Epidermal Nevus Syndrome and Didymosis Aplasticosebacea


Address correspondence to Dr. Svetlana Kavaklieva, Department of Dermatology and Venereology, Medical University, 1st, G. Sofijski Str., Sofia, Bulgaria, or e-mail: kavaklieva@abv.bg.


Abstract:  The epidermal nevus syndrome is a disease complex consisting of the association of an epidermal nevus with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular, and urogenital systems. The epidermal nevi are classified according to their predominant component; nevus sebaceus (sebaceous glands), nevus comedonicus (hair follicles), and nevus verrucosus (keratinocytes). We report a neonate who presented with a nevus sebaceus on the scalp and face as well as a coloboma and dermoid on his left eye. Within the sebaceous nevus on the scalp, circumscribed lesions of aplasia cutis congenita were detected, which is consistent with the recently proposed term in the literature didymosis aplasticosebacea.