6 Transient Skin Disorders in The Neonate and Young Infant
6 Transient Skin Disorders in The Neonate and Young Infant
6 Transient Skin Disorders in The Neonate and Young Infant
CHA PTER 6
Harper’s Textbook of Pediatric Dermatology, Fourth Edition. Edited by Peter Hoeger, Veronica Kinsler and Albert Yan.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
Chapter 6 Transient Skin Disorders in the Neonate and Young Infant 73
• Cutis marmorata
Pigmentary skin lesions
• Salmon patch
• Harlequin colour change • Mongolian spots
• Erythema neonatorum and acrocyanosis • Pigmentary lines of the newborn
• Periungual hyperpigmentation
Transient vesicopustular eruptions
Miscellaneous
• Erythema toxicum neonatorum
• Transient neonatal pustular melanosis • Subcutaneous fat necrosis
• Miliaria • Sucking blisters, erosions and calluses
• Benign neonatal cephalic pustulosis • Adnexal polyp
• Neonatal acne • Pedal papules of infancy
• Infantile acropustulosis • Perineal groove
Physiological desquamation
A fine desquamation that occurs in most newborns may Fig. 6.1 Lanugo on the dorsum.
persist during the first 3 months of life [11]. In healthy
full‐term neonates this process begins on the first or second Lanugo is often located on the dorsum, shoulders, face
day of life and in preterm infants it starts at 2–3 weeks of and scalp (Fig. 6.1). The first coat of lanugo normally
age. Desquamation is more evident on hands, feet and sheds in utero during the last trimester of gestation, and
ankles. In post‐term newborns it tends to be more gener consequently it is more prominent in preterm newborns
alized and thicker [2,11]. In extreme cases in which des [8,12]. Lanugo must be differentiated from congenital
quamation is persistent or severe, consideration should hypertrichosis lanuginosa, gingival fibromatosis with
be given to evaluation for other underlying disorders hypertrichosis, Cornelia de Lange syndrome and other
such as congenital syphilis or ichthyosis [11,12]. rare disorders associated with excess body hair [4,6,8,12].
Differential diagnosis includes milia, miliaria cristallina • Basal cell naevus syndrome (Gorlin syndrome)
and neonatal acne [10]. • Generalized basaloid follicular hamartoma syndrome
• Brooke–Spiegler syndrome
Milia • Basex–Dupré–Christol syndrome
Milia are small superficial keratinous cysts found com • Rombo syndrome
monly on newborn skin, produced by retention of keratin • Pachyonychia congenita
within the superficial dermis. They originate from the • Atrichia with papular lesions
pilosebaceous apparatus of vellus hair [6,13]. Milia may • Nicolau–Balus syndrome
be divided into two categories: primary milia which arise • Keratitis–ichthyosis–deafness syndrome (KID syndrome)
spontaneously, and secondary milia due to medications,
Secondary milia
diseases and cutaneous healing after trauma. Neonatal
milia are included in the first subtype [16,17]. They occur • Epidermolysis bullosa
in 40–50% of neonates, with no racial or gender prepon • Porphyria
derance. Milia are multiple superficial pinpoint white‐
yellowish papules 1–2 mm in diameter located on the
face, especially the nose, but also cheeks, forehead and
chin (Fig. 6.2). Scalp, upper trunk and upper limbs may
also be affected. There may be single or multiple lesions.
In some cases, a solitary and usually larger milium may
be present on the areola, genitalia or foreskin [13]. They
disappear spontaneously, usually within a few weeks of
life without scarring, although they may persist for several
months. In cases where profuse and persistent milia are
noted or associated with other anomalies, an underlying
genodermatosis must be suspected (Box 6.2) [6,13,17–19].
Miniature puberty
Maternal hormones, especially androgens, are acquired
transplacentally, producing a neonatal transient hormo
nal elevation that normalizes by 6–8 weeks of age.
Miniature puberty comprises a group of spontaneously
resolving manifestations secondary to this phenomenon
[6,13,20]. Darkening of the linea alba (linea nigra), areolas
and external genitalia is frequently seen, especially in Fig. 6.3 Miniature puberty: hypertrophic and darkened genitalia in a
female newborn.
non‐Caucasian infants [21]. Breast hypertrophy may
Physiological jaundice
Jaundice is produced by the accumulation of bilirubin in
Fig. 6.2 Milia: tiny whitish cysts. the skin, mucous membranes and sclerae that causes a
Chapter 6 Transient Skin Disorders in the Neonate and Young Infant 75
yellowish colouration. Physiological jaundice refers to the Adams–Oliver syndrome, phakomatosis pigmentovas
common and in general harmless jaundice seen in babies cularis and van Lohuizen syndrome [24,25]. Rarely,
during the first days of life, with no significant underly neonatal lupus erythematosus may present with cutis
ing disease. It is common in the first week, affecting marmorata telangiectatica congenita‐like lesions. Reticu
60% of term and 80% of preterm newborns. Up to 30% late capillary malformations (port wine stains) may also
of predominantly breastfed babies are still jaundiced at resemble cutis marmorata; however, these reticulate cap
Epstein pearls
Epstein pearls are single or multiple, small (1–3 mm),
whitish keratin‐filled cysts, located at the junction of the
hard and soft palates or at the palatal midline (Fig. 6.7).
They have been reported in 65–85% of newborns, and
arise from epithelial remnants along the line of fusion of
Bohn nodules
Bohn nodules are isolated or multiple firm small white
cystic structures located on the vestibular and lingual
surface of the alveolar ridges (Fig. 6.8). The maxillary arch
is more commonly affected than the mandibular. These
lesions are very common, reported in 85% of neonates.
They are keratin cysts derived from epithelial remnants of
minor salivary glands or from remnants of odontogenic
Oral lesions
Lesions affecting the oral cavity of infants may vary from
benign transient lesions to tumours. Knowledge of these
common conditions is important for appropriate manage
ment of patients and their families.
Transient inclusion cysts or developmental nodules of
the oral mucosa are very frequent, affecting almost 80% of
newborns. They represent the most common oral disor
der in infants, and are located in the alveolar ridges or on
the palate. Based on histological origin and location in the
oral cavity, they can be classified as Epstein pearls, Bohn Fig. 6.8 Bohn nodules: small white cystic structures on the surface of
nodules or dental lamina cysts [43,44]. alveolar ridges.
78 Section 2 Skin Disorders of the Neonate and Young Infant
cyst appears as a translucent, flesh‐coloured or bluish, in early to mid childhood. Superimposed darker spots on
dome‐shaped, compressible lesion on the alveolar ridge classic Mongolian spots may be present [61–64].
of the mandible or maxilla (Fig. 6.10) [45,57,58]. The Histopathology reveals the presence of scattered mel
differential diagnosis includes mucocoele, lymphatic anocytes in the lower dermis associated with occasional
malformation, congenital epulis, dental lamina cysts, melanophages [62].
teratoma and Bohn nodules [58]. Radiological examina Rarely, Mongolian spots may be associated with lysoso
tion shows a natal or neonatal tooth. Cystic content mal storage diseases. In these cases Mongolian spots are
obtained by needle aspiration has a yellowish colouration persistent, aberrant in appearance and extensive, affecting
and low viscosity and presents cholesterol crystals. not only the dorsum but also the ventral aspect of the
Although treatment options include marsupialization or trunk, with darker and progressive pigmentation. An
surgical removal, most eruption cysts resolve spontane extensive confetti appearance has also been observed in
ously within several weeks [45,58]. some patients. The most common lysosomal storage
disease associated with Mongolian spots is GM1 gangli
Sucking pads of the lips osidosis 1, followed by mucopolysaccharidosis type 1
Sucking pads or sucking calluses on the lips are present (Hurler disease) and, to a lesser degree, mucopolysaccha
in both term and preterm neonates, and are considered ridosis type 2 (Hunter disease), mucolipidosis, Niemann–
a physiological adaptive reaction of lip structures to Pick disease and mannosidosis [61,65–68]. Phakomatosis
sucking. They are characterized by painless, whitish and pigmentovascularis consists of the association of a vascu
hyperkeratotic swelling of the lips, predominantly in the lar malformation and a cutaneous melanocytic lesion.
middle of the upper lip. Skin biopsy, if performed, shows Persistent Mongolian spots are part of phakomatosis
hyperkeratosis, epidermal hyperplasia and intracellular pigmentovascularis types II, IV and V, also known as
oedema. They disappear spontaneously after 3–6 months, phakomatosis cesioflammea and phakomatosis cesio
when breastfeeding is stopped [45,59,60]. marmorata [61,62,65,69].
Miscellaneous
Subcutaneous fat necrosis
Subcutaneous fat necrosis of the newborn is a rare and
transient disorder of fat tissue that affects term and post‐
term babies. It is a lobular form of panniculitis that
appears in the first week of life, few cases developing as
late as 6 weeks of age. It has been associated with various
physiological stressors as risk factors, including maternal
factors (gestational diabetes, pre‐eclampsia, smoking or
exposure to passive smoking, intake of calcium channel
blockers, cocaine abuse) and neonatal factors (perinatal
asphyxia, meconium aspiration, umbilical cord prolapse,
hypothermia, obstetrical trauma, Rhesus incompatibility)
[75–79]. Subcutaneous fat necrosis was reported in 1–3%
of neonates managed with moderate therapeutic hypo Fig. 6.12 Subcutaneous fat necrosis: erythematous plaques on the back.
thermia for hypoxic ischaemic encephalopathy. Lesions
may develop in areas directly exposed to the cooling
blanket. Induction of therapeutic cooling improves sur
vival and reduces neurological sequelae in newborns
with hypoxic ischaemic encephalopathy, and has become
common practice in these cases [80–82]. Subcutaneous fat
necrosis was also documented in infants who underwent
whole‐body cooling before cardiovascular surgery [82].
Perinatal asphyxia induces blood shunting from skin and
spleen to brain, heart and adrenal glands, producing
impaired tissue perfusion with local hypoxia [78,82].
Neonatal adipose tissue has a relatively high concentra
tion of saturated fatty acids (stearic and palmitic acids)
with a high melting point that makes them more prone to
solidify and crystallize under cold stress, resulting in adi
pocyte necrosis and subsequent formation of granuloma
tous inflammation [75,76,82]. Also, enzymes involved in
fatty acid metabolism are immature [83].
This condition is characterized by multiple indurated, Fig. 6.13 Subcutaneous fat necrosis (H&E): radially arranged needle‐
shaped birefringent crystals.
erythematous or violaceous painful plaques and nodules
located on the back, shoulders, extremities, buttocks and
cheeks (Fig. 6.12). Some lesions may become fluctuant Skin biopsy in subcutaneous fat necrosis demonstrates
with drainage of liquefied fat from nodules [75,78,82]. lobular panniculitis with fat necrosis, mixed inflammatory
Lesions resolve spontaneously within a few weeks, infiltrate with abundant histiocytes, giant multinucleated
leaving on rare occasions atrophy, fibrosis, scarring, cells with granuloma formation and adipocytes with
ulcers or necrosis. radially arranged needle‐shaped birefringent crystals and
The differential diagnosis most commonly includes, clefts (Fig. 6.13) [75,77]. Fine‐needle aspiration is an
but is not limited to, infection (cellulitis, abscess) and option for diagnosis.
tumours (such as infantile myofibromatosis, rhabdo Although subcutaneous fat necrosis is a benign condi
myosarcoma and deep haemangioma of infancy). tion, complications such as transitory hypoglycaemia,
Sclerema neonatorum is the main differential diagnosis, hypertriglyceridaemia and thrombocytopenia have been
though it is encountered uncommonly. The latter appears reported [75,76,78,79]. Hypercalcaemia is a potentially
in preterm infants with sepsis or underlying disease in life‐threatening complication found in 25–56% of patients.
the first week of life, with generalized hardening of the It usually appears when the cutaneous lesions start to
skin except on palms, soles and genitalia. Its prognosis is regress [75,84]. Although the first 6 weeks of life is the
poor [75,77]. period of highest risk for developing hypercalcaemia,
Chapter 6 Transient Skin Disorders in the Neonate and Young Infant 81
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