Korean J Dermatol.
1979 Aug;17(4):253-257.
A Case of Hidrotic Ectodermal Dysplasia
Abstract
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Congenital ectodermal dysplasia are a group of familial disorders that affect tissues and organs of ectodermal origin to varying degrees. It devided hidrotic type and anhidrotic type by geneologic origin and distinct clinical manifestationa. The hidrotic type usually trait by autosomal dominant gene and manifested dystrofic nails, defect of hair and eyebrow and palmoplantar hyperkeratosis. This patient is 16 year oId male. He had been dystrophic nails, alopecia since at birth, thereafter palmoplantar hyperkeratosis, clubbing fingers noted. C.R.C. Urinalysis, stool examination, chest x-ray were within normal limit. Biopsy specimen from palm revealed hyperkeratosis, hypergranulosis, acanthosis and, mild inflamrnatory cell infiltrate on upper dermis. Biopsy speeimen from the scalp revealed norrriaI findings except slight hypoplasia of ebaceaus gland.