Collodion Baby

An inherited syndrome apparent at birth and present throughout life. The newborn is born encased in a collodion-like membrane that sheds within the next 12 weeks, revealing generalized scaling with variable redness of the skin. Frequent consequences are life-threatening sepsis and dehydration by protein and electrolyte loss.

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Ichthyosis Congenita; Lamellar Exfoliation of Newborn; Desquamation of Newborn; Collodion Fetus.

The term was first used by Arnold M. Seligman, an American biochemist, in 1841. It is a descriptive term for infants born encased in a membrane-like thick scale rather than a disease entity on its own. Among this group of keratinizing disorders are several heterogeneous conditions including nonbullous congenital ichthyosiform erythroderma and lamellar ichthyosis.

Estimated 1:300,000 live births. Approximately 300 cases have been described in the literature.

Autosomal recessive inheritance. The gene is located on 14q11.2.

The pathogenesis of “collodion baby” has not been clarified, but there is increased epidermal germinal cell activity and a failure of stratum corneum cells to separate.

Characteristic clinical picture. Histopathologic examination of skin biopsies shows a compact, hyperkeratotic stratum corneum and normal underlying epidermis. In the second stage of the disease (i.e., after shedding of the collodion membrane), the etiologic diagnostic approach should consist of a thorough family history (including parental consanguinity), a complete clinical examination (inspection of the skin, other associated findings), histology of the skin and hair (e.g., trichothiodystrophy, trichorrhexis invaginata), and finally laboratory tests (biochemical/metabolic screening, mutation screening).

Collodion babies are frequently born premature. The condition may be self-limiting with shedding of the parchment-like membrane within the first 1 to 4 weeks (maximally 12 weeks) of life, however, the majority of children eventually progress to a form of ichthyosis (i.e., congenital erythrodermic ichthyosis in 48%, lamellar ichthyosis in 12%, and dominant ichthyosis vulgaris in 10%), whereas only 10% of the patients eventually develop normal skin. Often the clinical course is complicated by fungal and bacterial skin infections, that may result in sepsis. Hands, feet, and the conjunctivae are the favorite sites of infections. High humidity and application of nonocclusive lubricants may facilitate shedding of the membranes. Other complications in the neonatal period include severe hypothermia, dehydration, and hypernatremia. Ectropion and/or eclabium, flattened ears and nose, and characteristic fixation of the lips in an O-shaped form are additional clinical findings. Respiratory distress frequently occurs. Mortality now is below 10%, but the condition remains serious.

Assess hydration state: Transepidermal water loss may be significant and lead to severe hypernatremic dehydration and hypoproteinemia (which may be masked by hypovolemia). Intravenous fluid therapy and maintenance of a temperature-regulated environment (incubator) to prevent hypovolemia and hyperthermia or hypothermia may be necessary. An up to sevenfold increase in fluid losses has been reported when compared to healthy infants. Assess for signs of infection/sepsis, commonly involving the lungs. Preoperative laboratory investigations should include a complete blood count with white cell differentiation, serum levels of urea, creatinine, and electrolytes. A chest radiograph should be obtained.

These patients have an increased morbidity (and mortality) as a result of impaired barrier function of the skin. Thermoregulation is impaired. On the one hand, hyperthermia commonly occurs if the ambient temperature is set too high. On the other hand, hypothermia may occur with excessive transcutaneous water losses. Venous access often is difficult in the presence of the skin changes. Fixation of the endotracheal tube probably is best done with a band of gauze than with adhesive tape. Ectropion requires careful protection and lubrication of the eyes. A poor nutritional status is often present with negative nitrogen balance and lower serum protein and albumin levels. Strict perioperative asepsis is mandatory because intravenous catheters are a well-known risk factor for severe infections in these children.

Hypovolemia, hypoalbuminemia, and a reduction in renal blood flow may affect the pharmacokinetics and pharmacodynamics of drugs.

Harlequin Syndrome: Congenital skin disease in which the skin builds up and scales (“fish skin disease”). Associated with a very poor prognosis, with death generally occurring in the first week of life.