J Korean Pediatr Soc.  1993 Aug;36(8):1171-1177.

A case of aplasia cutis congenita associated with epidermolysis bullosa

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 examinaton 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.

Keyword

Aplasia cutis congenita; Epidermolysis bullosa

MeSH Terms

Dermis
Ectodermal Dysplasia*
Epidermolysis Bullosa*
Fingers
Humans
Infant, Newborn
Lower Extremity
Male
Microscopy, Electron
Scalp
Skin
Toes
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