[Extraoral manifestation of a white sponge nevus of the mucosa].

The nevus spongiosus albus mucosae (resp. white sponge nevus in the angloamerican literature) is a hereditary leukokeratosis localised 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 nevus spongiosus albus mucosae of the vulva. The differential diagnosis and clinical as well as therapeutical problems of this extremely rare disease are discussed.