The neonatal period is defined as the first 30 days of life. Neonatal skin diseases evolve much more rapidly than adult skin diseases, and some conditions that initially appear to be serious turn out to be trivial, whereas in others, the opposite is true. Infancy is defined as beginning after the first 30 days of life.
After birth, the neonate's skin undergoes a series of changes in adaptation to the extrauterine environment. In utero, the skin of the fetus is protected by the vernix caseosa and is immersed in amniotic fluid. After birth, the vernix is wiped off, and the skin is exposed and adapts to the dry ambient air. For example, desquamation of the upper layers of the stratum corneum occurs normally in all infants and is believed to be a normal adaptive process.
Research regarding the maturation of the neonatal stratum corneum in neonates has produced varying results, and the question of when full barrier function is achieved is not fully answered. Barrier stabilization appears to be a dynamic process, one dependent upon a balance between different biologic and environmental parameters. Postnatal life is believed to accelerate stratum corneum maturation in premature and term infants. Parameters such as skin thickness, skin pH, and stratum corneum hydration indicate that neonatal skin is continuously adjusting to the extrauterine environment, in contrast to the adult skin, which remains in a steady state.2
In vivo studies of human skin show that infant stratum corneum and epidermis is thinner than adult skin and has higher transepidermal water loss (TEWL) rates, but infant stratum corneum has higher water content. Infant corneocytes and granular cells are smaller than adult corneocytes suggesting a more rapid cell turnover than in adults.3
Infants have an increased risk for systemic toxicity from topically applied substances. This is due in large part to the great surface area–body mass ratio in the infant. In addition, the infant's metabolism, excretion, distribution, and protein binding of substances can be significantly different from those of an adult and add to increased risk of toxicity.4
The postmature infant (>40 weeks’ gestation) typically has dry and cracked or peeling skin noted soon after birth (Fig. 107-1). Shedding of the dry peeling skin of postdates infants occurs spontaneously in the first month of life, leaving normal, healthy skin. Topical care should include moisturizers and avoidance of overbathing.
The feet of a postmature newborn. The dry, hyperlinear, and scaly skin is typical of a postdates baby. There are also pustules of transient neonatal pustular melanosis.
Premature infants, particularly those born before 34 weeks of gestation, have markedly decreased epidermal barrier function and an even greater surface area–body mass ratio than term infants. In addition, the immature organs of the premature infant may affect the metabolism, excretion, distribution, and protein binding of chemical agents. Local or systemic toxicity can occur in the premature infant not only from topical medications, but also soaps, lotions, or other cleansing solutions.4,5
Increased skin fragility is a hallmark of prematurity (gestational age less than 37 weeks). Epidermal and dermal injury may lead to significant cutaneous pain even with routine handling and nursing care. The premature infant is at risk for infection and sepsis from skin-associated organisms entering through breaks in the thin and fragile skin and via iatrogenic portals of entry. Sweating in the premature infant is functionally reduced and contributes to poor thermal regulation. Heat regulation is dysfunctional due to a thin subcutaneous fat layer for insulation, poor autonomic control of cutaneous vessels, and a large surface–body ratio. In the nursery, the premature infant is usually placed in a temperature and humidity-controlled isolette until the infant matures and temperature and fluid regulation stabilizes.
In the 1990s researchers reported application of petrolatum-based emollient therapy to be beneficial in hospitalized preterm infants, decreasing transepidermal water loss.6 Subsequently, various emollients and regimens have been tested in infants of variable prematurity and birthweight. Improved skin integrity consistently improved in these studies, however, a threefold increase in the incidence of systemic candidiasis was reported after emollient therapy was implemented in extremely low birthweight (≤1,000 g) premature infants in one neonatal intensive care unit.7 Another outbreak of systemic candidiasis occurred in very low birthweight neonates (≤1,500 g) in a different neonatal intensive care unit.8 A 2004 Cochrane review concluded that prophylactic application of topical ointments increased the risk for nosocomial infection and advised against their routine use in preterm infants. In contrast, randomized, controlled studies in impoverished Bangledeshi preterm neonates have demonstrated decreased mortality rates when sunflower seed oil or Aquaphor ointment was applied by massage, compared to premature infants not receiving massage or emollients.9 Until prospective, controlled trials are performed, neonates receiving petrolatum-based emollient therapy should be carefully monitored for infections, particularly those infants with birthweights less than 1,500 g.
Once at home, bathing once or twice a week in plain water is sufficient for most infants; if bathing is more frequent, moisturization with unscented, simple emollients is recommended. The face, hands, and diaper area may be cleansed daily using a small amount of a mild, unscented, pH-neutral cleanser. Well-meaning parents often bathe their infants too frequently and use a multitude of products on their infant's skin. In addition to irritation and asteatosis, these practices may increase the risk of allergic contact dermatitis in infants. It has been estimated that the average newborn is exposed to approximately 10 skin care products in the first month of life, leading to exposure to more than 50 different chemicals ranging from mildly toxic to toxic.10 Parents should be taught that “less is best.”11
A complete history includes gestational and birth history as well as family history. Exposures during pregnancy, including medications, illicit drugs, and infectious diseases such as varicella and sexually transmitted diseases, should be reviewed. Obstetric data, including placental appearance and cultures, can be invaluable. In examining an infant, the most important element is thoroughness. Whether the infant is examined in the lap of the parent or on the examination table, all surfaces, including the creases and valleys of body folds and the diaper region (including the genitalia), deserve close examination. A vascular stain, vasoconstricted macule, or erosion can be the presenting sign of a hemangioma.12 A stray hair may later strangulate an appendage and should be removed. Infants with digital tourniquet (pseudoainhum) and clitoral tourniquet have been described.13,14 Congenital lesions of all classifications (e.g., pigmented, vascular, aplasias) warrant closer inspection to rule out associated findings. Midline lesions on the face, scalp, or spine may have central nervous system (CNS) connections and should not be biopsied without proper evaluation (see Table 107.7). The diaper area has its own unique set of problems and deserves examination at every visit.
Table 107-7 Biopsy Pitfalls in Pediatric Dermatology ||Download (.pdf)
Table 107-7 Biopsy Pitfalls in Pediatric Dermatology
Lesion and Site
Erosion or vesicle on the scalp
Aplasia cutis congenita Differential diagnosis: herpes, fetal scalp electrode trauma
Possible intracranial connection, risk of meningitis with biopsy or scraping
Protect site; do not scrape lesion or biopsy, consider ultrasound or MRI of head if a typical
“Hair collar” sign surrounding lesion on scalp or midline lesion
Encephalocele, spina bifida occulta, meningomyelocele
Possible intracranial connection; risk of meningitis
Preoperative imaging; consider neurosurgical consultation
Tuft of hair over midline spine
Spina bifida occulta, meningomyelocele
Possible intracranial connection; risk of meningitis
Preoperative imaging; consider neurosurgical consultation
Risk of chondritis if removed by shave or snip excision or if ligated with suture37
Appropriate closure when excised, if cartilaginous component present
Infant or child
Mass along midline scalp, glabella, side of forehead, or other embryonic fusion plane
Intracranial connection in up to 25% for midline dermoids; risk is higher if sinus present38; dermoids near the lateral eyebrows rarely have intracranial connections39
Consider MRI and neurosurgical consultation
Infant, young child
Nasal midline mass
Nasal glioma, encephalocele, or other dysraphic state Differential diagnosis: “hypertelorism,” hemangioma
Intracranial connection in 100% of encephaloceles; gliomas may extend into oropharynx or have intranasal connections38
Consider MRI and neurosurgical consultation
Infant, young child
Vascular mass with greatly increased warmth, often with pulsation or bruit
Arteriovenous malformation Differential diagnosis: hemangioma
Uncontrolled bleeding, problematic bony or soft tissue hypertrophy
Consider Doppler studies, MRI, and surgical consultation
The infant is vulnerable and completely dependent on the caretakers. The social support system and family structure must be considered when implementing a medical plan. There are times when the parents’ desire for treatment may not be in the best interest of the patient.
Medical and surgical decision making for the infant is based primarily on function rather than cosmesis. For example, extraction of a natal tooth is indicated if breast-feeding is impaired; whether or not the tooth is a component of a genetic syndrome is a separate issue.
Diseases of Neonates and Infants
Transient Dermatoses of the Neonate
Skin conditions encountered in newborns that tend to resolve by 30 days of age are considered to be transient. They are very common and many are expected in newborns.
Caput Succedaneum and Cephalohematoma
Caput succedaneum is subcutaneous edema over the presenting part of the head and is a common occurrence in newborns. Cephalohematoma is a subperiosteal collection of blood and is less common. Both lesions are due to shearing forces on the scalp skin and skull during labor.
Caput succedaneum is soft to palpation, and borders are ill-defined. Cephalohematoma is bounded by the suture lines of the skull and often feels fluctuant. If purpura is extensive, it can lead to hyperbilirubinemia. Congenital lymphedema or lymphatic malformations (such as in Turner syndrome) can mimic caput succedaneum. Both caput succedaneum and cephalohematoma resolve spontaneously; however, caput usually fades in 7–10 days, whereas cephalohematoma slowly resolves over several weeks.
Milia are multiple pinpoint- to 1-mm papules representing benign, superficial keratin cysts. They are seen most commonly on the nose of infants and may be present in the oral cavity as well, where they are called Epstein's pearls. They are expected findings in the newborn and resolve spontaneously within a few weeks of life.
Sebaceous Gland Hyperplasia
At least 50% of normal newborns have sebaceous gland hyperplasia (Fig. 107-2). Tiny (<1-mm) yellow macules or papules are seen at the opening of each pilosebaceous follicle over the nose and cheeks of term newborns. It is a benign condition that clears spontaneously by 4–6 months of age.
Sebaceous hyperplasia on the nose of a 1-day-old infant.
Erythema Toxicum Neonatorum
Erythema toxicum neonatorum (ETN) is an idiopathic, common condition seen in up to 75% of term newborns. It is rarely seen in premature infants. Blotchy erythematous macules 1–3 cm in diameter with a 1–4-mm central vesicle or pustule are seen in ETN (Fig. 107-3). They usually begin at 24–48 hours of age, but delayed eruption as late as 10 days of age has been documented.15 These follicular-based lesions can be located anywhere but tend to spare the palms and soles. A smear of the central vesicle or pustule contents will reveal numerous eosinophils on Wright-stained preparations. A peripheral blood eosinophilia of up to 20% may be associated, particularly in infants, with numerous lesions. Transient neonatal pustular melanosis (TNPM) lesions conrail neutrophils rather than eosinophils, and individual lesions heal with residual pigmentation, which is not seen in ETN. Bacterial infections, Pityrosporum folliculitis, and congenital candidiasis also may mimic ETN. Bacterial and fungal culture of lesions and Gram staining will help differentiate among these entities. ETN is benign and clears spontaneously by 2–3 weeks of age without residua.
Erythema toxicum neonatorum. Erythematous macules, some with a tiny central papule or pustule, on the arm of a 1-day-old newborn.
Transient Neonatal Pustular Melanosis
TNPM is an idiopathic pustular eruption of the newborn that heals with tiny brown-pigmented macules (Fig. 107-4). It is less common than ETN and is more prevalent among newborns with darkly pigmented skin. Lesions are usually present at birth or shortly thereafter, but may appear as late as 3 weeks of age, as superficial vesicles and pustules, with ruptured lesions evident as collarettes of scale. Pigmented macules are also often present at birth or develop at the sites of resolving pustules or vesicles within hours or during the first day of life; occasionally babies are born with the tiny melanocytic macules, suggesting in utero vesiculation. Lesions can occur anywhere but are common on the forehead and mandibular area. The palms and soles may be involved. Smear of the vesicle or pustule contents will reveal a predominance of neutrophils with occasional eosinophils on Wright-stained preparations.16
Transient neonatal pustular melanosis. A. A newborn with congenital, thin-walled pustules that rupture easily. B. Hyperpigmented macules appeared by 10 hours of age.
Miliaria rubra is frequently confused with ETN and TNPM. The erythema around miliaria rubra is small in area (1–2 mm versus 20–30 mm in ETN). The central pustule of TNPM may mimic congenital candidiasis, Pityrosporum folliculitis, or bacterial folliculitis lesions. Herpes simplex should be considered if lesions are vesicular. A Gram-stained slide of the pustules of ETN or TNPM will not show organisms. A Wright-stained slide usually will show a predominance of neutrophils. TNPM is a harmless condition that requires no treatment. The pustules usually disappear within 5–7 days of age, leaving residual pigmented macules that resolve over 3 weeks to 3 months.
Mottling is a blotchy or lace-like pattern of dusky erythema over the extremities and trunk of neonates that occurs with exposure to cold air. Virtually all babies demonstrate mottling at some time during the newborn period due to immaturity of the autonomic control of the cutaneous vascular plexus. This physiologic mottling disappears on rewarming, differentiating it from cutis marmorata telangiectatica congenita and livedo reticularis. Normal mottling resolves spontaneously by 6 months of age.
Harlequin color change is a rare vascular phenomenon occurring in low-birthweight infants. When the infant is placed on one side, an erythematous flush with a sharp demarcation at the midline develops on the dependent side, and the upper half of the body becomes pale. The color change usually subsides within a few seconds of placing the baby in the supine position but may persist for as long as 20 minutes. The exact mechanism of this unusual phenomenon is not known, but it may be due to immaturity of autonomic vasomotor control. Harlequin color change is seldom seen after 10 days of age.
Sucking blisters may be present at birth as the result of intrauterine sucking, but are more commonly seen during the first weeks of life. Sucking blisters are usually solitary, intact oval or linear blisters, erosions, or drying crusts, arising on noninflamed skin of the dorsal-radial aspect of forearms, wrists, or fingers or on the upper lip. They resolve within a few days. If the affected extremity is brought up to the infant's mouth, the infant will often commence sucking at the site, confirming the diagnosis. Herpesvirus infection is often considered when sucking blisters are encountered, but lesions of Herpes simplex infection are grouped vesicles occurring on an erythematous base or punched out, hemorrhagic erosions.
Benign Cephalic Pustulosis
Neonatal acneiform facial lesions usually develop within the first 30 days of life and are estimated to occur in 50% of newborns (Fig. 107-5). This benign eruption appears to be hormonally mediated (see Chapter 80) and has been attributed to overgrowth of Malassezia sp., and termed “benign neonatal cephalic pustulosis.”17 Most cases resolve spontaneously, but the eruption can be treated topically with ketoconazole, benzoyl peroxide, or erythromycin. True neonatal acne is probably much less common than benign cephalic pustulosis and can be distinguished by the presence of comedonal lesions. Similarly, infantile acne usually shows true comedones, sometimes with papules, pustules, and even cysts. An example of infantile acne is seen in Figure 107-6.
Benign cephalic pustulosis. Tiny papulopustules on the cheeks of a 3-week-old infant.
Infantile acne. True comedones and inflammatory papules are noted on the cheeks of a healthy 10-month-old girl.
Hair Loss in the Infant18
Telogen effluvium occurs frequently in newborns and is often overlooked. The hair loss may be gradual or sudden, and may occur as soon as the first few days of life, with the telogen hairs shed by 3–4 months of age. No treatment is indicated as spontaneous resolution is the rule.
A transient circumscribed patch of nonscarring alopecia develops at the occiput in many infants. Thought to be due to a combination of physiologic telogen effluvium and localized pressure from lying in the supine position, occipital alopecia spontaneously resolves.
Triangular Temporal Alopecia
Triangular temporal alopecia is a form of nonscarring hair loss noted at 2–5 years of age as a triangular-, oval-, or lancet-shaped area of alopecia at the frontotemporal scalp. Often, a thin row of hair separates the affected area from the forehead. The terminal hairs are replaced by vellus hair. The condition is often mistaken for alopecia areata; however, distinguishing features include the typical location and shape, the presence of vellus hairs, and the absence of exclamation point hairs or histologic findings of alopecia areata. There is no known treatment, and the condition persists unchanged. However, triangular temporal alopecia is benign and will not expand.
Alopecia areata occurs in all ages (see Chapter 88). All forms of alopecia areata occur in infants and children (patchy, universalis, etc.), with the same disease presentation and treatment challenges as in adults. Onset at younger than 2 years of age is estimated to occur in 1%–2% of alopecia areata patients; however, it may be under-recognized and more common. Several cases of congenital alopecia areata have been documented. Early onset is considered to be a poor prognostic marker. Total alopecia during the first year of life after having hair at birth should be distinguished from genetic disorders, such as congenital atrichia with papular lesions and vitamin D resistance.
Tinea capitis can occur at any age, including infancy (see Chapter 188). Hair loss associated with scaling, broken hairs, pustules, or black dots should prompt a potassium hydroxide scraping and fungal culture to confirm the diagnosis. Just as in older children, Trichophyton tonsurans is the most common dermatophyte, and oral griseofulvin is the treatment of choice. Tinea capitis and fungal infections are discussed in Chapter 188.
Birthmarks represent an excess of one or more of the normal components of skin per unit area: blood vessels, lymph vessels, pigment cells, hair follicles, sebaceous glands, epidermis, smooth muscle, collagen, or elastin. Although most birthmarks are of little medical or psychosocial consequence, the social and cultural impact of a disfiguring birthmark should not be underestimated, from both the patient's and the parents’ perspectives.19 The age-old theory of maternal imprinting is still widely accepted in many countries, including the United States, and the mother may be subtly or actively blamed for congenital conditions in the newborn. With selective photothermolysis offered by lasers and advances in surgical and topical therapies, therapeutic options are increasing.
The two most common birthmarks are the nevus simplex (see Chapter 172) and Mongolian spots (see Chapter 122). Nevus simplex (also known as salmon patch, nevus flammeus, angel's kiss, or stork bite) represents a capillary malformation of the skin. It occurs most commonly on the glabella, upper eyelids, and nuchal area. Nevus simplex appears with high frequency in all races, occurring in 70% of white infants and 59% of black infants. Mongolian spots, which represent collections of dermal melanocytes, are seen in 80%–90% of infants of color but in only 5% of white infants.20,21 Solitary café-au-lait macules are extremely common and benign, however, the appearance of multiple café-au-lait macules raises the possibility of neurofibromatosis type 1 (see Chapter 141). Other common birthmarks are listed in Table 107-2.
Table 107-2 Common Birthmarks in the Newborn ||Download (.pdf)
Table 107-2 Common Birthmarks in the Newborn
- Mongolian spots
- Nevus simplex (nevus flammeus, salmon patch, “stork bite,” “angel's kiss”)
- Port-wine stain
- Hemangioma of infancy (infantile hemangioma)
- Epidermal nevus, including nevus sebaceus
- Congenital nevocellular nevi
- Congenital melanocytic nevi
- Nevus depigmentosus
- Café-au-lait spots
Infantile hemangiomas are the most common tumors of infancy. They must be differentiated from vascular malformations and other vascular anomalies. Hemangiomas are discussed in detail in Chapter 126.
Both types of lymphatic malformations, microcystic (lymphangiomas) and macrocystic (cystic hygromas), are discussed in Chapter 172.
Uncommon Dermatoses of the Neonate
Selected dermatoses of the neonate are discussed in the following sections. Table 107-3 lists differential diagnoses for selected cutaneous conditions encountered in neonates and infants.
Table 107-3 Selected Differential Diagnosis of Neonates and Infants ||Download (.pdf)
Table 107-3 Selected Differential Diagnosis of Neonates and Infants
- Erythema toxicum neonatorum
- Transient neonatal pustular melanosis
- Congenital candidiasis
- Pustular psoriasis
- Langerhans cell histiocytosis
- Neonatal cephalic pustulosis
- Bacterial sepsis
- Herpes simplex infection
- Sucking blisters
- Herpes simplex virus
- Aplasia cutis congenital
- Cutaneous mastocytosis
- Epidermolysis bullosa
- Neonatal pemphigus
- Incontinentia pigmenti
- Epidermolytic ichthyosis
- The red scaly baby
- Physiologic scaling and redness (postdates) infant
- Atopic dermatitis
- Seborrheic dermatitis
- Hypohidrotic ectodermal dysplasia
- Netherton syndrome
- Acrodermatitis enteropathica
- The collodion baby
- Lamellar ichthyosis
- Congenital ichthyosiform erythroderma
- Gaucher syndrome
- X-linked ichthyosis
- Epidermolytic ichthyosis
- The “blueberry muffin” baby
- TORCH infections
- Congenital leukemia
- Congenital self-healing reticulohistiocytosis
- Blue rubber bleb nevus syndrome
- Twin-twin transfusion
Cutis Marmorata Telangiectatica Congenita
Cutis marmorata telangiectatica congenita is characterized by persistent coarse cutis marmorata, telangiectasia, and sometimes associated underlying cutaneous atrophy and ulceration (Fig. 107-7). Its incidence is sporadic, and its etiology is obscure. Theories of vascular malformation are currently favored. Diagnosis is usually evident on clinical examination. Usually a lower extremity is involved, but location on the trunk or upper extremity is not uncommon. A multitude of associated anomalies can occur, including limb asymmetry, hemangiomas, other vascular birthmarks, pigmented nevi, and aplasia cutis congenita (ACC). However, the majority of patients have a good prognosis, with half demonstrating improvement of the mottled appearance over the first 2 years.22
Cutis marmorata telangiectatica congenita. Note the atrophic, dusky, stellate patches with overlying telangiectasias.
Subcutaneous Fat Necrosis of the Newborn
Subcutaneous fat necrosis of the newborn is characterized by firm, circumscribed, reddish or purple subcutaneous nodules or plaques that appear over the back, cheeks, buttocks, arms, and thighs (Fig. 107-8; see Chapter 70). The lesions usually begin within the first 2 weeks of life and resolve spontaneously over several weeks.23
Subcutaneous fat necrosis of the newborn. This infant developed an erythematous firm mass on the back by 2 weeks of age. She later developed hypercalcemia.
Sclerema is diffuse hardening of the skin in a sick premature newborn that is now rare because of improved neonatal care. The onset is characteristically after 24 hours of age. The skin feels hard and immobile and looks yellow and shiny. The trunk is always involved. Severely ill premature newborns that have suffered sepsis, hypoglycemia, metabolic acidosis, or other severe metabolic abnormalities are at risk. Biopsy sections show edema of fibrous septa surrounding fat lobules, but no fat necrosis, differentiating it from subcutaneous fat necrosis of the newborn. The etiology of this rare condition is unclear, and infant mortality is high.
Aplasia Cutis Congenita (ACC)
ACC represents a failure of skin to fully develop, most often on the scalp, and less commonly elsewhere (Fig. 107-9). It is often an isolated finding, but a multitude of associated conditions have been described. ACC has no single underlying cause. Some cases may represent a forme fruste of a neural tube defect.24 In the most common form of ACC, oval, sharply marginated atrophic macules are seen on the midline of the posterior scalp. They are usually solitary, but may be multiple. Aplasia cutis is always hairless, and may appear vesicular, ulcerated, or covered by a thin epithelial membrane. When healed, lesions are usually atrophic scars, but sometimes develop a keloidal scar. Epidermis, dermis, and fat all may be missing, or a single layer may be absent. Lesions may range from a few millimeters to many centimeters in diameter. Lesions at birth of epidermolysis bullosa (see Chapter 62) may resemble ACC, especially on one or both legs. Scalp ulcers at birth of aplasia cutis may be mistaken for obstetric trauma. Other forms of congenital circumscribed hair loss should be considered. Midline blisters or erosions should not be biopsied, scraped for herpes cultures, or otherwise traumatized. After a careful examination to rule out associated malformations, ACC is treated conservatively to allow healing. Surgical revision of the scar later in childhood or adolescence can be done electively to improve cosmesis.
Two infants with aplasia cutis congenita. A. Scalp erosions grouped at the vertex scalp. Scraping or biopsy is contraindicated. B. An atrophic, well-circumscribed round patch with visible capillaries on the scalp of an infant. (A used with permission from 1991 Yale Resident Photograph Collection.)
The “hair collar sign” is a ring of darker and/or coarser terminal hairs on the scalp, typically surrounding ACC, dermoid cyst, encephalocele, meningocele, or heterotopic brain tissue.25 The hair collar sign itself is a marker of cranial dysraphism and its presence, like aplasia cutis, mandates careful examination of the infant, particularly of midline structures and fusion planes.
Anetoderma of Prematurity
A specific form of iatrogenic anetoderma (see Chapter 67) has been described in extremely premature infants (born at 24–30 weeks’ gestation) with very low birthweight and prolonged neonatal intensive care hospitalization.26 A retrospective review of 11 cases noted the appearance of round, flat atrophic patches on the chest and abdomen (including the periumbilical region), developing at age 6 weeks to 5 months. Eight patients had lesions at sites where adhesive monitoring leads had been removed, and five patients had circular ecchymotic patches from removal of adhesive monitoring leads prior to the occurrence of the anetoderma. The anetoderma did not improve with time. One child underwent surgical excision of the disfiguring lesions at age 7 years. Given the presumed relationship of the development of anetoderma and skin trauma mainly from adhesive leads, avoidance of pressure (e.g., placing leads on the ventral chest when the infant slept on the back) markedly reduced the risk of occurrence.27
Infections of the Neonate
Infants younger than the age of 2 months are less able to localize infection due to immature immunologic function, and life-threatening sepsis can develop insidiously. Subtle clues such as a decrease in body temperature, poor feeding, or other nonspecific signs are taken seriously by the pediatrician, and a “rule out sepsis” admission is implemented when suspicions are high. At a minimum, blood, cerebrospinal fluid, and urine cultures are obtained, and intravenous antibiotic therapy begun pending cultures.
Congenital Viral Infection
Petechiae, purpura, jaundice, hepatomegaly, splenomegaly, microcephaly, encephalopathy, ocular abnormalities, anemia, thrombocytopenia, conjugated hyperbilirubinemia, or elevated serum hepatic transaminases should prompt the consideration of congenital viral infection, particularly if these signs arise in combination. Cutaneous infections of the neonate are listed in Table 107-4. Bacterial infections, such as staphylococcal scalded skin syndrome; and viral infections, such as varicella, are discussed in Chapters 193 and 194.
Table 107-4 Selected Cutaneous Infections in the Neonate ||Download (.pdf)
Table 107-4 Selected Cutaneous Infections in the Neonate
- Staphylococcal infections
- Staphylococcal scalded-skin syndrome
- Breast abscess (usually due to Staphylococcus aureus and Gram-negative organisms)
- Viral infections
- Herpes simplex virus
- Fungal/candidal infections
Neonatal Herpes Simplex Virus Infection
(See Chapter 193.) It is estimated that untreated neonatal herpes simplex virus (HSV) has a 50% mortality rate, with three-fourths of survivors suffering neurologic sequelae. The greatest risk of neonatal herpes occurs when the delivery is vaginal and the mother has primary genital herpes (as opposed to recurrent herpes, in which the mother has antibodies protective to the neonate), the herpes infection involves the cervix, and the infant is premature and delivered with instrumentation (e.g., scalp electrodes).
The vast majority of cases are due to HSV type 2. The typical lesions of herpes are present in the skin, eye, or mouth in a large majority of infected neonates, but some patients with CNS or disseminated disease never have skin lesions (Fig. 107-10).28 Vesicles present during the first 24 hours of life suggest in utero acquisition of HSV, but onset during the first week to 10 days of life is more common, representing exposure to the virus during the delivery.28
A and B. Herpes simplex infection in two infants. Grouped vesicles on an erythematous base. Pustules and erosions are also present. (A used with permission from Alvin H. Jacobs, MD.)
A high index of suspicion should be maintained even in the absence of maternal infection or history of genital herpes. Specimens for Tzanck smear, direct fluorescent antibody assays, and/or viral cultures for HSV should be obtained. Cultures require 12–120 hours to grow, and in all infected or suspected neonates, cultures of skin lesions, urine, nasopharynx, eyes, and cerebrospinal fluid are indicated. If available, a polymerase chain reaction test can be very helpful in rapid diagnosis. Intravenous acyclovir should be instituted as soon as possible after specimens are collected to minimize the chance of replication of virus in the CNS and systemic dissemination of HSV. Prompt recognition and early therapeutic intervention lead to an improved outcome in the HSV-infected infant.28
Congenital Malformations of the Skin
Congenital malformations are developmental defects and are frequently observed in newborns. Single minor anomalies occur in approximately 15% of all newborns and are, by definition, of no functional significance. The supernumerary digit (Fig. 107-11), supernumerary nipple and the accessory tragus are common examples of minor malformations. The occurrence of two is less common, however, and the presence of three or more is unusual; a complete thorough physical examination is indicated to rule out other congenital abnormalities.29,30
Supernumerary digit, a common minor malformation.
Cutaneous Markers of Dysraphism
The skin and the nervous system are both derived from the ectoderm. The neural ectoderm separates from the epithelial ectoderm during the third to fifth week of gestation, occurring simultaneously with the formation and closure of the neural tube. Hence, errors in neural tube development [i.e., dysraphism (incomplete fusion)] can be associated with cutaneous lesions. Midline facial lesions, such as dermoid cysts, nasal gliomas (Fig. 107-12), and ACC (Fig. 107-9), can be markers of cranial dysraphism, whereas cutaneous lesions seen on the lumbosacral midline can signify underlying spinal dysraphism (Fig. 107-13). Imaging studies should be considered for midline nasal lesions before biopsy to rule out intracranial connections.31
A and B. Nasal glioma initially thought to be a hemangioma of infancy. A firm, reddish slightly pedunculated was noted off of the midline of the nasal root. The MRI showed no intracranial connection. C. Nasal glioma. Firm subcutaneous mass noted at birth.
A large, wide tuft of thick terminal hair on the lumbosacral spine noted at birth. She had underlying tethered cord (note midline scar due to surgical repair).
A retrospective review of 54 cases of congenital lumbosacral skin lesions (including deviated gluteal cleft)32 demonstrated that the highest risk of occult spinal dysraphism occurs with the presence of two or more congenital midline lumbosacral skin lesions, or if spinal cord dysfunction exists in the presence of one lumbar skin lesion. In addition, lumbosacral lipoma, human tail, and dermal sinus (as isolated findings) were highly associated with occult spinal dysraphism. Lumbosacral magnetic resonance imaging is indicated when these high-risk congenital lesions are present. An intermediate risk of occult spinal dysraphism was associated with atypical sacral dimple (≥5-mm diameter, or location ≥2.5 cm away from the anus), ACC, overlying hamartoma, or deviated gluteal cleft. In the presence of any of these lesions, ultrasound can be used to screen for occult spinal dysraphism if the infant is less than 4 months of age. If the infant is older, magnetic resonance imaging is needed to rule out dysraphism. Low-risk lesions that do not require imaging include isolated lumbosacral hemangioma, port-wine stain, focal, mild hypertrichosis, pigmented nevus, Mongolian spot, or simple sacral dimple (≤5-mm diameter and location 2.5 cm or closer to the anus).32 These findings are summarized in Table 107-5.
Table 107-5 Congenital Lumbosacral Midline Skin Lesions and Risk of Occult Spinal Dysraphism ||Download (.pdf)
Table 107-5 Congenital Lumbosacral Midline Skin Lesions and Risk of Occult Spinal Dysraphism
Risk of Occult Spinal Dysraphism
Congenital Lumbosacral Midline Lesion
Group 1: MRI indicated
- Two or more lesions of any kind
- One lesion + spinal cord dysfunction
- Dermal sinus
Group 2: Ultrasound if younger than age 4 month (then MRI if ultrasound is abnormal), MRI indicated for age >4 month, or if sonographic expertise unavailable
- Atypical sacral dimple (deep, farther than 2.5 cm from the anus, size ≥5 mm)
- Unclassified hamartoma
- Aplasia cutis congenita
- Deviation of gluteal crease
Group 3: No imaging needed
- Port-wine stain
- Hypertrichosis (unless large and/or unusual)
- Pigmented nevus
- Simple sacral dimple (<5-mm diameter, 2.5 cm or closer to the anus)
- Mongolian spot