Systematized epidermal nevi: case report and review of clinical manifestations

A 19-year-old woman developed papillary and pigmentary changes of the skin over her entire body with extensive eyelid involvement causing trichiasis. A four-lid blepharoplasty with rotation of the anterior lid lamella led to resolution of the ocular symptoms and secondary cosmetic improvement. Histologic examination of the tissue demonstrated epidermal nevi which are congenital abnormalities of surface or adnexal epithelium. This patient was diagnosed with systematized epidermal nevus as described by Lever's classification. There is a high association of epidermal nevi with other systemic abnormalities; that association is known as "epidermal nevus syndrome" (ENS).