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A Case of Aplasia Cutis Congenita Associated with Epidermolysis Bullosa

Journal of the Korean Pediatric Society 1993;36(8):1171-1177.
Published online August 15, 1993.
A Case of Aplasia Cutis Congenita Associated with Epidermolysis Bullosa
Seung Jun Youn1, Gang Youl Bae1, Woo Sik Chung1, Kil Seo Kim1, Chul Hyon Ahn2
1Department of Pediatrics, Dae Dong Hospital, Pusan, Korea
2Department of Dematology, Dae Dong Hospital, Pusan, Korea
표피 수포증을 동반한 피부 형성 부전증 1례
윤승준1, 배강열1, 정우식1, 김길서1, 안철현2
1대동병원 소아과
2대동병원 피부과
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.
Key Words: Aplasia cutis congenita, Epidermolysis bullosa


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