Dermatologic Conditions
James G. H. Dinulos
Gary L. Darmstadt
INTRODUCTION
As an organ, the skin provides essential functions for survival and can be an indicator of overall health. At no other time in life are these roles more evident than in a newborn infant who is adjusting to life outside the uterus. Abnormalities in the skin may be extremely distressing to parents and it is important for medical providers to be able to distinguish a worrisome from a banal newborn skin disorder. To assist in this process, this chapter reviews basics of skin structure and development, techniques of skin examination and recognition and management of important newborn skin disorders.
SKIN DEVELOPMENT, STRUCTURE, AND FUNCTION
The skin is composed of three anatomic layers, epidermis, dermis, and fat; and three adnexal structures, hair, nails, and glands. The formation of these skin layers and structures from primitive embryonic tissues occurs in a predicable and sequential manner. Most structural development is complete by 24 weeks of gestation, however, complete functional activity of the skin, such as epidermal barrier development, is not achieved until after birth (1).
The epidermis plays critical functions in fluid homeostasis and in protection from infections, toxins, and adverse effects of ultraviolet radiation (2). The outermost layer, the stratum corneum, provides most of this protection. Lamellar bilayers composed of hydrophobic lipids, principally fatty acids, cholesterol and ceramides, are “cemented” between multiple layers of tightly knit protein- and keratin-rich cornified cells (2,3). These lipids and proteins protect by creating an impermeable barrier and by providing an acidic and xeric environment that impedes microbial invasion. The epidermis is not merely a static barrier, however, as keratinocytes actively produce antibacterial products, including cytokines, lipid breakdown products and cationic antimicrobial peptides that provide an important link between the innate and adaptive immune systems (4,5).
Prematurity and a number of skin disorders disrupt epidermal permeability barrier function and may allow for massive transepidermal water loss (TEWL) (6). Premature infants can lose up to 30% of their total body weight in 24 hours, as their rate of TEWL may be 10 to 15 times greater than in a full-term infant (7). Such significant fluid losses can cause hypotension, electrolyte imbalance, increased caloric demands, and may contribute to intraventricular hemorrhage (IVH) and necrotizing enterocolitis. Epidermal barrier development is accelerated by exposure to the dry, extra-uterine environment (7,8). This barrier maturation typically takes 2 to 4 weeks and may be delayed for 8 weeks in extremely premature infants. Thus, premature infants are particularly vulnerable to infections and toxins, particularly during the first week of life when approximately two thirds of all neonatal deaths worldwide occur.
During development, skin and its structures are thought to migrate along the “lines of Blaschko” (9). Most authorities believe that Blaschko lines are an expression of epidermal and not dermal migration. Conditions that follow these lines are thought to result from a single postzygotic mutation of a single clone of cells or from a mutation on the X chromosome that is brought about by X-inactivation (10). Incontinentia pigmenti and linear epidermal nevi are two examples of conditions that occur along lines of Blaschko.
Newborn skin may look and feel different depending on the newborn’s gestational age. Premature infants less than 32 weeks estimated gestational age (EGA), have skin that appears thin and transparent. Infants born after 40 weeks EGA have thicker skin with wrinkles and peeling. Most term infants have a pasty substance on their skin, referred to as vernix caseosa. Vernix caseosa is a substance composed of sloughed corneocytes, shed lanugo hair and lipids (11). The role of this substance is not fully understood, although it is thought to assist in passage through the birth canal and have an important role in skin hydration and innate immune defense (12). Most premature and postmature infants have little vernix.
Skin adnexal structures (hair, nails, and glands) can provide valuable clues to the overall well-being of the newborn.
Most infants are born with hair on the scalp and some have hair on the body. Typically, hair in the newborn undergoes “physiologic” shedding at approximately 3 months of age.
Most infants are born with hair on the scalp and some have hair on the body. Typically, hair in the newborn undergoes “physiologic” shedding at approximately 3 months of age.
TABLE 55-1 STEPS IN A NEWBORN SKIN EXAMINATION | |
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EXAMINATION OF NEWBORN SKIN
Newborns should be examined with an adequate light source, preferably natural sunlight. Side-lighting and magnification are helpful to accentuate surface topography. Handheld devices applied directly to the skin such as a dermatoscope can be helpful, especially to examine pigmented lesions. The environment should be kept warm to minimize reactive vascular changes as a result of temperature. Table 55-1 summarizes the steps of a newborn skin examination and an approach to dermatological diagnosis. Table 55-2 lists primary lesions, secondary lesions, special configurations and distributions of skin lesions.
SCALY RASHES
Rashes are common in the first month of life, affecting 80% of newborns (13). During the first week of life, the vast majority of term and postterm infants will have superficial peeling or desquamation of skin; it rarely occurs in those under 35 weeks of gestation. Deeper skin peeling that results in denuded skin or continuous peeling for longer than 1 week are potential warning signs of an under- lying systemic process. Scales should be examined for quality (firm, adherent), color (white, yellow), and associated inflammation (redness), features which help in identification of a variety of skin diseases.
Inflammatory Rashes
Diaper Dermatitis
Diaper dermatitis is common in newborns. It is characterized by a pink to red scaly rash localized to the diaper area and is caused by prolonged exposure to urine and stool combined with the frictional forces of the diaper. This irritant contact dermatitis should be distinguished from numerous other dermatological conditions affecting the diaper area. Irritant contact dermatitis affects skin contacting the diaper, such as the pubis, penis, labia majora, scrotum, upper thighs, and buttocks. Generally, the creases are spared. Continued irritant exposure may produce painful fissures and ulcerations (Jacquet’s erosive dermatitis), especially in premature infants (Fig. 55-1). Involved skin is susceptible to secondary infection, primarily with Candida albicans, Staphylococcus aureus and Streptococcus pyogenes. Candida albicans causes pustules on a red base (“beefy-red”) that, after rupture, form erosions surrounded by fine superficial white scale. Frequently, the inguinal and gluteal folds are involved. Staphylococcus aureus and S. pyogenes cause a superficial skin infection called impetigo, which is characterized by pustules on a red base that form yellow crusts when ruptured. Certain types of S. aureus produce an epidermolytic toxin, causing superficial bullae. However, secondary bacterial infection with S. aureus and S. pyogenes may be characterized primarily by superficial erosions and crusting.
TABLE 55-2 PRIMARY AND SECONDARY SKIN LESIONS, CONFIGURATIONS, AND LOCATIONS | |
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 Figure 55-1 Erosive dermatitis. (See color plate) |
Factors involved in the genesis of diaper dermatitis include increased skin wetness (e.g., sweat, urine and stool) of the stratum corneum (14), which makes the skin more susceptible to friction from the diaper material; and irritation from elevated pH caused by urinary ammonia and from activation of fecal proteases and lipases in the alkaline milieu (15,16). When alkaline urine combines with feces, the potential for irritation is compounded. Breast-fed infants have been noted to have stools with lower pH, which may account for their decreased incidence of diaper dermatitis.
The differential diagnosis of irritant diaper dermatitis includes psoriasis, seborrheic dermatitis, secondary syphilis, histiocytosis, acrodermatitis enteropathica, and cystic fibrosis. In those conditions, however, unlike diaper dermatitis, skin outside the diaper area is usually involved.
Treatment of diaper dermatitis should be targeted toward reducing skin wetness, minimizing contact of the skin with urine and feces, and eradicating infectious organisms. Skin wetness and irritation can be minimized in several ways. Ultra-absorbent diapers have been shown to be superior to cloth diapers in decreasing skin wetness and maintaining an acidic skin pH (17,18). Frequent diaper changes also help to minimize wetness. Barrier ointments (e.g., containing petrolatum) help keep urine and feces from contacting the skin. Other commercial products containing zinc, vitamin A and D also may be effective. The skin should be gently cleansed with water, or if necessary, a mild nonalkaline soap before barrier ointment re-application. Complete removal of barrier ointment with diaper changes is not necessary, however if attempted, may further exacerbate skin injury. When diaper dermatitis is unusually severe, recalcitrant, or when risk factors are likely to be ongoing (e.g., malabsorption syndromes), a thick layer of pectin-based paste without alcohol, followed by a barrier ointment or zinc oxide ointment, may prove efficacious. Commercially available diaper wipes can exacerbate irritation, and should be reserved for healthy appearing skin or for circumstances in which soap and water is not available.
Mild, low-potency topical corticosteroids such as 1% hydrocortisone ointment can be applied if inflammation is significant; however, higher potency topical steroids should be avoided because of the risk of cutaneous atrophy, striae, adrenal suppression, and Cushing syndrome. Combination antifungal-steroid compounds such as Lotrisone® and Mycolog II® have no use in treating neonatal skin conditions involving the diaper area. Each contains a potent topical corticosteroid (betamethasone dipropionate, and triamcinolone 0.1%, respectively) that when placed under diaper occlusion can cause cutaneous and systemic side effects. Use of powders also should be avoided. Fungal or bacterial superinfection can be managed with appropriate topical antimicrobial agents (19). Topical antifungal agents such as nystatin, clotrimazole, miconazole or ketoconazole can be safely applied to newborn skin. Bacterial infection generally responds to mupirocin or bacitracin. Neomycin carries a higher risk of allergic contact sensitization and should be avoided.
Intertrigo
Intertrigo is a symmetric, red, moist, macerated eruption that occurs in the skin folds. It is thought to be as a result of excessive sweating and close approximation of the skin surfaces. Yeast and bacterial super-infection is common (see diaper dermatitis). The most effective treatment is exposure of skin to air. Barrier creams and occlusive ointments can exacerbate intertrigo. A light application of 0.5% to 1% hydrocortisone cream two to three times a day, pimecrolimus or tacrolimus can help. Concurrent bacterial and/or yeast infections must be treated in a manner similar to irritant contact diaper dermatitis.
Seborrheic Dermatitis
Seborrheic dermatitis is characterized by red skin with white-yellow waxy scale, occurring on the scalp, eyebrows and in the intertriginous areas, especially in the posterior auricular, neck, axillary and inguinal folds (Fig. 55-2). Typical scale is absent in the intertriginous areas. The eruption begins at 2 to 3 weeks of age and in some infants becomes widespread over the ensuing months. The vast majority of patients with seborrheic dermatitis improve significantly during the first year of life. Persistent and severe seborrheic dermatitis may be associated with human immunodeficiency virus (HIV) infection and other underlying systemic diseases.
 Figure 55-2 Seborrheic dermatitis. |
Alterations in fatty acid metabolism, nutrition, and/or immunity, and infection with Pityrosporum ovalae have been thought to contribute to seborrheic dermatitis, but no firm cause has been established.
Atopic dermatitis, psoriasis, Langerhans cell histiocytosis, scabies, and dermatophyte infections are the principle conditions that must be excluded. In general, atopic dermatitis produces itching and psoriasis is more difficult to control than seborrheic dermatitis. Sometimes, several visits with careful observation and assessment of the therapeutic response are required to establish the proper diagnosis; skin biopsy sometimes becomes necessary for diagnosis.
When infants have limited involvement, no specific therapy is required other than mild shampoo and/or mineral oil to gently remove the scale. For more adherent, thick scalp scale, selenium sulfide shampoo or 2% ketoconazole shampoo may be helpful. Ketoconazole 2% cream, hydrocortisone 0.5% to 1% (lotion, cream, ointment), pimecrolimus cream and tacrolimus ointment applied once to twice daily are alternative therapies.
Psoriasis
Psoriasis can produce various cutaneous patterns such as guttate (tear drop), papules, pustules, and plaques. Most infants with psoriasis develop thick, pink plaques with firmly adherent thick white (micaceous) scale in a distribution similar to seborrheic dermatitis. Psoriasis in the newborn period is uncommon and congenital psoriasis is rare. When newborns are affected, they tend to have localized plaques, either on the scalp, diaper area, or hands. One can also see isolated nail and nail-fold involvement. Rarely, newborns develop generalized pustular erythroderma with fever.
The cause of psoriasis is unknown, but genetic influences play an important role because children with two psoriatic parents have an approximately 50% lifetime risk (20). Infection (S. pyogenes), cold weather, emotional stress, medications (antimalarials), and withdrawal from systemic corticosteroids have all been shown to provoke psoriatic flares. Psoriasis improves with therapies targeting T-lymphocytes (cyclosporine), suggesting that psoriasis is a T-lymphocyte mediated disease (21). T-lymphocytes comprise most of the psoriatic inflammatory cell infiltrate, further supporting the central role of T-lymphocytes (22).
A family history of psoriasis and “soft signs” of psoriasis (e.g., pink skin in the gluteal cleft, nail pitting, geographic tongue) are useful aids to diagnoses.
Medium-strength topical corticosteroids, tacrolimus, and pimecrolimus are the mainstay of therapy for infantile psoriasis. Short contact therapy (application for less than 30 minutes, followed by washing) with anthralin is a safe therapy, but may be limiting because of skin irritation. Calcipotriol may be applied, but to limited areas because of risk of vitamin D toxicity. Tar ointments are effective, but have an unpleasant odor, and stain the skin, and may not be well accepted by caregivers. Treatments targeting T-lymphocytes and associated cytokines are showing promising results in adults, but have not been studied in infants.
Atopic Dermatitis
Newborns with atopic dermatitis develop red, itchy, papules and plaques involving the forehead, cheeks and flexural surfaces, with relative sparing of the diaper area; there may be widespread generalized redness and scaling. Well-defined criteria exist to diagnose atopic dermatitis (23). In the newborn, atopic dermatitis is recognized when an infant has the typical rash and a family history of atopy, asthma or atopic dermatitis. In the young infant, pruritus may not be evident because of lack of scratching. Many infants are colonized with S. aureus and all are susceptible to viral infections (e.g., herpes simplex virus, small pox virus, molluscum contagiosum virus, and human papilloma virus).
Although up to 30% of infants have concurrent food hypersensitivity, atopic dermatitis is not thought to be caused simply by allergies. Rather, atopic dermatitis is a complex disorder subject to genetic, immunological, and environmental influences (24). Atopic dermatitis must be differentiated from seborrhea, psoriasis, fungal infection and scabies, and the treatment is similar to other noninfectious inflammatory conditions. Moisturizers, low-potency topical steroids, tacrolimus, pimecrolimus, avoidance of cutaneous irritation, and treatment of secondary infections are the fundamental elements of therapy.
Erythroderma
Erythroderma is a generalized red eruption as a result of a number of inherited and acquired conditions. Inflam-matory conditions (atopic dermatitis, seborrheic dermatitis, mastocytosis), infectious conditions (syphilis, herpes, S. aureus), metabolic conditions (methylmalonic aciduria, maple syrup urine disease, cobalamine deficiency), genetic skin diseases (ichthyosis, Netherton syndrome, nonbullous ichthyosiform erythroderma) immunodeficiency (severe combined immunodeficiency, common variable
hypogammaglobulinemia) are examples of conditions that can result in erythroderma. These infants have a compromised epidermal permeability barrier and are at risk for hypothermia, dehydration and sepsis. Diagnostic evaluation should be directed by history and physical examination. Typically, a skin biopsy is not helpful during acute erythroderma. Management focuses on fluid and temperature homeostasis, utilizing intravenous fluids, emollients and placing infants in isolettes with warm humidified air. Thecaloric requirements of the infant are dramatically increased and parenteral nutrition is sometimes required. Emollients applied two to three times daily assist in healing the skin barrier. Fissures should be treated with topical antibiotics such as mupirocin or bacitracin. Infants with signs of skin infection (e.g., crusting, oozing, malodorous areas) and sepsis should be cultured and treated with appropriate systemic antibiotics.
hypogammaglobulinemia) are examples of conditions that can result in erythroderma. These infants have a compromised epidermal permeability barrier and are at risk for hypothermia, dehydration and sepsis. Diagnostic evaluation should be directed by history and physical examination. Typically, a skin biopsy is not helpful during acute erythroderma. Management focuses on fluid and temperature homeostasis, utilizing intravenous fluids, emollients and placing infants in isolettes with warm humidified air. Thecaloric requirements of the infant are dramatically increased and parenteral nutrition is sometimes required. Emollients applied two to three times daily assist in healing the skin barrier. Fissures should be treated with topical antibiotics such as mupirocin or bacitracin. Infants with signs of skin infection (e.g., crusting, oozing, malodorous areas) and sepsis should be cultured and treated with appropriate systemic antibiotics.
Neoplastic
Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH) is a malignant condition as a result of abnormal clones of Langerhans cells. Langerhans cells are skin antigen presenting cells, derived from the bone marrow. Infants with LCH can develop a range of cutaneous findings, including crusted papules, pustules, vesicles, bullae, petechiae, purpura and nodules. Lesions can occur in the “seborrhea areas” (scalp, face, and flexural areas), and extremities, including the palms and soles. Many infants with LCH develop lesions limited to the skin. Cutaneous features of LCH often precede systemic signs (e.g., fever, hepatosplenomegaly, lymphadenopathy, anemia), however, making early diagnosis in more aggressive forms of LCH more difficult.
Acral vesicles and bullae must be differentiated from bullous impetigo, scabies and syphilis. LCH in the seborrheic areas can mimic benign inflammatory conditions such as intertrigo, seborrhea, psoriasis and atopic dermatitis.
All infants with LCH should have an evaluation for systemic involvement (hematologic, pulmonary, hepatic, renal, and skeletal) and they should be followed closely, because extracutaneous relapses may occur months to years after diagnosis. Topical corticosteroids and moisturizers can be used for cutaneous lesions, however, the rash typically responds poorly. Patients with systemic signs of disease should also be followed by pediatric hematology and/or oncology specialists.
 Figure 55-3 Collodion baby. |
Genetic Skin Diseases
Collodion Baby
Infants born with a thick “saran-wrap” covering of skin (collodion membrane) are referred to as collodion babies (Fig. 55-3). In the early newborn period, this covering can produce respiratory distress, painful cracks and fissures, temperature and fluid instability. Newborns frequently require oxygen, a humidified air isolette, and liberal applications of emollients. The eyes should be meticulously cared for because desiccation may cause corneal scarring. The collodion membrane sloughs over a period of 2 to 4 weeks. The vast majority of infants who present with a collodion membrane have lamellar ichthyosis. Other causes of collodion membrane include trichothiodystrophy, Sjogren-Larsson syndrome, Conradi-Hunermann syndrome, Gaucher’s disease (Type IIB), and Refsum disease. Ten percent to 15% of infants have no underlying disorder and have normal skin when the collodion membrane resolves.
Harlequin Ichthyosis
Infants with harlequin ichthyosis are born with an extremely thick and cracked, armor-like covering with pronounced eclabium and ectropion. Harlequin infants are exceedingly rare and usually die during infancy; however, there are some long term survivors. This condition is inherited in an autosomal recessive manner and the underlying genetic defect remains unknown. Treatment with a systemic retinoid may increase survival of these infants.
Ichthyosis
Ichthyosis is a term utilized to describe inherited and acquired conditions that produce “fish-like” scales. There are different clinical forms, including ichthyosis vulgaris, X-linked ichthyosis, and lamellar ichthyosis. Ichthyosis vulgaris, the most common form is inherited in an autosomal dominant manner and usually manifests after 3 months of age. Ichthyosis vulgaris can be associated with atopic dermatitis. Infants with ichthyosis vulgaris often have fine light-colored scale that becomes thicker on the lower extremities. X-linked ichthyosis occurs with an incidence of 1:6,000 males. Most infants show signs of this form of ichthyosis by 3 months of age. The skin develops thick brown firmly adherent scale with relative sparing of the flexural regions. X-linked ichthyosis is caused by a mutation in the steroid sulfatase gene, resulting in decreased
shedding of keratinocytes. Infants with X-linked ichthyosis are frequently born after prolonged labor, because absence of steroid sulfatase can lead to increased fetal productions of DHEAS and decreased placental estrogen. Undescended testes, testicular cancer, and cataracts are associated with X-linked ichthyosis. Lamellar ichthyosis is rare and inherited in an autosomal recessive manner, although autosomal dominant forms have been described. Most infants with lamellar ichthyosis have mutations in the transglutaminase 1 gene. Transglutaminase is important in keratinocyte differentiation. Alterations in this protein produce the thick plate-like scale seen in infants with lamellar ichthyosis. Sometimes, infants with lamellar ichthyosis are born with a saran-wrap covering of skin referred to as a collodion membrane (see section on collodion membrane). New-borns with ichthyosis are primarily managed with emollients, such as vaseline and aquaphor. A thinner lotion may be preferable, if a thicker emollient is difficult to apply. Keratolytic emollients containing lactic and salicylic acid should be avoided, because they can be systemically absorbed. Infants with severe forms of ichthyosis can benefit from topical and/or systemic retinoids, but these medications should be used in consultation with a dermatologist. Genetic counseling is essential for families of infants with ichthyosis.
shedding of keratinocytes. Infants with X-linked ichthyosis are frequently born after prolonged labor, because absence of steroid sulfatase can lead to increased fetal productions of DHEAS and decreased placental estrogen. Undescended testes, testicular cancer, and cataracts are associated with X-linked ichthyosis. Lamellar ichthyosis is rare and inherited in an autosomal recessive manner, although autosomal dominant forms have been described. Most infants with lamellar ichthyosis have mutations in the transglutaminase 1 gene. Transglutaminase is important in keratinocyte differentiation. Alterations in this protein produce the thick plate-like scale seen in infants with lamellar ichthyosis. Sometimes, infants with lamellar ichthyosis are born with a saran-wrap covering of skin referred to as a collodion membrane (see section on collodion membrane). New-borns with ichthyosis are primarily managed with emollients, such as vaseline and aquaphor. A thinner lotion may be preferable, if a thicker emollient is difficult to apply. Keratolytic emollients containing lactic and salicylic acid should be avoided, because they can be systemically absorbed. Infants with severe forms of ichthyosis can benefit from topical and/or systemic retinoids, but these medications should be used in consultation with a dermatologist. Genetic counseling is essential for families of infants with ichthyosis.
Ectodermal Dysplasia
Ectodermal dysplasia is a term that refers to conditions resulting in abnormalities of the skin and adnexal structures (teeth, hair, and nails). There are many different forms of ectodermal dysplasia. Hypohidrotic ectodermal dysplasia, hidrotic ectodermal dysplasia, keratosis ichthyosis and deafness (KID) are examples. Infants with ectodermal dysplasia are difficult to diagnose in the newborn period, because teeth are rarely present at birth, and most infants have sparse hair and thin nails. Unexplained fevers may be the first clue to hypohidrotic ectodermal dysplasia. There is no specific therapy for ectodermal dysplasia. Skin health should be maintained with mild soaps and meticulous applications of bland emollients. Families should be referred to a dermatologist and geneticist to assist in diagnosis and genetic counseling.
Netherton Syndrome
Netherton syndrome is a condition that causes progressive and diffuse cutaneous findings (Fig. 55-4). Newborns are frequently born prematurely and fail to thrive. Typically, there is erythroderma in the newborn period. Newborns with extensive skin barrier compromise are at risk for dehydration and sepsis. The rash can appear very similar to atopic dermatitis with significant involvement of the face and scalp. Infants develop sparse scalp hair as a result of hair fragility. Netherton syndrome is diagnosed after finding a characteristic hair shaft abnormality referred to as trichorrhexis invaginata (“bamboo hair” or “ball-and-socket hair”). Only 30% of hairs many be involved and typically trichorrhexis invaginata occurs after 3 months of age, making early diagnosis of Netherton syndrome difficult. There are no specific laboratory abnormalities, except most infants have elevated levels of immunoglobulin E (IgE). A mutation in SPINK5, encoding a serine protease inhibitor, (LEKTI) has been shown to cause Netherton syndrome in some patients (25). Patients are managed with topical corticosteroids, emollients and antihistamines. Tacrolimus and pimecrolimus should be used with caution in infants with Netherton syndrome, because high serum levels of tacrolimus have been detected in some infants. Serum levels of tacrolimus should be checked in infants treated with this medication. Special attention should be given to nutrition, because these patients tend to have problems with growth.
 Figure 55-4 Netherton syndrome. |
Metabolic and Nutritional Disorders
There are a number of metabolic diseases and nutritional deficiencies that can cause diffuse red dry flaky skin in the newborn period (26). Examples include maple syrup urine disease, carbamoyl phosphate synthetase deficiency, argininosuccinic aciduria, propionic acidemia, methylmalonic aciduria, cystic fibrosis, biotinidase deficiency (multiple carboxylase deficiency), and essential fatty acid deficiency. Acrodermatitis enteropathica (zinc deficiency) produces a unique rash and will be discussed in more detail.
Acrodermatitis Enteropathica
Acrodermatitis enteropathica (AE) is caused by zinc deficiency. This condition results from decreased zinc intake, poor gastrointestinal absorption and/or increase metabolic needs. Infants develop a tetrad of diarrhea, periorificial and acral vesiculobullous dermatitis, alopecia, and apathy (Fig. 55-5). Autosomal recessive AE is as a result of an altered gastrointestinal zinc transport mechanism and develops within the first several months of life. Usually, the dermatitis is the first sign. Acquired forms of AE are more common and result from low or absent levels of zinc in the breast milk, decreased gastrointestinal absorption (as a result of diarrhea), or increased metabolic requirements.
Absent or low zinc in the breast milk occurs because of altered transfer of zinc from maternal serum. Mothers usually have normal serum zinc levels. Breast-fed premature infants can develop symptoms when their nutritional requirements rise. Premature infants and those with HIV infection develop more severe disease. A low plasma zinc level (<65 mg/dL) is diagnostic, although AE can occur in the setting of normal zinc levels. Administration of zinc gluconate or sulfate at a dose of 5 mg/kg per day divided into two doses results in rapid improvement of symptoms in 2 to 4 days and may prevent death. Metabolic disorders such as cystic fibrosis may also produce a similar rash (27,28).
Absent or low zinc in the breast milk occurs because of altered transfer of zinc from maternal serum. Mothers usually have normal serum zinc levels. Breast-fed premature infants can develop symptoms when their nutritional requirements rise. Premature infants and those with HIV infection develop more severe disease. A low plasma zinc level (<65 mg/dL) is diagnostic, although AE can occur in the setting of normal zinc levels. Administration of zinc gluconate or sulfate at a dose of 5 mg/kg per day divided into two doses results in rapid improvement of symptoms in 2 to 4 days and may prevent death. Metabolic disorders such as cystic fibrosis may also produce a similar rash (27,28).
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