An Autosomal Genetic Disease: White Sponge Nevus

Abstract

White sponge nevus is a rare autosomal dominant genetic disease of the oral mucosa. The lesion is white or greyish, thickened, folded and spongy in nature. The genes related with WSN involve mutant cytokeratin keratin 4 (KRT4) and keratin 13 (KRT13). It was also called as familial white folded dysplasia. The condition principally affects labial mucosa, ventral tongue, soft palate, alveolar mucosa, and floor of the mouth, and, less commonly, extra oral sites are the nasal, esophageal, laryngeal, and anogenital mucosa; but not the skin.

White Sponge Nevus of the Oral Cavity: Affecting Members of Two Generations in a Family

“…Prominent hyperkeratosis, marked acanthosis, spongiosis, cleared cytoplasm with perinuclear eosinophilic condensation are a common histological characteristic feature. 6 Histological findings are characteristics but not pathognomonic to the lesion, also our patient didn't agree for biopsy so it was not performed. The clinical appearance of WSN may have overlapping features with a variety of oral lesions that have varied clinical behaviors and treatment protocols, hence it is important to distinguish.…”