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 |
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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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