White sponge naevus with minimal clinical and histological changes: report of three cases

摘要: White sponge naevus (WSN) is a rare autosomal dominant disorder that predominantly affects non-cornified stratified squamous epithelia: oral mucosa, oesophagus, anogenital area. It has been shown to be related keratin defects, because of mutations in the genes encoding mucosal-specific keratins K4 and K13. We illustrate three cases diagnosed as WSN, following clinical histological criteria, with unusual appearance. They presented minimal changes could misleading diagnosis. The patients showed diffuse irregular plaques range presentations from white rose coloured mucosae involving entire cavity. In one case lesion was also present vaginal findings included epithelial thickening, parakeratosis extensive vacuolization suprabasal keratinocytes, confirming WSN Clinical presentation histopathology are discussed relation differential diagnosis other leukokeratoses.