Korean J Dermatol.
2001 Aug;39(8):955-958.
A Case of Hypohidrotic Ectodermal Dysplasia
- Affiliations
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- 1Department of Dermatology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. eslee@smc.samsung.co.kr
Abstract
- Hypohidrotic ectodermal dysplasia is a rare X-linked recessive disorder. It is characterized by the absence or hypoplasia of hair, teeth and sweat glands and by the characteristic facial features. A 20-year-old man presented with hypohidrosis and dry skin since birth. The skin was dry and his face had the characteristic appearance with prominent supraorbital ridges, a wide nasal bridge, thick everted lips and bat-like ears. His scalp and pubic hairs were sparse and eyebrows were absent. His family showed a typical pedigree of X-linked recessive trait. A skin biopsy from the left palm revealed no eccrine gland structures. Herein we report a typical case of hypohidrotic ectodermal dysplasia with a brief review.
