White sponge nevus is a rare, inherited disorder that usually presents as nonpainful white plaque primarily involving the buccal mucosa, gingiva, and palate. Extraoral lesions most often occur in the esophagus or anogenital area, but almost invariably follow the development of typical buccal lesions. This article presents a familial case of white sponge nevus in which oral lesions were found in patients in three generations of the same family.
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