A rare association of epidermal nevus syndrome and ainhum-like digital constrictions.

Epidermal nevus syndrome is an unusual occurrence of epidermal nevi with central nervous system (CNS), eye, and skeletal developmental abnormalities. We report an as yet undocumented association of generalized epidermal nevus with striate keratoderma, ainhum-like digital constriction, and autoamputation of the right fifth toe in a 10-year-old girl. The excellent response to oral acitretin with softening of the constrictions is also highlighted.