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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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Collodion Baby
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Newborn with the most severe form of collodion baby.

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Collodion Baby
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Generalized scaling of the skin (shoulder area shown) leads to the collodion-like aspect in a collodion baby. The underlying skin is red but otherwise normal.

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

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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.

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

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

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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.

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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).

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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.

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