All issues > Volume 36(8); 1993

Case Report

J Korean Pediatr Soc. 1993;36(8):1171-1177. Published online August 15, 1993.

A Case of Aplasia Cutis Congenita Associated with Epidermolysis Bullosa

Seung Jun SJ Youn¹, Gang Youl GY Bae¹, Woo Sik WS Chung¹, Kil Seo KS Kim¹, Chul Hyon CH Ahn²

¹Department of Pediatrics, Dae Dong Hospital, Pusan, Korea
²Department of Dematology, Dae Dong Hospital, Pusan, Korea

Abstract

Alpasia cutis congenita is an anomaly characterized by absence of localized areas of the integument. The most common type of aplasia cutis congenita is Aplasia cutis congenita limited to the scalp, although other areas of the body may also be involved. We experienced a case of aplasia cutis congenita in a male newborn infant. The skin defects were extensive with symmetrical involvement of lower extremities. The multiple bullae were found on the both fingers and toes. No similar conditions and other associated congeital anomalies were found in the family membes of this particular case. The light microscopic examination of the denuded skin areas how absence of epidemis and the demis contain atrophic and hypoplastic adnexa. The bullae have a split within the dermis below lamina densa on electron microscopy. The skin defects were healed by supportive therapy for 4weeks.

Keywords :Aplasia cutis congenita, Epidermolysis bullosa