White sponge nevus

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White sponge nevus

ORPHA:171723Disease

Also called Hereditary mucosal leukokeratosis · White sponge nevus of Cannon

What it is

White sponge nevus (WSN) is a rare and autosomal dominant genetic disease in which the oral mucosa is white or greyish, thickened, folded, and spongy. The onset is early in life, and both sexes are affected equally. Other common sites include the tongue, floor of the mouth, and alveolar mucosa.

Key facts

Inheritance
Autosomal dominant
Classified as
Disease

Genes

KRT13Disease-causing germline mutation(s)
KRT4Disease-causing germline mutation(s)

ICD-10 codes

Q38.6filed under a broader ICD-10 category — shared with 1 other rare disease

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

GARD 8501MEDDRA 10072666MESH D053529MONDO 0015748OMIM 193900OMIM 615785UMLS C1721005

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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