Thursday, December 17, 2015

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ichthyosiform dermatoses

The ichthyosiform dermatoses are a heterogeneous group of disorders presenting with excessive scaling of the skin. The inherited forms of the ichthyoses are most common, although the condition can occur secondary to other diseases.



A, Grandfather and granddaughter with ichthyosis vulgaris. B, Palmar hyperkeratosis, a finding often associated with ichthyosis vulgaris. C, X-linked ichthyosis, showing characteristic coarse, brown
scales. D, Young child with congenital ichthyosiform erythroderma demonstrating diffuse erythema and scale.

collodion baby

A collodion baby is a newborn infant whose skin looks like a “baked apple,” with a shiny, tough, membrane-like covering. This term describes a phenotype that occurs in several types of ichthyosis. Although congenital ichthyosiform erythroderma is the most common underlying condition (Fig. 4-1D), lamellar ichthyosis, Netherton’s syndrome, Conradi’s syndrome and others may also present as a collodion baby. Collodion babies may also go on to have normal skin. These infants are at increased risk for infections and fluid and electrolyte imbalances due to cutaneous fissures and impaired barrier function of the skin. Treatment in a high-humidity environment with frequent application of
petrolatum allows gradual sloughing of the collodion membrane.
Manual debridement and keratolytics are not recommended.

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