White sponge nevus

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White sponge nevus
OMIM 193900
DiseasesDB 33866

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Overview

White sponge nevus, also known as Cannon's disease or Hereditary leukokeratosis of mucosa, appears to follow a hereditary pattern as an autosomal dominant trait. Although it is congenital in most cases, it can occur in childhood or adolescence.

Pathophysiology

It is caused by a mutation of the keratin 4[1] and keratin 13[2] genes.

White sponge nevus has an autosomal dominant pattern of inheritance.

Presentation

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.

Notes

  1. McGowan KA, Fuchs H, Hrabé de Angelis M, Barsh GS (2007). "Identification of a Keratin 4 mutation in a chemically induced mouse mutant that models white sponge nevus". J. Invest. Dermatol. 127 (1): 60–4. doi:10.1038/sj.jid.5700498. PMID 16858417. Unknown parameter |month= ignored (help)
  2. Terrinoni A, Rugg EL, Lane EB; et al. (2001). "A novel mutation in the keratin 13 gene causing oral white sponge nevus". J. Dent. Res. 80 (3): 919–23. PMID 11379896. Unknown parameter |month= ignored (help)

References

  • Kahn, Michael A. Basic Oral and Maxillofacial Pathology. Volume 1. 2001.

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