that is used to describe excessive scaling of the skin. There
are four distinct subtypes of ichthyosis: (1) lamellar, (2) X-linked, (3) epider-
molytic hyperkeratosis, and (4) vulgaris. Ichthyosis in the newborn period may
present with scaling, erythroderma, collodion membrane, or the thickened plates
of harlequin ichthyosis. Two descriptive terms have been used for ichthyotic
conditions that occur only in the newborn: harlequin ichthyosis (harlequin fetus)
and collodion baby.
Harlequin ichthyosis (harlequin fetus) is an extremely rare and very severe
disorder of keratinization inherited as an autosomal recessive trait. The skin of
affected infants is markedly thickened, hard (armorlike), and with deep crevices
running both transversely and vertically. The fissures are most prominent over
areas of flexion. Rigidity of the skin around the eyes results in marked ectropion,
although the globe is usually normal. The ears and nose are underdeveloped,


flattened, and distorted. The lips are everted and gaping, thus producing a ‘‘fish
mouth’’ deformity. Extreme inelasticity of the skin is associated with flexion
deformity of all joints. The hands and feet are ischemic, hard, and waxy in
appearance, with poorly developed distal digits.
Most harlequin fetuses are born prematurely, between 32 and 36 weeks’
gestation, adding to their morbidity and mortality. Complications include sepsis,
distal gangrene, and difficulties with feeding and respiration. Most infants die in
the neonatal period. Aggressive topical care is required for these infants,
including a humid environment, liberal use of bland emollients, and careful
monitoring of electrolyte needs.
The collodion baby is less severely affected than the infant who has harlequin
ichthyosis and also may represent a phenotypic expression of several genotypes.
Infants affected with this condition usually have lamellar ichthyosis or congenital
ichthyosiform erythroderma. Sjogren-Larsson syndrome, Conradi-Hunermann
syndrome, trichothiodystrophy, dominant lamellar ichthyosis, and neonatal
Gaucher’s disease account for the remainder of the infants born with a collodion
membrane. Rarely, shedding of the collodion membrane results in a normal
underlying integument (lamellar exfoliation of the newborn). Collodion babies
often are born prematurely. The infant is covered with a cellophanelike mem-
brane, which by its tautness may distort the facial features and the digits. Less
commonly, only part of the integument is involved. The membrane is shiny and
brownish-yellow, resembling an envelope of collodion or oiled parchment, and
may be perforated by hair. The presence of ectropion, eclabium, and crumpled
ears causes these infants to resemble one another for the first few days of life.
Fissuring and peeling begin shortly after birth, and large sheets may desquamate,
revealing erythema of variable intensity. Once the membrane has fissured, no
respiratory difficulties are encountered. Complete shedding of the collodion
membrane may take several weeks. Despite the apparent thickened skin, these
infants have an abnormal epidermal barrier that may lead to complications such
as dehydration, electrolyte imbalance, and temperature instability, as well as
cutaneous and systemic infection. Pneumonia from aspiration of squamous cells
found in the amniotic fluid is another potential complication. The infant should
be placed in an environment with increased humidity, and prompt attention paid
to signs of infection. A bland emollient will suffice for lubrication during the
neonatal period.
X-linked ichthyosis is a rare disorder that may cause alterations in the skin,
eye, and testes of affected infants. Steroid sulfatase (arylsulfatase) deficiency is
the cause of the clinical manifestations of X-linked ichthyosis. Most, but not
all, patients have cutaneous findings at birth and 80% to 90% show scaling by
3 months of age. Rarely, a collodion membrane may be present. The hyperkera-
tosis is variable, but typically the scales are large, thick, dark and prominent over
the scalp, neck, anterior trunk, and extensor extremities. Sparing of palms and
soles and partial sparing of the flexures are helpful diagnostic features. Systemic
manifestations are generally absent, and complications are rare. This form of
ichthyosis is clinically apparent in affected males but not in heterozygous


females. Cryptorchidism is seen in approximately 25% of affected males; these
patients may be at increased risk for testicular cancer. Deep stromal corneal
opacities have also been found in about 50% of males who have the disorder,
and in a smaller percentage of female carriers, but they do not affect vision.
Female carriers of the gene for X-linked ichthyosis, when pregnant with an
affected male fetus, have a deficiency of placental steroid sulfatase, reflected by
low maternal urinary estriol excretion and a difficult or prolonged labor often
requiring intervention.
Babies affected with the rare condition multiple sulfatase deficiency may have
similar cutaneous findings, but also have systemic features of a storage disease
(metachromatic leukodystrophy), because they have a deficiency of several other
sulfatases. Approximately 10% of patients will have a contiguous gene deletion
syndrome; this condition results from a larger deletion of the terminal short arm
of the X chromosome. Deletion of the genes that are contiguous with the steroid
sulfatase gene may result in mental retardation, Kallmann syndrome, and a bone
dysplasia (X-linked recessive chondrodysplasia punctata).
Infants who have lamellar ichthyosis may have collodion membranes at birth,
and are commonly of low birth weight. After the collodion membrane is shed,
mild erythroderma, large, dark, plate-like scales, severe ectropion, alopecia, and
moderate keratoderma of the palmar and plantar surfaces characterize the
phenotype. These children have involvement of their entire skin surface, which
results in severe disfigurement, particularly of the face. Generally, the condition is
inherited as an autosomal recessive trait. Patients who have this type of ichthyosis
may suffer severe heat intolerance as a result of plugging of the eccrine gland
ducts. In severely erythrodermic patients, growth retardation may be a compli-
cating factor. Some patients who have the lamellar phenotype have been found to
have mutations in keratinocyte transglutaminase I.
Neonates who have congenital nonbullous ichthyosiform erythroderma usu-
ally have a collodion membrane at birth. It may be very difficult to differentiate
these infants from patients who have lamellar ichthyosis in the neonatal period
and in early childhood. Generally they tend to have more erythema and finer
scale than are seen in patients who have lamellar ichthyosis. As the child ages, the
skin continues to be red-pink, with diffuse fine scaling and mild keratoderma,
much less prominent than in children who have lamellar ichthyosis.
The infant who has epidermolytic hyperkeratosis (bullous congenital ichthyo-
siform erythroderma) may have large areas of denuded skin and has recurrent
blistering in the neonatal period. These skin lesions may be confused with those
of staphylococcal scalded-skin syndrome or epidermolysis bullosa. Trauma
should be avoided, and applications of antibacterial preparations and nonocclu-
sive moisturizers should be used as needed. Bullous lesions become less
prominent as the infant grows older, and may eventually disappear completely.
Hyperkeratosis becomes more pronounced as the child ages, particularly in the
flexural areas, in which the dark, warty scales often assume a ridged pattern.
Maceration and secondary bacterial colonization result in a strong unpleasant
odor that is difficult to eradicate. The histopathologic picture in epidermolytic


hyperkeratosis is diagnostic and consists of vacuolization of the epidermis,
abnormally large, clumped keratin granules, hyperkeratosis, and an increased
granular layer. Obtaining an accurate family history is critical because this
disorder is inherited as an autosomal dominant trait. The gene defects of epider-
molytic hyperkeratosis have been found in the DNA that encodes for keratins
1 and 10.
Ichthyosis vulgaris is a common, less severe form of scaling that is inherited
as an autosomal dominant disease. Fine white scales without significant erythema
become prominent by 6 to 9 months of age. The scaling is most prominent on the
lower legs and buttocks; the face is usually spared. Associated skin conditions
include keratosis pilaris and atopic dermatitis. The diagnosis is made clinically,
and treatment consist of bland emollients.

Histiocytosis
Langerhan’s cell histiocytosis is a rare proliferative disorder most often seen in
young infants. Classically, the disease is caused by infiltration of the skin, bone,
and viscera with histiocytes. Historically, several different subtypes of Langer-
hans’ cell histiocytosis have been described; however, currently they are all
considered to be within the spectrum of the same disease process [30]. It is
variable in its presentation and the clinical course varies from self-limited skin
nodules to progressive multisystem disease. Congenital skin lesions of histio-
cytosis present as small, 2- to 10-mm, firm, often necrotic, papules. The number
of lesions is variable, but most often they range from 2 to 12. Rarely, the infiltrate
may cause hemorrhagic vesicles with a central umbilication. They may occur at
any site, but have a predilection for the groin and palms and soles. Larger
ulcerated nodules or tumors may be present. Older infants may present with an
eczematous eruption in the groin and scalp. The scalp dermatitis often mimics
seborrheic dermatitis. Oral lesions result in gingival hyperplasia, ulceration, and
natal teeth. Extracutaneous findings include lytic bone lesions, exopthalmos,
diabetes insipidus, lymphadenopathy, hepatosplenomegaly, and invasion of the
lungs and gastrointestinal tract. Diagnosis is made by skin biopsy, which typically
reveals dermal infiltrate of histiocytes that are S-100 and Cd1A positive.

Summary
The concise discussions of the preceding topics are by no means all-inclusive,
but highlight the prerequisite ability of all pediatric health care professionals to
recognize both normal and abnormal dermatologic conditions and their signifi-
cance. Cultivated by worried parents who had persistent questions regarding
different skin lesions and pioneering physicians, the field of neonatal dermatol-
ogy was long ago seeded and continues to grow. Advances in skin biology and
genetics will undoubtedly lead to further growth and understanding, as well as to
exciting new potential diagnostic and therapeutic modalities.


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