White sponge nevus of the vulva.

The white sponge nevus is a hereditary leukokerotosis localized preferably in the oral mucosa, but may simultaneously manifest itself in other regions, e.g. perianally. We report the case of a 18-year-old patient with a primarily and exclusively extraorally located white sponge nevus of the vulva. The differential diagnosis and clinical as well as therapeutical problems of this extremely rare disease are discussed.