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Herditary Anhidrotic Ectodermal Dysplasiain Twins

Journal of the Korean Pediatric Society 1996;39(7):1005-1009.
Published online July 15, 1996.
Herditary Anhidrotic Ectodermal Dysplasiain Twins
Myung Gil Han, Do Hyun Kim, Hyung Ro Moon
Department of Pediatrics, EUL JI Medical Center, Seoul, Korea
쌍생아에서 발생한 선천성 외배엽 이형성증
한명길, 김도현, 문형로
서울 을지병원 소아과
Abstract
Hereditary anhidrotic ectodermal Dysplasia is a congenital disease displaying characteristics of anhidrosis, hypotrichosis and dental defect which are caused by developmental anomaly of ectodermal epidermis and its appendages. We experienced two cases of hereditary anhidrotic ectodermal dysplasia in two-year and four-month old twin brothers. These patients suffered from intermittent high fever early in life which brought them to our clinical attention. However the diagnosis of anhidrotic ectodermal dysplasia was not suspected by means physicians who cared the patients previously. The diagnosis was made on the basis of clinical features, and confirmed by starch iodine sweat test and skin biopsy on the palm and axilla. We report the two cases in a twin brothers with brief review of related literatures.
Key Words: Anhidrotic ectodermal dysplasia, Twins


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