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Nevus spongiosus albus mucosaeD10.31

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 02.05.2022

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Synonym(s)

Mucosal nevus whiter; OMIM 193900; White folded gingivostomatosis; white mucosal nevus; White mucosal nevus; white sponge nevus

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HistoryThis section has been translated automatically.

Cannon, 1935

DefinitionThis section has been translated automatically.

Nevus spongiosus albus mucosae is a rare (harmless), autosomal-dominant inherited keratinization disorder affecting the non-keratinized, stratified squamous epithelium of the mucous membranes. The oral mucosa is mainly affected. More rarely, the anal and vaginal mucosa and the nasal mucosa are also affected to varying degrees.

EtiopathogenesisThis section has been translated automatically.

Autosomal dominant inheritance; causative mutations are present in the KRT13 and KRT4 genes, which are mapped on chromosomes 17q21-q22 and 12p11.2-q11, respectively (nucleotide substitution from G to A in position 1345 of the KRT4 gene). To date, 6 mutations have been detected. The mutations lead to the formation of defective keratin proteins and consequent disruption of keratin filatments (Westin M et al. 2018).

ManifestationThis section has been translated automatically.

Occurring from birth or in early childhood.

LocalizationThis section has been translated automatically.

Mainly oral mucosa, palate, tongue edges, also lips, anal or vaginal mucosa.

Clinical featuresThis section has been translated automatically.

Two-dimensional, leukoplakic, mostly blurred, sometimes sharply defined white coloration, possibly with small erosions of the mucosa.

HistologyThis section has been translated automatically.

Significant acanthosis, intra- and extracellular edema with vacuolisation of epithelial cells, parakeratosis, inflammatory infiltrate. Epidermolytic hyperkeratosis has also been described.

Differential diagnosisThis section has been translated automatically.

Oral leukoplakia.

TherapyThis section has been translated automatically.

Exclusion of malignancy (see below leukoplakia) by means of punch biopsy. No invasive therapy necessary, as there is no risk of degeneration. In case of cosmetic disorder possibly cryosurgery, electrocoagulation or laser therapy with ablative laser.

Progression/forecastThis section has been translated automatically.

Cheap. The changes of the oral mucosa reach their maximum expression in the 2nd decade of life. After that they do not change any more.

LiteratureThis section has been translated automatically.

  1. Aloi FG et al (1988) White sponge nevus with epidermolytic changes. Dermatologica 177: 323-326
  2. Cannon AB (1935) White sponge nevus of the mucosa (nevus spongiosus albus mucosae). Arch Dermatol Syph 31: 365
  3. Chao SC et al (2003) A novel mutation in the keratin 4 gene causing white sponge nevus. Br J Dermatol 148: 1125-1128.
  4. Mostaccioli S et al (1997) White sponge nevus is caused by mutations in mucosal keratins. Eur J Dermatol 7: 405-408
  5. Schaarschmidt H et al (1992) Nevus spongiosus et albus mucosae. Dermatol 43: 35-37.

  6. Westin M et al (2018) Mutations in the genes for keratin-4 and keratin-13 in Swedish patients with white sponge nevus. J Oral Pathol Med 47:152-157.

Authors

Last updated on: 02.05.2022

Author: Prof. Dr. med. Peter Altmeyer