Skin Care of the Newborn

The skin of the newborn is covered with a grayish-white, greasy material termed vernix caseosa . The vernix represents a physiologic protective covering derived partially from secretions of the sebaceous glands and in part as a decomposition product of the infant’s epidermis. Vernix contains protein, lipids, and water and provides water-binding free amino acids that facilitate the adaptation from amniotic fluid immersion in utero to the dry ambient postnatal state. Although its function is not completely understood, it may act as a natural protectant cream to “waterproof” the fetus in utero , where it is submerged in the amniotic fluid. Some studies suggest that vernix be left on as a protective coating for the newborn skin and that it be allowed to come off by itself with successive changes of clothing (generally within the first few weeks of life). It has been suggested that vernix-based topical creams may be effective in augmenting stratum corneum repair and maturation in infants and could play a role in the treatment of epidermal wounds.

The skin acts as a protective organ. Any break in its integrity therefore affords an opportunity for initiation of infection. The importance of skin care in the newborn is compounded by several factors:

• 1.
  

  The infant does not have protective skin flora at birth.

  
  
• 2.
  

  The infant has at least one and possibly two open surgical wounds (the umbilicus and circumcision site).

  
  
• 3.
  

  The infant is exposed to fomites and personnel that potentially harbor a variety of infectious agents.

Skin care should involve gentle cleansing with a nontoxic, nonabrasive neutral material. During the 1950s, the use of hexachlorophene-containing compounds became routine for the skin care of newborns as prophylaxis against Staphylococcus aureus infection. In 1971 and 1972, however, the use of hexachlorophene preparations as skin cleansers for newborns was restricted because of studies demonstrating vacuolization in the central nervous system (CNS) of infants and laboratory animals after prolonged application of these preparations. At the minimum, neonatal skin care should include gentle removal of blood from the face and head, and meconium from the perianal area, by gentle rinsing with water. Ideally, vernix caseosa should be removed from the face only, allowing the remaining vernix to come off by itself. However, the common standard of care is for gentle drying and wiping of the newborn’s entire skin surface, which is most desirable from a thermoregulatory standpoint. For the remainder of the infant’s stay in the hospital nursery, the buttocks and perianal regions should be cleansed with water and cotton or a gentle cloth. A mild soap with water rinsing may also be used at diaper changes if desired.

There is no single method of umbilical-cord care that has been proven to limit colonization and disease. Several methods include local application of isopropyl alcohol, triple dye (an aqueous solution of brilliant green, proflavine, and gentian violet), and antimicrobial agents such as bacitracin or silver-sulfadiazine cream. The routine use of povidone-iodine should be discouraged, given the risk of iodine absorption and transient hypothyroxinemia or hypothyroidism. A safer alternative is a chlorhexidine-containing product.

Cutis Marmorata

Cutis marmorata is a normal reticulated bluish mottling of the skin seen on the trunk and extremities of infants and young children ( Fig. 2-1 ). This phenomenon, a physiologic response to chilling with resultant dilation of capillaries and small venules, usually disappears as the infant is rewarmed. Although a tendency for cutis marmorata may persist for several weeks or months, this disorder bears no medical significance and treatment generally is unnecessary. In some children cutis marmorata may tend to recur until early childhood, and in patients with Down syndrome, trisomy 18, and the Cornelia de Lange syndrome, this reticulated marbling pattern may be persistent. When the changes are persistent (even with rewarming) and are deep violaceous in color, cutis marmorata telangiectatica congenita ( Fig. 2-2 ; see also Chapter 12 ) should be considered. In some infants a white negative pattern of cutis marmorata (cutis marmorata alba) may be created by a transient hypertonia of the deep vasculature. Cutis marmorata alba is also a transitory disorder and appears to have no clinical significance.

Cutis marmorata. Reticulate bluish mottling that resolves with rewarming.

Cutis marmorata telangiectatica congenita. Violaceous, reticulate patches with subtle atrophy. These changes did not resolve with rewarming and were associated with mild ipsilateral limb hypoplasia.

Harlequin Color Change

Harlequin color change, not to be confused with harlequin ichthyosis (see Chapter 5 ), is occasionally observed in full-term infants but usually occurs in premature infants. It occurs when the infant is lying on his or her side and consists of reddening of one-half of the body with simultaneous blanching of the other half. Attacks develop suddenly and may persist for 30 seconds to 20 minutes. The side that lies uppermost is paler, and a clear line of demarcation runs along the midline of the body. At times, this line of demarcation may be incomplete; when attacks are mild, areas of the face and genitalia may not be involved.

This phenomenon appears to be related to immaturity of hypothalamic centers that control the tone of peripheral blood vessels and has been observed in infants with severe intracranial injury as well as in infants who appear to be otherwise perfectly normal. Although the peak frequency of attacks of harlequin color change generally occurs between the second and fifth days of life, attacks may occur anywhere from the first few hours to as late as the second or the third week of life.

Bronze Baby Syndrome

Bronze baby syndrome is a term used to describe infants who develop a grayish-brown discoloration of the skin, serum, and urine while undergoing phototherapy for hyperbilirubinemia. Although the exact source of the pigment causing the discoloration is not clear, the syndrome usually begins 1 to 7 days after the initiation of phototherapy, resolves gradually over a period of several weeks after phototherapy is discontinued, and appears to be related to a combination of photoisomers of bilirubin or biliverdin or a photoproduct of copper-porphyrin metabolism. Infants who develop bronze baby syndrome usually have modified liver function, particularly cholestasis, of various origins. Although not all babies with cholestasis develop bronze baby syndrome during phototherapy, those that do should be investigated for underlying liver disease. The disorder should be differentiated from neonatal jaundice, cyanosis associated with neonatal pulmonary disorders or con­genital heart disease, an unusual progressive hyperpigmentation (universal-acquired melanosis, the “carbon baby” syndrome), and chloramphenicol intoxication (the “gray baby” syndrome), which is a disorder in infants with immature liver function who are unable to conjugate chloramphenicol and is characterized by elevated serum chloramphenicol levels, progressive cyanosis, abdominal distention, hypothermia, vomiting, irregular respiration, and vasomotor collapse. A distinctive purpuric eruption on exposed skin has also been described in newborns receiving phototherapy and is possibly related to a tran­sient increase in circulating porphyrins. This condition, however, is unlikely to be confused with bronze baby syndrome.

Sclerema Neonatorum

Sclerema neonatorum is a diffuse, rapidly spreading, wax-like hardening of the skin and subcutaneous tissue that occurs in premature or debilitated infants during the first few weeks of life. The disorder, usually associated with a serious underlying condition such as sepsis or other infection, congenital heart disease, respiratory distress, diarrhea, or dehydration, is characterized by a diffuse nonpitting woody induration of the involved tissues. The process is symmetrical, usually starting on the legs and buttocks, and may progress to involve all areas except the palms, soles, and genitalia. As the disorder spreads, the skin becomes cold, yellowish-white, mottled, stony hard, and cadaver-like. The limbs become immobile, and the face acquires a fixed mask-like expression. Infants with this disorder become sluggish, feed poorly, show clinical signs of shock, and in a high percentage of cases die.

Although the etiology of this disorder is unknown, it appears to represent a nonspecific sign of severe illness rather than a primary disease. Infants with this disorder are characteristically small or premature, debilitated, weak, cyanotic, and lethargic. In 25% of cases the mothers are ill at the time of delivery. Exposure to cold, hypothermia, peripheral chilling with vascular collapse, and an increase in the ratio of saturated to unsaturated fatty acids in the triglyceride fraction of the subcutaneous tissue (because of a defect in fatty acid mobilization) have been hypoth­esized as possible causes for this disorder but lack confirmation.

The histopathologic findings of sclerema neonatorum consist of edema and thickening of the connective tissue bands around the fat lobules. Although necrosis and crystallization of the subcutaneous tissue have been described, these findings are more characteristically seen in lesions of SCFN.

The prognosis of sclerema neonatorum is poor, and mortality occurs in 50% to 75% of affected infants. In a series of 51 infants with sclerema neonatorum in a special-care nursery within a Bangladeshi hospital, the fatality rate was 98%. In infants who survive, the cutaneous findings resolve without residual sequelae. There is no specific therapy, although steroids and exchange transfusion have been used.

Subcutaneous Fat Necrosis

Subcutaneous fat necrosis (SCFN) is a benign, self-limited disease that affects apparently healthy, full-term newborns and young infants. It is characterized by sharply circumscribed, indurated, and nodular areas of fat necrosis ( Fig. 2-7 ). The etiology of this disorder remains unknown but appears to be related to perinatal trauma, asphyxia, hypothermia, and in some instances, hypercalcemia. Although the mechanism of hypercalcemia in SCFN is not known, it has been attributed to aberrations in vitamin D or parathyroid homeostasis. Birth asphyxia and meconium aspiration seem to be commonly associated. In one large series, 10 out of 11 infants with SCFN had been delivered via emergency cesarean section for fetal distress, and nine of the 11 had meconium staining of the amniotic fluid. The relationship between SCFN, maternal diabetes, and cesarean section, if any, is unclear. SCFN after ice-bag application for treatment of supraventricular tachycardia has been reported, and it has also been observed after selective head or generalized cooling for hypoxic–ischemic encephalopathy.

Subcutaneous fat necrosis. Indurated, erythematous plaques on the shoulders and back of this 1-week-old boy.

The onset of SCFN is generally during the first few days to weeks of life. Lesions appear as single or multiple localized, sharply circumscribed, usually painless areas of induration. Occasionally the affected areas may be tender, and infants may be uncomfortable and cry vigorously when they are handled. Lesions vary from small erythematous, indurated nodules to large plaques, and sites of predilection include the cheeks, back, buttocks, arms, and thighs. Many lesions have an uneven lobulated surface with an elevated margin separating it from the surrounding normal tissue. Histologic examination of SCFN reveals larger-than-usual fat lobules and an extensive inflammatory infiltrate, needle-shaped clefts within fat cells, necrosis, and calcification. Magnetic resonance imaging (MRI) reveals decreased T1 and increased T2 signal intensity in affected areas.

The prognosis for SCFN is excellent. Although lesions may develop extensive deposits of calcium, which may liquefy, drain, and heal with scarring, most areas undergo spontaneous resolution within several weeks to months. Hypercalcemia is a rare association, and infants with this finding may require low calcium intake, restriction of vitamin D, and/or systemic corticosteroid therapy. Etidronate therapy has been reported for treatment of recalcitrant SCFN-associated hypercalcemia. Infants should be monitored for several months after delivery, because the onset of hypercalcemia can be delayed for several months. Other rare systemic complications may include thrombocytopenia, hypoglycemia, and hypertriglyceridemia, all of which tend to be mild and/or self-limited.

Miliaria

Differentiation of the epidermis and its appendages, particularly in the premature infant, is often incomplete at birth. As a result of this immaturity, a high incidence of sweat-retention phenomena may be seen in the newborn. Miliaria, a common neonatal dermatosis caused by sweat retention, is characterized by a vesicular eruption with subsequent maceration and obstruction of the eccrine ducts. The pathophysiologic events that lead to this disorder are keratinous plugging of eccrine ducts and the escape of eccrine sweat into the skin below the level of obstruction (see Chapter 8 ).

Virtually all infants develop miliaria under appropri­ate conditions. There are two principal forms of this disorder:

• 1.
  

  Miliaria crystallina (sudamina), which consists of clear superficial pinpoint vesicles without an inflammatory areola;

  
  
• 2.
  

  Miliaria rubra (prickly heat), representing a deeper level of sweat gland obstruction and characterized by small discrete erythematous papules, vesicles, or papulovesicles ( Fig. 2-8 ).

  
  

  
  

  
  

  

  
  

  
  

  Miliaria rubra. Multiple, erythematous, pinpoint macules and papules in an infant with atopic dermatitis who was being treated with overapplication of greasy emollients.

  
  

The incidence of miliaria is greatest in the first few weeks of life owing to the relative immaturity of the eccrine ducts, which favors poral closure and sweat retention. A pustular form of miliaria rubra has been observed in association with pseudohypoaldosteronism during salt-losing crises.

Therapy for miliaria is directed toward avoidance of excessive heat and humidity. Light-weight cotton clothing, cool baths, and air conditioning are helpful in the management and prevention of this disorder. Avoidance of emollient overapplication (i.e., in infants with atopic dermatitis) should also be recommended, especially in warm, humid climates or in the winter when infants are bundled under heavy clothing.

Milia

Milia, small retention cysts, commonly occur on the face of newborns. Seen in 40% to 50% of infants, they result from retention of keratin within the dermis. They appear as tiny 1- to 2-mm pearly white or yellow papules. Particularly prominent on the cheeks, nose, chin, and forehead, they may be few or numerous and are often grouped ( Fig. 2-9 ). Lesions may occasionally occur on the upper trunk, limbs, penis, or mucous membranes. Although milia of the newborn may persist into the second or third month, they usually disappear spontaneously during the first 3 or 4 weeks of life and accordingly require no therapy. Persistent milia in an unusual or widespread distribution, particularly when seen in association with other defects, may be a manifestation of hereditary trichodysplasia (Marie-Unna hypotrichosis), dystrophic forms of epidermolysis bullosa, Bazex or Rombo syndromes, or the oral-facial-digital syndrome, type I.

Milia. Clustered, small, white papules on the lateral cheek.

Bohn Nodules and Epstein Pearls

Discrete, 2- to 3-mm round, pearly white or yellow, freely movable elevations at the gum margins or midline of the hard palate (termed Bohn nodules and Epstein pearls , respectively) are seen in up to 85% of newborns. Clinically and histologically the counterpart of facial milia, they disappear spontaneously, usually within a few weeks of life, and require no therapy.

Sebaceous Gland Hyperplasia

Sebaceous gland hyperplasia represents a physiologic phenomenon of the newborn manifested as multiple, yellow to flesh-colored tiny papules that occur on the nose, cheeks, and upper lips of full-term infants ( Fig. 2-10 ). A manifestation of maternal androgen stimulation, these papules represent a temporary disorder that resolves spontaneously, generally within the first few weeks of life.

Sebaceous gland hyperplasia. Yellow-white, pinpoint papules on the nasal tip of this 2-day-old boy.

Acne Neonatorum

Occasionally infants develop a facial eruption that re­­sembles acne vulgaris as seen in adolescents ( Fig. 2-11 ). Although the etiology of this disorder is not clearly defined, it appears to develop as a result of hormonal stimulation of sebaceous glands that have not yet involuted to their childhood state of immaturity. In mild cases of acne neonatorum, therapy is often unnecessary; daily cleansing with soap and water may be all that is required. Occasionally, mild keratolytic agents or topical antibiotics may be helpful (see Chapter 8 ). Unusually severe or recalcitrant cases of acne neonatorum warrant investigation for underlying androgen excess.

Acne neonatorum. Erythematous papules and papulopustules on the cheek.

A facial acneiform eruption in infants has been associated with the saprophytic Malassezia species and has been termed neonatal cephalic pustulosis (see Chapter 8 ). Lesions consist of pinpoint papules, papulopustules, or larger pustules, and they are located on the cheeks, chin, and forehead ( Fig. 2-12 ). A correlation may exist between the clinical severity of lesions and the colonization with this fungal saprophyte. In these infants, topical antifungal agents may lead to more rapid resolution of lesions.

Neonatal cephalic pustulosis. This 2-day-old male had numerous small and large pustules on the forehead, cheeks, and chin. They cleared rapidly over 1 week with ketoconazole cream.

Erythema Toxicum Neonatorum

Erythema toxicum neonatorum (ETN), also known as toxic erythema of the newborn, is an idiopathic, asymptomatic, benign, self-limiting, cutaneous eruption in full-term newborns. Lesions consist of erythematous macules, papules, and pustules ( Fig. 2-13 ), or a combination of these, and may occur anywhere on the body, especially the forehead, face, trunk, and extremities. The fact that these lesions (which histologically reveal follicular-centered eosinophils) often tend to spare the palms and soles may be explained by the absence of pilosebaceous follicles in these areas.

Erythema toxicum neonatorum. Blotchy, erythematous macules and edematous papules.

ETN often initially appears as a blotchy, macular erythema that then develops firm, 1- to 3-mm, pale yellow or white papules and pustules. The erythematous macules are irregular or splotchy in appearance, varying from a few millimeters to several centimeters in diameter. They may be seen in sharp contrast to the surrounding unaffected skin, may blend into a surrounding erythema, or may progress to a confluent eruption.

Although ETN appears most commonly during the first 3 to 4 days of life, it has been seen at birth and may be noted as late as 10 days of age. Exacerbations and remissions may occur during the first 2 weeks of life, and the duration of individual lesions varies from a few hours to several days. The etiology of ETN remains obscure. One study suggested that it represents an immune response to microbial colonization of the skin at the hair follicle. ETN incidence data are variable. Some authors report an incidence as low as 4.5%; others report incidences varying from 31% to 70% of newborns. Two large prospective series of skin findings in newborns (one in the United States and one in Turkey) found incidences of 7% and 13.1%, respectively. The incidence of ETN clearly appears to increase with increasing gestational age of the infant. No sexual or racial predisposition has been noted.

ETN is usually diagnosed clinically. Skin biopsy, which is rarely necessary, reveals a characteristic accumulation of eosinophils within the pilosebaceous apparatus. The diagnosis can be rapidly differentiated from other newborn pustular conditions by cytologic examination of a pustule smear that with Wright or Giemsa staining reveals a predominance of eosinophils. Affected infants may have a peripheral eosinophilia. Although the eosinophilic response has led some observers to attribute the etiology of this disorder to a hypersensitivity reaction, specific allergens have never been implicated or confirmed.

Since erythema toxicum is a benign, self-limiting, asymptomatic disorder, no therapy is indicated. Occasionally, however, it may be confused with other pustular eruptions of the neonatal period, including transient neonatal pustular melanosis (TNPM), milia, miliaria, and congenital infections including candidiasis, herpes simplex, or bacterial processes. Of these, the congenital infections are the most important diagnostic considerations because of the implications for possible systemic involvement. Table 2-3 lists the differential diagnosis of the newborn with vesicles or pustules.

Behçet syndrome. Shallow ulcerations on the scrotum, foreskin, and glans penis of an infant male with oral erosions and the human leukocyte antigen (HLA)-B51 group genotype. Note the associated papulopustular lesions on the medial thighs and buttocks, another characteristic feature of Behçet syndrome.

Eosinophilic Pustular Folliculitis

Eosinophilic pustular folliculitis (EPF) is an idiopathic dermatosis that occurs in both adults and infants. When it occurs in neonates or young infants, it may be clinically confused with other vesiculopustular disorders. Lesions consist of follicular pustules, most commonly occurring on the scalp and the extremities ( Fig. 2-15 ). They tend to recur in crops, in a similar fashion to acropustulosis of infancy (see below), and some suggest that these conditions may be related. As opposed to the adult form of EPF, the infancy-associated type does not reveal lesions grouped in an annular arrangement. EPF tends to present before 14 months of age in the majority of patients. Histologic evaluation reveals an eosinophilic, follicular, inflammatory infiltrate, and peripheral eosinophilia may be present. EPF of infancy appears to be distinct from classic (adult) and human immunodeficiency virus (HIV)-associated EPF, although an infant with HIV and EPF has been reported. Importantly, infantile EPF may occasionally be the presenting sign of hyperimmunoglobulinemia E syndrome (HIES) (see Chapter 3 ). Treatment for EPF is symptomatic, including topical corticosteroids and antihistamines, with eventual spontaneous resolution by 3 years of age in the majority of patients. Topical tacrolimus may be useful in patients who are unresponsive to topical corticosteroids.

Eosinophilic pustular folliculitis. Erythematous papules and pustules on the scalp of an infant female, who was subsequently diagnosed with hyperimmunoglobulinemia E syndrome.

Impetigo Neonatorum

Impetigo in newborns may occur as early as the second or third day or as late as the second week of life. It usually presents as a superficial vesicular, pustular, or bullous lesion on an erythematous base. Vesicles and bullae are easily denuded, leaving a red, raw, and moist surface, usually without crust formation. Blisters are often wrinkled, contain some fluid, and are easily denuded. Lesions tend to occur on moist or opposing surfaces of the skin, as in the diaper area, groin, axillae, and neck folds. S. aureus pustulosis (or neonatal pustulosis) is a characteristic manifestation of cutaneous S. aureus infection in the neonate or infant. Patients have small pustules on an erythematous base ( Fig. 2-16 ), often distributed in the diaper region. The lesions denude easily upon swabbing, and culture is positive for S. aureus . Streptococci may occasionally be causative. In term or late preterm neonates with localized involvement and without fever or systemic symptoms, evaluation for serious bacterial illness is generally not required, and treatment in the outpatient setting is often sufficient. However, a complete blood cell count and blood culture is advisable given the rare association with bacteremia.

Neonatal Staphylococcus aureus pustulosis with multiple pustules in the diaper region. Note some superficial erosions with peripheral collarettes of scale. The culture was positive for S. aureus .

The term pemphigus neonatorum is an archaic misnomer occasionally applied to superficial bullous lesions of severe impetigo widely distributed over the surface of the body. However, a transient neonatal form of pemphigus vulgaris does exist and is caused by transplacental passage of antibodies from a mother with the same disease (see Chapter 13 ).

Sucking Blisters

Sucking blisters, presumed to be induced by vigorous sucking on the affected part in utero , are seen in up to 0.5% of normal newborns as 0.5- to 2-cm oval bullae or erosions on the dorsal aspect of the fingers, thumbs, wrists, lips, or radial aspect of the forearms. These lesions, which must be differentiated from bullous impetigo, epidermolysis bul­losa, and herpes neonatorum, resolve rapidly and without sequelae.

Transient Neonatal Pustular Melanosis

Transient neonatal pustular melanosis (TNPM) is a benign self-limiting disorder of unknown etiology characterized by superficial vesiculopustular lesions that rupture easily and evolve into hyperpigmented macules ( Fig. 2-17 ). This disorder is seen in fewer than 1% of newborns and occurs most commonly in infants with black skin. Lesions begin as superficial sterile pustules ( Fig. 2-18 ) that rupture easily to leave a collarette of fine, white scale around a small hyperpigmented macule. Although the distribution may be diffuse, common areas of involvement include the inferior chin, forehead, neck, lower back, and shins. Rarely, vesicles that do not progress to pigmented macules may be detected on the scalp, palms, and soles.

Transient neonatal pustular melanosis. Papules and papulopustules that rupture to leave a collarette of fine scales and eventual hyperpigmentation.

(Courtesy of Nancy B. Esterly, MD.)

Transient neonatal pustular melanosis. Tense pustules and collarettes of scale at sites of older lesions.

Wright-stained smears of the pustules of TNPM, in contrast to lesions of ETN, demonstrate variable numbers of neutrophils, few or no eosinophils, and cellular debris. Histopathologic evaluation is usually unnecessary.

TNPM is a benign disorder without associated systemic manifestations, and therapy is unnecessary. The pustular lesions usually disappear within 24–48 h, leaving behind hyperpigmented macules that fade gradually, usually over several weeks to months. Occasionally, newborns may have solely the hyperpigmented macules, in which case it is presumed that the pustular phase occurred (and resolved) in utero .

Acropustulosis of Infancy

Acropustulosis of infancy, also known as infantile acropustulosis ( IA ), is an idiopathic pustular disorder with onset usually between birth and 2 years of age. It is characterized by recurrent, pruritic, vesiculopustular lesions that recur every few weeks to months. The lesions begin as pinpoint erythematous papules and enlarge into well-circumscribed discrete pustules. They are concentrated on the palms ( Fig. 2-19 ) and soles ( Fig. 2-20 ) and appear in lesser numbers on the dorsal aspect of the hands, feet, wrists, and ankles. Occasional lesions may occur on the face and scalp.

Acropustulosis of infancy. Multiple tense erythematous papules and pustules on the palm of this 4-month-old girl.

Acropustulosis of infancy. Tense pustules, some of which have ruptured, on the plantar and lateral surfaces of the foot of a 14-month-old female.

The differential diagnosis of IA includes scabies, dyshidrotic eczema, pustular psoriasis, ETN, TNPM, impetigo, and subcorneal pustular dermatosis. However, the characteristic presentation and course of IA is usually distinctive enough to render a clinical diagnosis. A smear of pustule contents (or histologic evaluation) reveals large numbers of neutrophils and occasionally eosinophils. Although the etiology of IA remains unclear, several authors have noted a possible association with preceding scabies infestation. IA appears to be common in internationally adopted children.

Patients with IA experience fewer and less intense flares of their lesions with time, and the entire process usually subsides within 2 to 3 years. Pruritus, however, may be severe early in the course, making therapy desir­able. Possible associations include irritability, sleeplessness, excoriation, and secondary bacterial infection. Systemic antihistamines, usually in high doses, may relieve pruritus. High-potency topical corticosteroids are quite effective for this condition, and given the limited distribution of lesions, the epidermal thickness at affected (acral) sites, and the periodicity of flares, concerns regarding sys­temic absorption of these medications should be minimal. Dapsone has long been a recommended therapy for severe cases, but the risk-to-benefit ratio of this agent is not generally justified in patients with IA.

Congenital Erosive and Vesicular Dermatosis

Congenital erosive and vesicular dermatosis healing with reticulated supple scarring is an uncommon disorder characterized by erosive and bullous lesions that, as the name implies, are present at birth and heal with characteristic scarring. Although its cause is unknown, it appears to represent a nonhereditary intrauterine event such as infection or amniotic adhesions, or perhaps an unusual healing defect of immature skin. The disorder generally involves skin of the trunk, extremities, scalp, face, and occasionally the tongue, with sparing of the palms and soles.

Congenital erosive and vesicular dermatosis occurs most often in premature infants and presents with extensive cutaneous ulcerations and intact vesicles that develop crusting and then heal during the first month of life. Occasionally, blistering may continue to occur beyond infancy. Generalized, supple, reticulated scars occur with alternating elevated and depressed areas ( Fig. 2-21 ). Up to 75% of the cutaneous surface may be involved, and the skin lesions have been described as having depressed hypopigmented regions alternating with normal to hyperpigmented zones. Scars on the trunk and head, which often have a cobblestone-like appearance, may be oriented along the cutaneous lines of cleavage; on the limbs they tend to follow the long axes of the extremities. Facial involvement was present in roughly 50% of published cases in one review. Although the eyebrows are usually normal, alopecia may be noted on the scalp. Nails may be absent or hypoplastic, and affected areas on the tongue may manifest scarring and absence of papillae. Dentition is usually normal. Hyperthermia, especially in warm weather or after exertion, is common and although sweating is absent in scarred areas, compensatory hyperhidrosis in normal-appearing skin may be noted. Chronic conjunctivitis is a major continuing problem for these patients, and corneal scarring may occur. Some patients have also been found to have neurologic defects, including mental and motor retardation, hemiparesis, microcephaly, pachygyria, cerebral palsy, and seizures.

Congenital erosive and vesicular dermatosis healing with reticulated supple scarring. There is generalized, supple, reticulated scarring. Note also the associated scalp alopecia.

Seborrheic Dermatitis

Seborrheic dermatitis is a common, self-limiting condition of the scalp, face, ears, trunk, and intertriginous areas characterized by greasy scaling, redness, fissuring, and occasional weeping. It appears to be related to the sebaceous glands and has a predilection for so-called “seborrheic” areas where the density of these glands is high. It usually presents in infants with a scaly dermatitis of the scalp termed cradle cap ( Fig. 2-22 ) and may spread over the face, including the forehead, ears, eyebrows, and nose. Other areas of involvement include the intertriginous zones, umbilicus, and anogenital region ( Fig. 2-23 ). (For a more detailed discussion of seborrheic dermatitis and its therapy, see Chapter 3 .)

Seborrheic dermatitis of the scalp (cradle cap). Erythema and greasy yellow scales involving the scalp of an infant male who also had similar changes in the eyebrows.

Seborrheic dermatitis. Erythema of the medial thighs, suprapubic region and buttocks. This presentation can be difficult to distinguish from infantile psoriasis, but the scaling tends to be more mild and the response to topical anti-inflammatory therapy more brisk. The whitish debris in this child represents the barrier cream that his parents had applied rather than scaling.

Leiner Disease

The term Leiner disease refers to a shared phenotype for a number of nutritional and immunologic disorders characterized by severe seborrheic dermatitis with exfoliation, failure to thrive, and diarrhea. The disorder may occur during the first week of life but generally starts around 2 to 4 months of age. Patients are particularly prone to recurrent yeast and Gram-negative infections. Among disorders that may show this phenotype are: deficiency or dysfunction of complement, Bruton agammaglobulinemia, severe combined immunodeficiency, and HIES.

Diaper Dermatitis

Diaper dermatitis is perhaps the most common cutaneous disorder of infancy and early childhood. The term is used to describe an acute inflammatory skin reaction in the areas covered by the diaper. The incidence of diaper dermatitis is estimated to be between 7% and 35%, with a peak incidence at 9 to 12 months of age.

The term diaper rash is commonly used as a diagnosis, as though the diverse dermatoses that may affect this region constitute a single clinical entity. In actuality, diaper dermatitis is not a specific diagnosis and is best viewed as a variable-symptom complex initiated by a combination of factors, the most significant being prolonged contact with urine and feces, skin maceration, and, in many cases, secondary infection with bacteria or Candida albicans . Although diaper dermatitis may often be no more than a minor nuisance, eruptions in this area may not only progress to secondary infection and ulceration but may become complicated by other superimposed cutaneous disorders or represent a manifestation of a more serious disease.

The three most common types of diaper dermatitis are chafing dermatitis, irritant contact dermatitis, and diaper candidiasis. However, the differential diagnosis of diaper dermatitis is broad ( Box 2-1 ). In patients in whom a response to therapy is slow or absent, alternative diagnoses should be considered and appropriate diagnostic evaluations performed. The following sections contain a brief discussion of several potential causes of diaper dermatitis. Many of these entities are discussed in more detail in other chapters.

Irritant Contact Dermatitis

Irritant contact diaper dermatitis usually involves the convex surfaces of the buttocks, the vulva, the perineal area, the lower abdomen, and the proximal thighs, with sparing of the intertriginous creases ( Fig. 2-24 ). The disorder may be attributable to contact with proteolytic enzymes in stool and irritant chemicals such as soaps, detergents, and topical preparations. Other significant factors appear to be excessive heat, moisture, and sweat retention associated with the warm local environment produced by the diaper.

Irritant contact diaper dermatitis. Erythema of the vulva, buttocks, and medial thighs. The inguinal creases were relatively spared.

The etiology of irritant contact diaper dermatitis is multifactorial, and past hypotheses have included potential roles for ammonia, bact­eria and bacterial products, and urine pH. In 1921 when Cooke demon­strated that an aerobic Gram-positive bacillus ( Bacillus ammoniagenes ) was capable of liberating ammonia from urea, this organism was pinpointed as the etiologic agent of most diaper dermatoses. More recent studies, however, have refuted the role of urea-splitting bacteria in the etiology of this disorder and incriminate a combination of wetness, frictional damage, impervious diaper coverings, and increase in skin pH. It is suggested that urinary wetness increases the permeability of the skin to irritants as well as the pH of the diaper environment, thus intensifying the activities of the fecal proteases and lipases, the major irritants responsible for this disorder.

Several technological innovations in the design of disposable diapers and other diapering products have aimed to reduce moisture and irritancy in this environment, thus decreasing the risk of irritant dermatitis. The introduction of absorbent gelling materials into diaper technology was one such breakthrough and has been shown to result in less diaper dermatitis than conventional cellulose-core disposable diapers. Other recent innovations include nonirritating disposable diaper wipes and diapers designed to deliver petrolatum-based formulations to the skin.

A blistering, erosive contact dermatitis has been ob­­served after oral ingestion (either intentional or accidental) of senna-containing laxatives. Patients typically have with well-demarcated, diamond-shaped eroded plaques with desquamation that in some cases initially could be mistaken for scalding burns. Prolonged contact with stool (i.e., via overnight wearing of the diaper) is often reported.

Diaper Candidiasis

Candidal (monilial) diaper dermatitis is a commonly overlooked disorder and should be suspected whenever a diaper rash fails to respond to usual therapeutic measures. Cutaneous candidiasis is a possible sequela of systemic antibiotic therapy and should be considered in any diaper dermatitis that develops during or shortly after antibiotic administration.

Candidal diaper dermatitis presents as a widespread, beefy-red erythema on the buttocks, lower abdomen, and inner aspects of the thighs. Characteristic features include a raised edge, sharp marginalization with white scales at the border, and pinpoint pustulovesicular satellite lesions (the diagnostic hallmark) ( Fig. 2-25 ). Although cutaneous candidiasis commonly occurs in association with oral thrush ( Fig. 2-26 ), the oral mucosa may be uninvolved. Infants harbor C. albicans in the lower intestine, and it is from this focus that infected feces present the primary source for candidal diaper eruptions.

Diaper candidiasis. Beefy-red, erythematous plaques with multiple red satellite papules and papulopustules.

Oral candidiasis (thrush). Gray-white, cheesy patches and plaques of the buccal mucosa, tongue, and gingiva.

If necessary, the diagnosis of candidal diaper dermatitis may be confirmed by microscopic examination of a potassium hydroxide preparation of skin scrapings, which reveals egg-shaped budding yeasts and hyphae or pseudohyphae. Growth of yeast on Sabouraud medium implanted with skin scrapings can also confirm the diagnosis, usually within 48 to 72 hours.

Psoriasis

Psoriasis of the diaper area must also be considered in persistent diaper eruptions that fail to respond to otherwise seemingly adequate therapy ( Fig. 2-27 ). The sharp demarcation of lesions suggests diaper area psoriasis, but the typical scaling of psoriasis may be obscured because of the moisture of the diaper region. The presence of nail changes and red, well-marginated plaques with silvery mica-like scales on the trunk, face, axillae, umbilicus, or scalp may help confirm this diagnosis (see Chapter 4 ), although affected infants may have involvement limited to the diaper area.

Psoriasis (diaper). Sharply demarcated, erythematous, scaly plaques involving the genitals and suprapubic region in this infant male.

Jacquet Dermatitis

The term Jacquet dermatitis is used to describe a severe erosive diaper eruption with ulcerated papules or nodules ( Fig. 2-28 ). In male infants, erosion and crusting of the glans penis and urinary meatus may result in painful or difficult urination.

Jacquet dermatitis. Severe diaper area erythema with ulcerated papules and islands of reepithelialization.

Acrodermatitis Enteropathica

Acrodermatitis enteropathica, a disorder of zinc deficiency, may mimic a severe irritant contact dermatitis in the diaper area (see Chapter 24 ). Patients have a periorificial erosive dermatitis that is often most accentuated in the diaper region ( Fig. 2-29 ) but also may involve the perioral face. Erythema and pustules may involve intertriginous or acral sites, and diarrhea, failure to thrive, and alopecia are commonly present.

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