A Case of Hypohidrotic Ectodermal dysplasia.
- Author:
Jin Hwan KIM
;
Mu Hyoung LEE
;
Choong Rim HAW
- Publication Type:Case Report
- Keywords:
Hypohidrotic Fictodermal Dysplasia
- MeSH:
Anodontia;
Biopsy;
Dermis;
Eccrine Glands;
Ectodermal Dysplasia 1, Anhidrotic*;
Eyebrows;
Facies;
Hair;
Humans;
Hypohidrosis;
Infant;
Male;
Nose;
Parturition;
Scalp;
Skin;
Skull;
Tooth
- From:Korean Journal of Dermatology
1995;33(5):961-966
- CountryRepublic of Korea
- Language:Korean
-
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.
