White sponge nevus is a rare genetic condition inherited in most cases as an autosomal dominant trait caused by mutations in either the keratin 4 or keratin 13 genes. Lesions may be evident at birth or early in childhood, but occasionally appear in the second decade.
Asymptomatic diffuse white plaques affect the buccal mucosa bilaterally. The degree of involvement varies considerably. Other oral mucosal sites of involvement may include ventral tongue, labial mucosa, floor of mouth and soft palate. Less commonly, other mucosal sites (anogenital, esophageal, nasal, laryngeal) may be affected.
White sponge nevus of the oral mucosa - Oral Mucosal Lesion
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Synopsis
Codes
ICD10CM:
Q38.6 – Other congenital malformations of mouth
SNOMEDCT:
389203001 – White sponge nevus of mucosa
Q38.6 – Other congenital malformations of mouth
SNOMEDCT:
389203001 – White sponge nevus of mucosa
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Differential Diagnosis & Pitfalls
- Leukoplakia, especially proliferative verrucous leukoplakia – This may be white and rough-looking but usually does not have a symmetric distribution. The plaques are usually denser.
- Lichen planus – This is often white and bilaterally located in a symmetric fashion, but it is usually associated with pain, sensitivity, and reticulations.
- Aspirin burn will have a history of aspirin tablet applied to mucosa.
- Contact stomatitis is usually symptomatic and resolves after discontinuing the offending agent.
- Leukoedema – Disappears when stretched.
- Chronic cheek chewing – This may appear as painless, shaggy white plaques that are bilateral; the buccal mucosa may also be involved. Patients are not usually immunocompromised, although they may coincidentally be so. A biopsy distinguishes between the two.
- Candidiasis – This may appear similar but tends not to be bilaterally symmetrical and not to primarily affect the lateral tongue. The plaques scrape off with some difficulty, leaving a raw, bleeding surface. Often, papules will be present elsewhere on the oral mucosa.
- Oral hairy leukoplakia
- Hairy tongue (overgrowth and retention of filiform papillae) – This generally involves the dorsum of the tongue only, where filiform papillae are located.
- Hereditary benign intraepithelial dyskeratosis – This has characteristic bulbar conjunctival lesions that form in the spring.
- Pachyonychia congenita has characteristic nail changes.
- Dyskeratosis congenita is usually associated with hematologic abnormalities.
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Last Updated:01/25/2022
White sponge nevus of the oral mucosa - Oral Mucosal Lesion