Korean J Dermatol.
1995 Oct;33(5):961-966.
A Case of Hypohidrotic Ectodermal dysplasia
Abstract
- Hypohidrotic ectodermali dysplasia is a rare and heterogenous genodermatosis that is characterzed by hypohidrosis, hyptrichosis, dental hypoplasia, and a characteristic facies. A 16-month-old male was evaluated for anodontia and anhidrosis since birth. There was no family history of this disease. His face was characterized by a broad nose and sparse eyebrows and periorbital wrirkling. The skin was dry, and scalp hairs were thin and sparse. Skull X-ray finding revealed noth ing remarkable except de;ayed teeth eruption. His developmental status for which we used DDS'I' was slightly reduced on inental development and showed normal on physical development. A skin biopsy was done on skin from the left palm, which showed a few immature eccrine glands in the dermis. We report a typical form of hypohidrotic ectodermal dysplasia with a review of the literature.
