| White sponge nevus | |
|---|---|
| Classification and external resources | |
| ICD-10 | D10 (ILDS D10.310) |
| ICD-9 | 750.26 |
| OMIM | 193900 |
| DiseasesDB | 33866 |
White sponge nevus (WSN), also known as Cannon's disease, Hereditary leukokeratosis of mucosa and White sponge nevus of Cannon,[1][2] is an autosomal dominant[3] skin condition.[4] Although congenital in most cases, it can first occur in childhood or adolescence.
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It presents in the mouth, most frequently as a thick bilateral white plaque with a spongy texture, usually on the buccal mucosa, but sometimes on the labial mucosa, alveolar ridge or floor of the mouth. The gingival margin and dorsum of the tongue are almost never affected.
Although this condition is perfectly benign, it is often mistaken for leukoplakia. There is no treatment, but because there are no serious clinical complications, the prognosis is excellent.
WSN is caused by a mutation of the keratin 4 or keratin 13 genes,[3][5] located respectively at human chromosomes 12q13[6] and 17q21-q22.[7] The condition is inherited in an autosomal dominant manner.[3] This indicates that the defective gene responsible for a disorder is located on an autosome (chromosomes 12 and 17 are autosomes), and only one copy of the defective gene is sufficient to cause the disorder, when inherited from a parent who has the disorder.
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