Abstract
White sponge nevus (WSN) is a rare inherited, autosomal dominant genodermatosis, which is characterized by white and corrugated plaques mainly affecting the oral mucosa. Mutations of keratin 4 and keratin 13 have been associated with WSN. This condition shows a high degree of penetrance and variable expressivity. Our report describes the case of a Hispanic family in which oral WSN lesions were seen in four generations. Clinical examination revealed that of their 18 descendants, 6 (33.3%) were afflicted. Of these, 3 were females and 3 were males. The most commonly affected sites were the buccal and labial mucosa. No extraoral lesions were noted. No history of consanguinity was found within the family. It was determined that both unaffected and affected members had transferred the disease via an autosomal dominant mode of inheritance with incomplete penetrance. This family was discovered after the proband initial consultation of an eight-year old girl.
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