All issues > Volume 37(10); 1994

Case Report

J Korean Pediatr Soc. 1994;37(10):1453-1456. Published online October 15, 1994.

A Case of Hereditary Anhidrotic Ectodermal Dysplasia

Jong Won JW Lee¹, Jin Kyung JK Jung¹, Jin Gun JG Bang¹, Jing Sam JS Rho¹, Jung Hee JH Park²

¹Department of Pediatrics, St. Francisco General Hospital, Seoul ,Korea
²Department of Pathology, St. Francisco General Hospital, Seoul ,Korea

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.

Keywords :Hereditary, Ectodermal, Dysplasia, Anhidrosis