72 

CHA PTER 6

Transient Skin Disorders in the Neonate

and Young Infant
SECTION 2: SKIN DISORDERS
OF NEONATES AND INFANTS

Margarita Larralde1,2 & Maria Eugenia Abad1,2

1
 Dermatology Department, Hospital Alemán, Buenos Aires, Argentina
2
 Pediatric Dermatology Department, Hospital Ramos Mejía, Buenos Aires, Argentina

Introduction, 72 Transient vesicopustular eruptions, 76 Miscellaneous, 80

Physiological skin findings, 72 Oral lesions, 77
Transient vascular physiological changes, 75 Pigmentary skin lesions, 79

Abstract (erythema toxicum neonatorum and transient neonatal ­pustular

melanosis), oral lesions (Epstein pearls, Bohn nodules, dental
lamina cysts, natal and neonatal teeth, eruption cyst and sucking
Cutaneous lesions are common in the neonatal period. Most are
pads of the lips), pigmentary lesions (Mongolian spots or congeni-
physiological, transient and benign. Transient skin disorders may
tal dermal melanocytosis, pigmentary lines of the newborn and
be classified as physiological skin disorders (vernix caseosa, phys-
periungual hyperpigmentation) and miscellaneous lesions (subcu-
iological desquamation, lanugo, sebaceous gland hyperplasia,
taneous fat necrosis, sucking blisters and erosions, adnexal polyps,
milia, physiological jaundice, and the collective effects of maternal
pedal papules of infancy and perineal groove). In this chapter we
or placental hormones resulting in what has been referred to as
discuss the categories mentioned above and also consider that
miniature puberty), transient vascular physiological changes (cutis
serious and potentially life‐threatening diseases or associations
marmorata, salmon patch, harlequin colour change, erythema
should sometimes be ruled out.
neonatorum and acrocyanosis), transient vesicopustular eruptions

Key points eruptions, oral lesions, pigmentary lesions and miscellaneous

lesions.
• Transient skin disorders should be differentiated from serious
• Most cutaneous lesions in the neonatal period are physiological,
and potentially life‐threatening diseases, e.g. infections or
transient and benign.
genodermatoses.
• Transient skin disorders include physiological skin disorders,
transient vascular physiological changes, transient vesicopustular

Introduction Most cutaneous lesions in neonates represent transient

Cutaneous lesions are common in the neonatal period.
Most are benign, transient or physiological conditions
that should be differentiated from serious and potentially
life‐threatening diseases that carry more significant risks
of morbidity or mortality, as might be seen with infections
or genodermatoses. For this reason, initial evaluation
should include a careful physical examination and a
complete clinical history focusing on family and prenatal
history, pregnancy, delivery, gestational age, birthweight,
and the presence of general symptoms and specific
anomalies. When needed, useful diagnostic tools include
routine laboratory (blood chemistry and haematological
profile) testing; screening for infections through bacterial,
viral and mycological smears, cultures, antibody detec­
tions and polymerase chain reaction (PCR); skin biopsy;
and genetic testing [1–3].
skin disorders characterized by benign and self‐limiting
conditions (Box 6.1).
Physiological skin findings
Vernix caseosa
This white, greasy and lipophilic natural skin barrier is
present at birth. It is synthesized by fetal keratinocytes
and sebaceous glands in late pregnancy, and formed by
sebaceous secretions and shed epithelial cells and lanugo
[4]. It may cover the entire cutaneous surface of full‐term
babies or may be found concentrated in skin folds and on
the back. After a few hours or days of life, it disappears.
Vernix caseosa may be brownish‐yellow if there was
contact with meconium or as might be seen in cases of
haemolytic disease. Vernix caseosa may have a noticeable
typical odour in a case of neonatal sepsis [4–6]. It consists

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

Box 6.1  Transient skin disorders

Physiological skin disorders • Eosinophilic pustular folliculitis

• Vesiculopustular eruption in neonatal transient myeloproliferative disorder
• Vernix caseosa
• Physiological desquamation
Oral lesions

SECTION 2: SKIN DISORDERS

• Lanugo

OF NEONATES AND INFANTS

• Sebaceous gland hyperplasia • Epstein pearls
• Milia • Bohn nodules
• Miniature puberty • Dental lamina cysts
• Physiological jaundice • Natal and neonatal teeth
• Eruption cyst
Transient vascular physiological changes • Sucking pads of the lips

• 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

mainly of water (81%), but also contains lipids (9%) and

proteins (10%) [7,8]. Vernix caseosa protects neonatal skin
and facilitates the transition from intrauterine to extrau­
terine life. Other functions are skin surface adaptation
after birth, temperature regulation, skin hydration,
wound healing, prevention of water loss and antimicro­
bial action. Vernix caseosa contains several antimicrobial
peptides and proteins (lysozyme, lactoferrin, cathelici­
dins and defensins) that may protect the fetus prenatally
and also facilitate postnatal skin colonization by non­
pathogenic bacteria [4,5,8,9]. Vernix caseosa also acts as a
lubricant to facilitate passage through the birth canal.
Although the traditional practice has been to wipe off the
vernix caseosa after birth, more current recommendations
advocate leaving it in place and avoidance of removal by
wiping or bathing [5,10].

Physiological desquamation
A fine desquamation that occurs in most newborns may
persist during the first 3 months of life [11]. In healthy
full‐term neonates this process begins on the first or second
day of life and in preterm infants it starts at 2–3 weeks of
age. Desquamation is more evident on hands, feet and
ankles. In post‐term newborns it tends to be more gener­
alized and thicker [2,11]. In extreme cases in which des­
quamation is persistent or severe, consideration should
be given to evaluation for other underlying disorders
such as congenital syphilis or ichthyosis [11,12].
Fig. 6.1  Lanugo on the dorsum.
Lanugo is often located on the dorsum, shoulders, face
and scalp (Fig.  6.1). The first coat of lanugo normally
sheds in utero during the last trimester of gestation, and
consequently it is more prominent in preterm newborns
[8,12]. Lanugo must be differentiated from congenital
hypertrichosis lanuginosa, gingival fibromatosis with
hypertrichosis, Cornelia de Lange syndrome and other
rare disorders associated with excess body hair [4,6,8,12].

Lanugo Sebaceous gland hyperplasia

Newborn skin is often covered by fine, soft, nonpig­ Sebaceous gland hyperplasia is found at birth in 50–89%
mented and unmedullated immature hair termed lanugo. of newborns, especially full‐term neonates [10,13,14].
It is replaced by vellus hair during the first months of life. Both sexes are affected equally, with the highest incidence
 74 Section 2  Skin Disorders of the Neonate and Young Infant

in African Americans and the lowest in Asians [10,15].

Box 6.2  Genodermatoses associated with milia
It is produced by the increased activity of the sebaceous
gland stimulated by transplacental transfer of maternal
Primary congenital milia
androgens. It is characterized by multiple tiny whitish‐
yellow papules located at the opening of pilosebaceous • Orofaciodigital syndrome type 1
follicles in areas in which sebaceous glands are prominent • Familial milia with absent dermatoglyphics (Basan syndrome)
SECTION 2: SKIN DISORDERS

• Familial profuse congenital milia

OF NEONATES AND INFANTS

such as around the nose, cheeks, forehead and upper lip

[6,7,13]. No treatment is needed since it generally resolves
spontaneously within the first few weeks after birth. Primary milia developing later in life

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

occur in both sexes, as may neonatal acne and sebaceous

gland hyperplasia [20]. The enlarged breast gland may
secrete a colostrum‐like substance. Male genitalia may
appear well‐developed with hyperpigmentation of scro­
tum. Female genitalia may appear full with clitoral hyper­
trophy and darkening of the labia and vulva (Fig.  6.3).
Within a few days after birth, a whitish, creamy vaginal
discharge can be seen. Rarely, on the third to fourth day of
life, bleeding similar to menses may occur [6,13,20,21].
Hyperpigmentation of labia may occasionally be misdi­
agnosed as naevi.

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
common and in general harmless jaundice seen in babies
during the first days of life, with no significant underly­
ing disease. It is common in the first week, affecting
60% of term and 80% of preterm newborns. Up to 30%
of predominantly breastfed babies are still jaundiced at
Adams–Oliver syndrome, phakomatosis pigmentovas­
cularis and van Lohuizen syndrome [24,25]. Rarely,
neonatal lupus erythematosus may present with cutis
marmorata telangiectatica congenita‐like lesions. Reticu­
late capillary malformations (port wine stains) may also
resemble cutis marmorata; however, these reticulate cap­

SECTION 2: SKIN DISORDERS

OF NEONATES AND INFANTS
1 month of age [4,6,22]. illary malformations are persistent. Extensive reticulate
Red blood cell breakdown produces unconjugated or capillary malformations or congenital livedo reticularis‐
indirect bilirubin which is mostly bound to albumin as it like findings in conjunction with overgrowth or macro­
circulates. Unconjugated bilirubin is metabolized in the cephaly represent some of the cutaneous hallmarks of
liver to produce conjugated or direct bilirubin which macrocephaly–capillary malformation [26,27] and other
passes into the gut and is excreted in stool. Hepatic PIK3‐related overgrowth syndromes.
immaturity associated with a shorter lifespan and higher
concentration of red blood cells is responsible for hyper­ Salmon patch
bilirubinaemia [22]. Also known as naevus simplex, Unna naevus, ‘angel kiss’
Early‐onset or prolonged jaundice may be a sign of or ‘stork bite’, this is the most common vascular stain pre­
pathological jaundice. Pathological jaundice has many sent at birth. It affects almost half of newborns with no
aetiologies including blood group incompatibility gender predilection. It involves the median part of the
(Rhesus or ABO incompatibility), as well as other causes face (glabella, forehead, eyelids, nasal alae and philtrum),
of haemolysis, such as large haematomas (hepatic sub­ the nape and occipital area and in some cases the sacral
capsular or cephalohaematoma), sepsis, in addition region (Fig. 6.4) [26]. Its colour typically varies from pale
to  primary liver disease (Gilbert and Crigler–Najjar pink to bright red with undefined borders that become
syndrome), biliary atresia and glucose‐6‐phosphate more prominent with heat, crying and physical activity.
dehydrogenase deficiency [4,22,23]. Facial lesions usually fade within the first 2 years of life.
Lesions involving the nape may persist [28,29]. Patients
with lumbosacral naevus simplex should have imaging
Transient vascular physiological performed to rule out underlying spinal dysraphism if
changes the naevus is extensive or atypical, or other cutaneous
Cutis marmorata anomalies such as lipoma, hypertrichosis or dermal sinus,
Cutis marmorata is a frequent, benign and generalized among others, are also present [12,29].
cutaneous vasomotor phenomenon induced by hypother­
mia. The immature autonomic nervous system is respon­
Harlequin colour change
This transient and sudden, uncommon phenomenon
sible for an irregular distribution of superficial capillary
consists of sharply demarcated erythema on one half of
blood flow [6,8]. Although it is more common in preterm
the body with simultaneous blanching on the contralat­
infants, full‐term babies may also be affected. It is charac­
eral half. A line of demarcation along the midline is well‐
terized by a transient, blanchable, bluish‐red, reticulated
defined. The change of colour, which generally fades
mottling that in general lasts minutes, aggravated by cold
within 30 seconds to 20 minutes, appears between the
and abated by warm temperatures. Physiological cutis
second and fifth days after birth, but may occur up to the
marmorata improves spontaneously within the first few
third week [8,30,31]. Although harlequin colour change
weeks [4,6,8].
may arise as a single episode, it sometimes reappears. It is
Persistent or severe cutis marmorata has been reported
in several diseases including Down syndrome, trisomy
18, congenital hypothyroidism, homocystinuria, Divry–
Van Bogaert syndrome and Cornelia de Lange syndrome
[4,24,25].
Cutis marmorata telangiectatica congenita is a rela­
tively uncommon capillary malformation that can mimic
physiological cutis marmorata but does not disappear
with warming. It is characterized by deep purple reticular
or stellate areas of mottling, is present at or shortly after
birth, and may have a localized, segmental or generalized
distribution. Atrophy or skin ulceration and loss of
underlying subcutaneous fat, as well as prominent
veins, telangiectasias and hyperkeratosis may be seen.
Extracutaneous findings such as growth and develop­
mental delay, discrepancies in length and circumference
of limbs, glaucoma, macrocephaly, neurological abnor­
malities and other vascular anomalies have been reported
in 20–80% of patients. Cutis marmorata telangiectatica
congenita may be present in some disorders such as Fig. 6.4  Salmon patch: erythematous macules on the nose and philtrum.
 76 Section 2  Skin Disorders of the Neonate and Young Infant
most commonly observed when the baby is in a lateral
decubitus position. The colour characteristically changes
location when the baby is rotated [6,30]. Newborns with
harlequin colour change are in good general condition
with no other signs or symptoms associated. Although it
is observed in healthy newborns, it has also been reported
SECTION 2: SKIN DISORDERS
OF NEONATES AND INFANTS
in the context of hypoxia, such as prematurity or low
birthweight. Exacerbation of the phenomenon has been
reported in patients with congenital cyanotic cardiomyo­
pathy receiving prostaglandin E1 treatment [30,32]. Its
aetiopathogenesis is poorly understood, but it has been
suggested that hypothalamic immaturity produces a
transient imbalance in cutaneous vessel regulation [30,32].
Erythema neonatorum and acrocyanosis
Generalized erythema or rubor neonatorum is a physio­
logical condition present from the first hours until the sec­
ond day of life. Its mechanism is related to cutaneous
vasodilation and hyperaemia. Neonatal polycythaemia is
an associated factor that contributes to erythema [4,6].
Acrocyanosis is characterized by bilateral and symmet­
rical purple‐bluish colouration of hands and feet as well
as the perioral area. It is intermittent, may intensify with
vigorous crying, cold, polycythaemia or other hypervis­
cosity syndromes, and may revert with warmth. In gen­
eral it resolves within the first week of life [4,6,8]. Its
pathogenesis is not well understood, but it is proposed
that cutaneous vasomotor instability related to immatu­
rity is involved. Blood flow is reduced in dilated subpap­
illary and papillary venous vessels favouring oxygen
release in peripheral tissues and subsequent formation of
desaturated haemoglobin [4]. It should be distinguished
from Raynaud’s phenomenon and other disorders associ­
ated with central cyanosis such as congenital cardiac dis­
ease or respiratory diseases [33]. Since this condition is
benign and self‐healing, no treatment is required.
Transient vesicopustular eruptions
Erythema toxicum neonatorum
Erythema toxicum neonatorum is a benign and self‐
limited condition with no racial, gender or seasonal
predilection. Its incidence varies from 16.7% to 55% in
different reports [34,35]. Higher incidence rates are seen
in term neonates with birthweight over 2500 g. It is rarely
seen in preterm infants and its presence in this population
should prompt an evaluation for underlying infection.
Different hypotheses have been proposed to explain
the pathogenesis of erythema toxicum, including allergic
response to transplacental or environmental allergens;
response to mechanical, chemical or thermal irritation;
hormonal influences on the extracellular matrix; and a
graft‐versus‐host reaction triggered by maternal lympho­
cytes transferred to the infant before or during delivery
[3,34,36]. Currently it is accepted that pro‐inflammatory
mediators (IL‐1, IL‐8, eotaxin, aquaporins 1 and 3, psoria­
sin, nitric oxide synthases 1, 2 and 3) identified in the
erythema toxicum infiltrate reflect an immunological
cutaneous reaction to microbial skin colonization of hair
follicles from birth [37].
Fig. 6.5  Erythema toxicum neonatorum: erythematous macules and
pustules.
Although this rash may be present at birth, it usually
begins 2–3 days after birth. Typical lesions consist of
erythematous macules 2–3 cm in diameter with a central
papule or pustule. Lesions can vary in number from one
to several and be located on the face, trunk and proximal
extremities, without involvement of palms and soles
(Fig. 6.5). The lesions are highly evanescent and usually
evolve in crops that wax and wane, with spontaneous
resolution of individual lesions within hours to days.
Spontaneous involution of the eruption occurs within
1–2 weeks without sequelae, although lesions may recur
[3,34,36,38].
Diagnosis is made clinically. Cytological examination
of a pustular smear with Giemsa or Wright staining dem­
onstrates eosinophilia. Skin biopsy, if performed, shows a
dense inflammatory infiltrate around hair follicles com­
posed mostly of eosinophils and subcorneal pustules.
Peripheral eosinophilia may be present [34,37]. Differential
diagnosis includes transient neonatal pustular melanosis,
miliaria and eosinophilic pustular folliculitis, as well as
infections including congenital candidiasis, staphylococ­
cal impetigo and neonatal herpes simplex [3,34,36].
As erythema toxicum neonatorum is a self‐limiting
condition, no treatment is required. However, atypical
presentations often appropriately prompt further evalua­
tion to rule out an underlying infection.
Transient neonatal pustular melanosis
This benign, transient disorder is found in 0.2‐–4% of full‐
term neonates. It is more common in more dark‐skinned
infants, but there is no sex predilection. It is characterized
by fragile pustules or vesicles without surrounding ery­
thema that are almost always present at birth. Lesions
measure 1–5 mm in diameter, are single or grouped in
clusters, and may occur anywhere but are commonly seen
on the forehead, mandibular area, neck, trunk, buttocks,
thighs, palms and soles (Fig. 6.6). The vesicopustules are
very fragile and break down easily, leaving pigmented
macules surrounded by fine, white collarettes of scale.
Brownish macules may persist for months, but in general
fade spontaneously within a few weeks. In some cases,
 Chapter 6  Transient Skin Disorders in the Neonate and Young Infant 77

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

SECTION 2: SKIN DISORDERS

OF NEONATES AND INFANTS
palatal components during embryogenesis. No treatment
is indicated, as they regress spontaneously within the first
several weeks after birth. They are considered analogous
to milia [10,44,45].

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

Fig. 6.6  Transient neonatal pustular melanosis: grouped pustules without

surrounding erythema on the scalp.

the vesicular stage develops in utero and the newborn

presents with only hyperpigmented macules at birth
[3,36,39,40].
The differential diagnosis includes erythema toxicum
neonatorum, staphylococcal pustulosis, congenital can­
didiasis, syphilis, herpes simplex and acropustulosis of
infancy.
Giemsa or Wright staining of pustular content reveals
neutrophils and occasional eosinophils. No organism is
observed and cultures are negative. Skin biopsy of pustu­
lar lesions shows an intracorneal or subcorneal collection
of neutrophils; in contrast to classical erythema toxicum,
there are few eosinophils [3,7,36].
Based on similarities in clinical findings and the pres­
ence of both neutrophils and eosinophils in the skin
biopsy, as well as the coexistence of lesions of erythema
toxicum neonatorum and transient neonatal pustular
melanosis in the same patient, it has been postulated that Fig. 6.7  Epstein pearls: multiple small whitish palatal cysts.
these disorders may be variations of the same disease.
The term sterile transient neonatal pustulosis was pro­
posed by Ferrandiz et  al. to include these overlapping
conditions [41,42].

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
epithelium over the dental lamina [43,46]. Differential
diagnosis includes Epstein pearls, natal or neonatal teeth
and dental lamina cysts. Spontaneous rupture and sub­
sequent involution of the cysts within a few weeks is the
rule. No treatment is needed [43,45–47].
SECTION 2: SKIN DISORDERS
Dental lamina cysts
OF NEONATES AND INFANTS
Also known as gingival cysts of the newborn, they occur
on the crest of alveolar ridges, and derive from remnants
of the dental lamina. They may be single or multiple, and
are located mainly on the maxilla. Clinically, they are
small (1–3 mm), pearly, firm papules observed in 25–53%
of newborns [44,48]. These keratin‐filled cysts disappear
within the first weeks of life.
Natal and neonatal teeth
Normal eruption of primary teeth usually occurs around
6 months of age. Premature eruption of teeth is a rare
condition; depending on the time of eruption such teeth
are defined as natal teeth when present at birth, and neo­
natal teeth when they erupt during the first month. The
incidence of natal and neonatal teeth varies from 1 : 716
to 1 : 3500 live births, with no gender predilection [49,50].
Natal teeth are approximately three times more frequent
than neonatal teeth. Most natal and neonatal teeth repre­
sent early eruption of the normal primary deciduous den­
tition, whereas less than 10% are supernumerary teeth
[45,49,51]. The exact pathogenesis is not known, but it has
been suggested that premature eruption of primary teeth
may be related to superficial location of the developing
tooth germ in the alveolar bone [51]. It has also been
hypothesized that increased resorption of overlying bone
results in premature tooth eruption [49,52]. Autosomal
dominant inheritance has been reported in some cases,
as have several predisposing factors such as hormonal
disturbances (pituitary, thyroid), poor maternal health,
febrile episodes during pregnancy and environmental
factors (including polyhalogenated aromatic hydrocar­
bons) [49–52]. Some syndromes are associated with natal
and neonatal teeth (Table 6.1) [10,45,49,51,53]. Natal and
neonatal teeth have been reported in infants with con­
genital hypothyroidism and in the context of cleft lip
and palate [54,55].
Table 6.1  Syndromes associated with natal and neonatal teeth
Syndrome Inheritance
Pachyonychia congenita AD
Ellis–van Creveld syndrome AR
Hallermann–Streiff syndrome Isolated cases
Wiedemann–Rautenstrauch syndrome (neonatal AR
progeroid syndrome)
Short‐rib thoracic dysplasia 13 with or without AR
polydactyly
Restrictive dermopathy AR
Raine syndrome (osteosclerotic bone dysplasia) AR
Osteopathia striata with cranial sclerosis XLD
Epidermolysis bullosa, lethal acantholytic AR
AD, autosomal dominant; AR, autosomal recessive; XLD, X‐linked dominant.
Fig. 6.9  Partially erupting natal tooth and ulceration of the tongue (Riga–
Fede disease).
Clinically, natal and neonatal teeth are typically poorly
developed, loose and conical, with yellowish‐brown or
whitish opaque discolouration, and have hypoplastic
enamel. Occasionally they are normal in size, shape and
colour (Fig. 6.9). Natal and neonatal teeth may be classi­
fied as mature when they are nearly or fully developed
and immature when their structure is incomplete, leading
to a poor prognosis [49,50]. The most common location of
natal and neonatal teeth is mandibular central incisors
(85%), followed by maxillary incisors (11%), mandibular
canines or molars (3%) and maxillary canines or molars
(1%) [45,50,52].
Radiographic examination helps to differentiate between
supernumerary primary teeth and teeth of the normal
dentition, as well as Bohn nodules and dental lamina
cysts, the major differential diagnosis.
Extraction is the treatment of choice in the case of
supernumerary teeth as they may interfere with normal
tooth eruption. Excessively mobile teeth should be
extracted to prevent the risk of exfoliation and subse­
quent swallowing or aspiration [45,49]. A major compli­
cation is the development of a traumatic ulceration on
the ventral surface of the tongue, lips or mother’s breast,
which represents a sign of Riga–Fede disease and associ­
ated pain insensitivity. The lesion begins as an eroded
area, evolving into an enlarged granulomatous ulcerated
mass. Discomfort during breastfeeding with subsequent
refusal to eat, malnutrition and dehydration may be asso­
ciated. A conservative approach is the first treatment
option for Riga–Fede disease. Sharp tooth edges may be
polished using a finishing burr or covered with compos­
ite resin. In some cases tooth extraction may be required
[49,50,52,56].
Eruption cyst
Eruption cyst is characterized by circumscribed fluctuant
mucosal swelling overlying a tooth during the eruption
process. Given that eruption cysts are related to both pri­
mary and permanent tooth eruption, they are rarely
observed in the neonatal period, in which case they are
secondary to natal or neonatal teeth [45]. The eruption
 Chapter 6  Transient Skin Disorders in the Neonate and Young Infant
Fig. 6.10  Eruption cyst: bluish, dome‐shaped lesion on the alveolar ridge
of the mandible.
cyst appears as a translucent, flesh‐coloured or bluish,
dome‐shaped, compressible lesion on the alveolar ridge
of the mandible or maxilla (Fig.  6.10) [45,57,58]. The
differential diagnosis includes mucocoele, lymphatic
malformation, congenital epulis, dental lamina cysts,
teratoma and Bohn nodules [58]. Radiological examina­
tion shows a natal or neonatal tooth. Cystic content
obtained by needle aspiration has a yellowish colouration
and low viscosity and presents cholesterol crystals.
Although treatment options include marsupialization or
surgical removal, most eruption cysts resolve spontane­
ously within several weeks [45,58].
Sucking pads of the lips
Sucking pads or sucking calluses on the lips are present
in both term and preterm neonates, and are considered
a physiological adaptive reaction of lip structures to
sucking. They are characterized by painless, whitish and
hyperkeratotic swelling of the lips, predominantly in the
middle of the upper lip. Skin biopsy, if performed, shows
hyperkeratosis, epidermal hyperplasia and intracellular
oedema. They disappear spontaneously after 3–6 months,
when breastfeeding is stopped [45,59,60].
Pigmentary skin lesions
Mongolian spots
Mongolian spots (congenital dermal melanocytosis) are
congenital homogeneous greyish macules located on
sacral and gluteal areas, resulting from the aberrant pres­
ence in the lower dermis of melanocytes that failed to
migrate from the neural crest to the epidermis during
fetal life. The prevalence varies with race, affecting around
90–100% of Africans and Asians, 50% of Hispanics, and
10% of Caucasians [61,62].
Clinically Mongolian spots are characterized by single
or multiple grey, blue‐grey, blue‐green or dark blue mac­
ules of varying sizes and shapes, with irregular borders,
located mainly on the sacrogluteal area. Aberrant extrasa­
cral locations include the lower and upper extremities,
upper back and shoulders (Fig. 6.11). They typically fade
79
SECTION 2: SKIN DISORDERS
OF NEONATES AND INFANTS
Fig. 6.11  Mongolian spots: aberrant and superimposed.
in early to mid childhood. Superimposed darker spots on
classic Mongolian spots may be present [61–64].
Histopathology reveals the presence of scattered mel­
anocytes in the lower dermis associated with occasional
melanophages [62].
Rarely, Mongolian spots may be associated with lysoso­
mal storage diseases. In these cases Mongolian spots are
persistent, aberrant in appearance and extensive, affecting
not only the dorsum but also the ventral aspect of the
trunk, with darker and progressive pigmentation. An
extensive confetti appearance has also been observed in
some patients. The most common lysosomal storage
disease associated with Mongolian spots is GM1 gangli­
osidosis 1, followed by mucopolysaccharidosis type 1
(Hurler disease) and, to a lesser degree, mucopolysaccha­
ridosis type 2 (Hunter disease), mucolipidosis, Niemann–
Pick disease and mannosidosis [61,65–68]. Phakomatosis
pigmentovascularis consists of the association of a vascu­
lar malformation and a cutaneous melanocytic lesion.
Persis­tent Mongolian spots are part of phakomatosis
pigmentovascularis types II, IV and V, also known as
phakomatosis cesioflammea and phakomatosis cesio­
marmorata [61,62,65,69].
Pigmentary lines of newborns
This rare, transient, pigmentary dermatosis is character­
ized by horizontal linear hyperpigmentation on the skin
folds of the abdomen, back and extremities from birth.
Spontaneous resolution occurs between 2 and 6 months
of age. Most reported cases were darkly‐pigmented male
newborns. Pathogenesis is thought to be nonhormonal,
possibly induced by mechanical trauma related to flexion
in utero, which produces slight hyperkeratosis within the
skin folds resulting in postinflammatory hyperpigmenta­
tion [70–72].
Periungual hyperpigmentation
Hyperpigmentation of the distal phalanges and the proxi­
mal nail fold is an occasional clinical finding in dark‐
skinned newborns, although it has also been described
in Caucasian babies. Pigmentation is uniform in each
 80 Section 2  Skin Disorders of the Neonate and Young Infant

patient: light‐brown in fair‐skinned patients and intensely

hyperpigmented in dark‐skinned neonates. It occurs in
both fingers and toes, is present at birth in full‐term
neonates, and fades spontaneously within the second
year of life [73,74]. Its pathogenesis is unclear, although it
is speculated to be a result of melanocyte‐stimulating
SECTION 2: SKIN DISORDERS
OF NEONATES AND INFANTS

hormone in utero [71,73].

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
calcium should be evaluated periodically up to 6 months
of age. The pathogenesis of hypercalcaemia is not fully
understood. The mechanism involved includes increased
prostaglandin E2 activity leading to osteoclast activation,
direct calcium release from necrotic fat tissue and secre­
tion of 1,25‐dihydroxyvitamin D3 from granulomas
which produces increased intestinal calcium absorption
[76,78,83,85]. Infants with hypercalcaemia present with
irritability, lethargy, hypotonia, vomiting, polyuria, poly­
dipsia, dehydration, constipation and failure to thrive.
Persistent moderate‐to‐severe hypercalcaemia may result
in metastatic calcification of skin, myocardium, falx cer­
ebri, liver, gastric mucosa and kidney, leading to nephro­
calcinosis, nephrolithiasis and, rarely, renal failure [76,78].
Treatment of hypercalcaemia includes conservative
management with formulas that are low in calcium and
vitamin D, increased hydration, calcium‐wasting diuretics
and corticosteroids. In cases of refractory hypercalcaemia,
treatment with pamidronate has been reported as being
effective.
Treatment of subcutaneous fat necrosis in most cases is
unnecessary because it is a self‐limited condition. Severe
fluctuant abscess‐like nodules or plaques should be
aspirated to prevent rupture and infection, and to mini­
mize pain and skin sequelae. It is important to prevent
and manage complications [76,82]. Cooled neonates
should be turned regularly to prevent prolonged contact
with the cooling interface [82].
Sucking blisters, erosions and calluses
Sucking blisters and erosions are the result of repetitive
vigorous fetal sucking. They occur in 0.4–2% of healthy
newborns at birth [86,87]. They present as an intact flaccid
bulla or erosion on non‐inflamed skin of the dorsal aspect
of forearms, wrists and fingers (thumb and index) and
rarely on the feet. Although a solitary lesion is the most
common form of presentation, multiple and bilateral lesions
have also been reported. Calluses are the result of chronic
and less intense sucking [13,86,87]. They spontaneously
regress without sequelae within a few days to 2 weeks
after birth. The differential diagnosis with serious neonatal
blistering diseases such as bullous impetigo, herpes
simplex, congenital syphilis, mastocytosis or epidermolysis
bullosa should be effected [13,86,87]. Observation of focal
sucking of the area involved as well as failure to develop
new lesions postnatally aids diagnosis.
Adnexal polyp
Adnexal polyp of neonatal skin is a solitary, small, con­
genital polypoid tumour occurring most frequently on
the areola of the nipple (Fig. 6.14). It may also develop in
other sites such as eyelid, cheek, scapula, arm, axilla, labia
majora and scrotum. Although it usually falls off sponta­
neously within a few days, persistent lesions in a 53‐day‐
old and a 2‐year‐old infant have been reported. Its
incidence is estimated between 0.7 and 4%.
Histopathology demonstrates the presence of hair fol­
licles, eccrine glands and vestigial sebaceous glands in the
centre of the tumour [11,88–90].
81
SECTION 2: SKIN DISORDERS
OF NEONATES AND INFANTS
Fig. 6.14  Adnexal polyp: small pedunculated flesh‐coloured lesion.
Differential diagnostic considerations should include
skin tags, accessory auricles and supernumerary nipples.
Skin tags are multiple acquired tumours common in
adults but rare in infants, often located on axilla, groin
and neck. Histologically, adnexal structures are absent in
skin tags [88,90].
Pedal papules of infancy
Pedal papules, also known as precalcaneal congenital
fibrolipomatous hamartomas or benign anteromedial
plantar nodules, were first described by Larralde et al. in
1990 [91]. They are present at birth and consist of bilateral,
symmetrical, asymptomatic, subcutaneous flesh‐coloured
nodules located on the plantar region of the heel. As only
relatively few case series have been reported in the lit­
erature, the natural history of this entity is unclear.
However, one group reported an incidence rate of 5.9% in
newborns and 39.4% among infants, with the majority of
lesions appearing by 2–3 months of age and largely
showing spontaneous regression by 3 years of age [92].
The pathogenesis is not yet understood. Histopathological
study shows mature adipose tissue enveloped in pre­
dominantly collagenous fibrous sheaths. Differential
diagnostic considerations include other lipomas, naevus
lipomatosus and childhood fibrous hamartoma. They are
an entity distinct from piezogenic pedal papules, whose
onset is more frequently in adulthood [91–94].
Perineal groove
This rare and benign congenital anomaly of the perineum
occurs mostly in females, with only one male reported.
It is characterized by a wet groove that extends from the
posterior fourchette to the anus with a normal vestibule.
On rare occasions an anogenital or urogenital anomaly
may be associated. It is an asymptomatic lesion, with few
reported complications such as constipation or infection.
Given that complete epithelialization is achieved by 1–2
years of age, treatment is not generally necessary. Although
its pathogenesis remains unclear, some embryological
mechanisms have been proposed such as failure to fuse of
the midline perineal raphe [95,96].
 82 Section 2  Skin Disorders of the Neonate and Young Infant
References
1 Hulsmann AR, Oranje AP. Educational paper: Neonatal skin
lesions. Eur J Pediatr 2014;173:557–66.
2 Conlon JD, Drolet BA. Skin lesions in the neonate. Pediatr Clin North
Am 2004;51:863–88.
3 De Araujo T, Schachner L. Benign vesicopustular eruptions in the
neonate. An Bras Dermatol 2006;81:359–66.
4 Eichenfield L, Lee P. Transient physiologic changes and cutaneous
SECTION 2: SKIN DISORDERS
OF NEONATES AND INFANTS
lesions in the newborn. In: Schachner L, Hansen R (eds) Pediatric
Dermatology, 4th edn. Philadelphia: Mosby Elsevier, 2011:301–8.
5 Monteagudo B, Labandeira J, León‐Muiños E et al. Influence of neo­
natal and maternal factors on the prevalence of vernix caseosa. Actas
Dermosifiliogr 2011;102:726–9.
6 Serdaroglu S, Çak B. Physiologic skin findings of newborn. J Turk
Acad Dermatol 2008;2:83401r.
7 Ghosh S. Neonatal pustular dermatoses: An overview. Indian J
Dermatol 2015;60:211.
8 Afsar FS. Physiological skin conditions of preterm and term neo-
nates. Clin Exp Dermatol 2010;35:346–50.
9 Jha AK. Is There a preventive role for vernix caseosa?: An in vitro
study. J Clin Diagnostic Res 2015;9:13–16.
10 Larralde M, Luna PC. Neonatal transient benign cutaneous lesions:
An update. In: Sarkar R, Inamadar A, Palit A (eds) Advances in
Pediatric Dermatology‐2. New Delhi: Jaypee Brothers Medical
Pubishers, 2014:14–23.
11 Boralevi F, Taieb A. Common transient neonatal dermatoses. In:
Irvine A, Hoeger P, Yan A (eds) Harper`s Textbook of Pediatric
Dermatology, 3rd edn. Oxford: Blackwell Publishing, 2011.
12 Giachetti A. Cambios transitorios de la piel de recien nacido. In:
Larralde M, Abad E, Luna P (eds) Dermatologia Pediatrica. Buenos
Aires: Ediciones journal, 2010:13–17.
13 Larralde M, Luna PC. Benign self‐limiting cutaneous lesions. In:
Schachner L, Hansen R (eds) Pediatric Dermatology, 4th edn.
Philadelphia: Mosby Elsevier, 2011:311–13.
14 Haveri FTTS, Inamadar AC. A Cross‐Sectional Prospective Study of
Cutaneous Lesions in Newborn. ISRN Dermatol 2014. http://www.
hindawi.com/journals/isrn.dermatology/2014/360590/ (accessed 6
October 2018).
15 Kanada KN, Merin MR, Munden A, Friedlander S. A prospective
study of cutaneous findings in newborns in the United States: correla­
tion with race, ethnicity, and gestational status using updated classifi­
cation and nomenclature. J Pediatr 2012;161:240–5.
16 Monteagudo B, Labandeira J, Cabanillas M et al. Prevalence of milia
and palatal and gingival cysts in Spanish newborns. Pediatr Dermatol
2012;29:301–5.
17 Berk DR, Bayliss SJ. Milia: A review and classification. J Am Acad
Dermatol 2008;59:1050–63.
18 Luna PC, Larralde M. Profuse congenital familial milia with absent
dermatoglyphics (Basan’s syndrome): Description of a new family.
Pediatr Dermatol 2012;29:527–9.
19 Rutter KJ, Judge MR. Profuse congenital milia in a family. Pediatr
Dermatol 2009;26:62–4.
20 Adya KA, Inamadar AC, Palit A, Shirasangi A. “Miniature puberty”
in an eight‐days‐old female neonate. Indian J Pediatr 2014;81:1266–7.
21 Monteagudo B, Labandeira J, León‐Muiños E et  al. Estudio pro-
spectivo de la hiperpigmentación del área genital en recién nacidos
españoles: Frecuencia y factores predisponentes. Med Cutan Ibero
Lat Am 2013;41:3–6.
22 Ng M, How C. When babies turn yellow. Singapore Med J 2015;56:
599–603.
23 Cohen RS, Wong RJ, Stevenson DK. Understanding neonatal jaundice:
A perspective on causation. Pediatr Neonatol 2010;51:143–8.
24 Levy R, Lam JM. Cutis marmorata telangiectatica congenita: a mim­
icker of a common disorder. CMAJ 2011;183:249–51.
25 Mazereeuw‐Hautier J, Carel‐Caneppele S, Bonafé JL et al. Cutis mar­
morata telangiectatica congenita: Report of two persistent cases.
Pediatr Dermatol 2013;88:249–51.
26 Happle R. Capillary malformations: A classification using specific
names for specific skin disorders. J Eur Acad Dermatology Venereol
2015;29:2295–305.
27 Trevisan F, Cunha PR, Pinto CAL, Cattete FG. Cutaneous neonatal
lupus with cutis marmorata telangiectatica congenita‐like lesions. An
Bras Dermatol 2013;88:428–31.
28 Cordoro KM, Speetzen LS, Koerper MA, Frieden IJ. Physiologic
changes in vascular birthmarks during early infancy: Mechanisms
and clinical implications. J Am Acad Dermatol 2009;60:669–75.
29 Juern AM, Glick ZR, Drolet BA, Frieden IJ. Nevus simplex: A recon-
sideration of nomenclature, sites of involvement, and disease
associations. J Am Acad Dermatol 2010;63:805–14.
30 Januário G, Salgado M. The Harlequin phenomenon. J Eur Acad
Dermatology Venereol 2011;25:1381–4.
31 Tang J, Bergman J, Lam JM. Harlequin colour change: Unilateral
erythema in a newborn. CMAJ 2010;182:E801.
32 Rao J, Campbell ME, Krol A. The harlequin color change and associa­
tion with prostaglandin E1. Pediatr Dermatol 2004;21:573–6.
33 Sharathkumar AA, Castillo‐Caro P. Primary Raynaud’s phenomenon
in an infant: a case report and review of literature. Pediatr Rheumatol
Online J 2011;9:16.
34 Morgan AJ, Steen CJ, Schwartz RA, Janniger CK. Erythema toxicum
neonatorum revisited. Cutis 2009;83:13–16.
35 Monteagudo B, Labandeira J, Cabanillas M et al. Prospective study of
erythema toxicum neonatorum: Epidemiology and predisposing
factors. Pediatr Dermatol 2012;29:166–8.
36 Larralde M, Luna PC. Vesicopustular, bullous and erosive diseases
of the newborn. In: Schachner L, Hansen R (eds) Pediatric
Dermatology, 4th edn. Philadelphia: Mosby Elsevier, 2011:328–42.
37 Marchini G, Nelson A, Edner J et al. Erythema toxicum neonatorum
is an innate immune response to commensal microbes penetrated
into the skin of the newborn infant. Pediatr Res 2005;58:613–16.
38 Connor NR, McLaughlin MR, Ham P. Newborn skin: Part I. Common
rashes. Am Fam Physician 2008;77:47–52.
39 Agusti‐Mejias A, Messeguer F, Febrer I, Alegre V. Transient neonatal
pustular melanosis. Actas Dermosifiliogr 2013;104:84–5.
40 Chia PS, Leung C, Hsu YL, Lo CY. An infant with transient neonatal
pustular melanosis presenting as pustules. Pediatr Neonatol
2010;51:356–8.
41 Fujisawa Y, Miyazono Y, Kawachi Y, Otsuka F. A case of sterile tran­
sient neonatal pustulosis presenting with large flaccid pustules.
Pediatr Dermatol 2013;30:e238–9.
42 Ferrandiz C, Coroleu W, Ribera M et al. Sterile transient neonatal
pustulosis is a precocious form of erythema toxicum neonatorum.
Dermatology 1992;185:18–22.
43 Cambiaghi S, Gelmetti C. Bohn’s nodules. Int J Dermatol 2005;44:
753–4.
44 Singh RK, Kumar R, Pandey RK, Singh K. Dental lamina cysts in a
newborn infant. BMJ Case Rep 2012. http://www.ncbi.nlm.nih.gov/
pubmed/23048002 (accessed 6 October 2018).
45 Larralde M, Luna PC. Benign oral neonatal lesions. In: Schachner L,
Hansen R (eds) Pediatric Dermatology, 4th edn. Philadelphia:
Mosby Elsevier, 2011:308–11.
46 Dutta A, Gosh S, Nag S. Bohn’s nodules. Indian Pediatr 2014;51:
849–50.
47 Larralde M. Lesiones orales del recien nacido. In: Larralde M, Abad
E, Luna P (eds) Dermatologia Pediatrica. Buenos Aires: Ediciones
journal, 2010:17–20.
48 Kumar A, Grewal H, Verma M. Dental lamina cyst of newborn: A case
report. J Indian Soc Pedod Prev Dent 2008;26:175–6.
49 Mhaske S, Yuwanati MB, Mhaske A et al. Natal and Neonatal Teeth:
An Overview of the Literature. ISRN Pediatr 2013 http://www.ncbi.
nlm.nih.gov/pubmed/24024038 (accessed 6 October 2018).
50 Basavanthappa NN, Kagathur U, Basavanthappa RN, Suryaprakash
ST. Natal and neonatal teeth: A retrospective study of 15 cases. Eur J
Dent 2011;5:168–72.
51 Malki GA, Al‐Badawi EA, Dahlan MA. Natal teeth: A case report and
reappraisal. Case Rep Dent 2015. http://www.ncbi.nlm.nih.gov/
pubmed/25722895 (accessed 6 October 2018).
52 Volpato LER, Simões CAD, Simões F et al. Riga‐Fede disease associ-
ated with natal teeth: two different approaches in the same case.
Case Rep Dent 2015. http://www.ncbi.nlm.nih.gov/pubmed/26421196
(accessed 6 October 2018).
53 Shah S, Boen M, Kenner‐Bell B et al. Pachyonychia congenita in pedi­
atric patients. JAMA Dermatol 2014;150:146.
54 Venkatesh C, Adhisivam B. Natal teeth in an infant with congenital
hypothyroidism. Indian J Dent Res 2011;22:498.
55 Yilmaz RBN, Cakan D, Mesgarzadeh N. Prevalence and management
of natal/neonatal teeth in cleft lip and palate patients. Eur J Dent
2016;10:54.
56 Khandelwal V, Nayak UA, Nayak PA, Bafna Y. Management of an
infant having natal teeth. BMJ Case Rep 2013. http://www.ncbi.nlm.
nih.gov/pubmed/23737593 (accessed 6 October 2018).
57 Alemán Navas R, Martínez Mendoza M. Congenital eruption cyst.
Pediatr Dermatol 2010;27:671–2.
 Chapter 6  Transient Skin Disorders in the Neonate and Young Infant
58 Navas RMA, Mendoza MGM, Leonardo MR et al. Congenital erup­
tion cyst: A case report. Braz Dent J 2010;21:259–62.
59 Thariat J, Roth V, Marcy P‐Y. Sucking pads in a full‐term newborn.
J Pediatr 2011;158:166.
60 Heyl T, Raubenheimer J. Sucking pads (sucking calluses) of the lips in
neonates: a manifestation of transient leukoedema. Pediatr Dermatol
1987;4:123–9.
61 Gupta D, Thappa D. Mongolian spots: How important are they?
World J Clin Cases 2013;1:230–2.
62 Marcoux D, Durán‐McKinster C. Melanocytes and melanogenesis.
In: Schachner L, Hansen R (eds) Pediatric Dermatology, 4th edn.
Philadelphia: Mosby Elsevier, 2011:700–19.
63 Gupta D, Thappa DM. Mongolian spots  –  A prospective study.
Pediatr Dermatol 2013;30:683–8.
64 Musumeci M, Lacarrubba F, Santagati C, Micali G. Multiple and
superimposed Mongolian spots. BMJ Case Rep 2013. http://www.
ncbi.nlm.nih.gov/pubmed/24014338 (accessed 6 October 2018).
65 Ma H, Liao M, Qiu S et al. The case of a boy with nevus of Ota, exten­
sive Mongolian spot, nevus flammeus, nevus anemicus and cutis
marmorata. An Bras Dermatol 2015;90:S10–12.
66 Hanson M, Lupski JR, Hicks J, Metry D. Association of dermal
melanocytosis with lysosomal storage disease: clinical features and
hypotheses regarding pathogenesis. Arch Dermatol 2003;139:916–20.
67 Hackbart BA, Arita JH, Pinho RS et al. Mongolian spots are not always
a benign sign. J Pediatr 2013;162:1070.
68 Mimouni‐Bloch A, Finezilber Y, Rothschild M, Raas‐Rothschild A.
Extensive Mongolian Spots and Lysosomal Storage Diseases. J Pediatr
2016;170:333–333.e1
69 Happle R. Phacomatosis pigmentovascularis revisited and reclassi-
fied. Arch Dermatol 2005;141:385–8.
70 Fosse N, Itin P. Pigmentary lines of the newborn: a case report and
review of the literature. Dermatology 2014;228:198–201.
71 Martin JM, Jorda E, Alonso V. Transient pigmentary lines of the
newborn. Pediatr Dermatol 2009;26:768.
72 Garg G, Bhalla M, Thami G. Transient infantile patterned hyper-
pigmentation. Pediatr Dermatol 2012;29:372–3.
73 Iorizzo M, Oranje AP, Tosti A. Periungual hyperpigmentation in
newborns. Pediatr Dermatol 2008;25:25–7.
74 Perez‐Bescos L, Martinez‐Elgarresta M, de la Fuente‐de la Prieta M,
Iturralde‐Iriso J. Hiperpigmentación de las falanges distales del
lactante. An Pediatr 2006;65:384–92.
75 Larralde M, Abad ME, Corbella C et al. Subcutaneous fat necrosis of
the newborn, report of five cases. Dermatol Argent 2009;15:200–4.
76 Tran JT, Sheth AP. Complications of subcutaneous fat necrosis of
the newborn: A case report and review of the literature. Pediatr
Dermatol 2003;20:257–61.
77 Gomes M, Porro A, Enokihara M, Floriano M. Subcutaneous fat
necrosis of the newborn: clinical manifestations in two cases. An Bras
Dermatol 2013;88:154–7.
78 Rubin G, Spagnut G, Morandi F et al. Subcutaneous fat necrosis of the
newborn. Clin Case Rep 2015;3:1017–20.
83
79 Akin M, Akin L, Sarici D et  al. Follow‐up during early infancy of
newborns diagnosed with subcutaneous fat necrosis. J Clin Res
Pediatr Endocrinol 2011;3:216–18.
80 Strohm B, Hobson A, Brocklehurst P et al. Subcutaneous fat necrosis
after moderate therapeutic hypothermia in neonates. Pediatrics
2011;128:e450–2.
81 Grass B, Weibel L, Hagmann C, Brotschi B. Subcutaneous fat necrosis
in neonates with hypoxic ischaemic encephalopathy registered in
SECTION 2: SKIN DISORDERS
OF NEONATES AND INFANTS
the Swiss National Asphyxia and Cooling Register. BMC Pediatr
2015;15:73.
82 Oza V, Treat J, Cook N et al. Subcutaneous fat necrosis as a com-
plication of whole‐body cooling for birth asphyxia. Arch Dermatol
2010;146:882–5.
83 Samedi VM, Yusuf K, Yee W et al. Neonatal hypercalcemia second­
ary to subcutaneous fat necrosis successfully treated with
pamidronate: A case series and literature review. Am J Perinatol
Rep 2014;4:e93–6.
84 Shumer D, Thaker V, Taylor G, Wassner A. Severe hypercalcemia
due to subcutaneous fat necrosis: presentation, management
and  complications. Arch Dis Child Fetal Neonatal Ed 2014;99:
419–21.
85 Farooque A, Moss C, Zehnder D et al. Expression of 25‐hydroxyvita­
min D3‐1α‐hydroxylase in subcutaneous fat necrosis. Br J Dermatol
2009;160:423–5.
86 Monteagudo B, Labandeira J, León‐Muiños E et al. Neonatal suck-
ing blisters: prevalence and differential diagnosis. An Pediatr
(Barc) 2011;74:62–4.
87 Monteagudo B, León‐Muiños E. Neonatal sucking blisters. Indian
Pediatr 2010;47:794.
88 Hidano A, Kobayashi T. Adnexal polyp of neonatal skin. Br J Dermatol
1975;92:659–62.
89 Koizumi H, Itoh E, Ohkawara A. Adnexal polyp of neonatal skin
observed beyond the neonatal period. Acta Derm Venereol
1998;78:391–2.
90 Lucas Costa A, Betlloch I, Pérez‐Crespo M et al. Mammary skin tag
in a 2‐year‐old girl: a long‐lasting adnexal polyp of neonatal skin?
Pediatr Dermatol 2009;26:618–20.
91 Larralde de Luna M, Ruiz León J, Cabrera H. Pápulas podálicas en
el recién nacido. Med Cutan Ibero Lat Am 1990;18:9–12.
92 Greenberg S, Krafchik BR. Infantile pedal papules. J Am Acad
Dermatol 2005;53:333–4.
93 Rubio‐Flores C, López‐Barrantes González O, Garrido‐Gutiérrez C,
Díaz‐Díaz R. Precalcaneal congenital fibrolipomatous hamartoma.
Actas Dermosifiliogr 2012;103:649–51.
94 Ortega‐Monzó C, Molina‐Gallardo I, Monteagudo‐Castro C et  al.
Precalcaneal congenital fibrolipomatous hamartoma: a report of four
cases. Pediatr Dermatol 2000;17:429–31.
95 Diaz L, Levy M, Kalajian A, Metry D. Perineal groove: a report of 2
cases. JAMA Dermatol 2014;150: 101–2.
96 Harsono M, Pourcyrous M. Perineal groove: a rare congenital midline
defect of perineum. Am J Perinatol Rep 2016;6:e30–2.