White sponge nevus presenting in the esophagus--case report and literature review

White sponge nevus (WSN) is a rare inherited disorder which usually presents as non-painful white plaques primarily involving the buccal mucosa, gingiva and palate. Extra-oral lesions most often occur in the esophagus or anogenital area but almost invariably follow the development of typical buccal lesions. We present a non-familial case in which the first manifestation of the disease was in the esophagus in the absence of oral lesions. This sequence of events has not previously been reported in the literature. The cytohistological and electron microscopical appearances which allow the recognition and differentiation of WSN from other conditions presenting as esophageal white lesions are discussed.