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May 1967


Arch Dermatol. 1967;95(5):548-553. doi:10.1001/archderm.1967.01600350110021

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Congenital Ichthyosiform Erythroderma. Presented by the University Hospital Staff.  A 16-month-old white girl was noted at birth to have a generalized erythematous ichthyosiform dermatosis. The eruption has become associated with sparseness of scalp hair, dystrophy of the nails, and poorly formed teeth. The case has been diagnosed variously as congenital ichthyosiform erythroderma, incontinentia pigmenti, and congenital ectodermal defect. Growth has been slower than average but mental development has not been effected. The history was negative for familial diseases or consanguinity. There was one sibling, a normal 8-year-old boy.Examination showed a fairly pronounced erythematous ichthyosiform and hyperkeratotic dermatosis on the face, scalp, right side of the trunk, and the extremities. The involvement of the extremities was more prominent on the right side and flexural in distribution. Erythema and hyperkeratosis of the palms and soles was marked. The nail plates were dystrophic and many had subungual hyperkeratosis.The only significant laboratory

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