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Journal of the Korean Pediatric Society 1994;37(10):1453-1456.
Published online October 15, 1994.
A Case of Hereditary Anhidrotic Ectodermal Dysplasia
Jong Won Lee1, Jin Kyung Jung1, Jin Gun Bang1, Jing Sam Rho1, Jung Hee Park2
1Department of Pediatrics, St. Francisco General Hospital, Seoul ,Korea
2Department of Pathology, St. Francisco General Hospital, Seoul ,Korea
무한형의 유전성 외배엽 이형성 1례
이종원1, 정진경1, 방진건1, 노진삼1, 박정희2
1방지거 병원 소아과
2방지거 병원 해부병리과
Abstract
Hereditary anhidrotic ectodermal dysplasia is a rare condition characterized by underdeveloped ectodermal structure including the skin, teeth or skin appendages. The patient has characteristic featureof anhidrosis, hypotrichosis and defective dentition. We experienced a case of hereditary anhidrotic ectodermal dysplasia in a 1-month-old male infant who had unexplained recurring fever, anhidrosis and characteristic facial feature, so we established the diagnosis with clinical feature and skin biopsy.
Key Words: Hereditary, Ectodermal, Dysplasia, Anhidrosis


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