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Verruciform xanthoma
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Verruciform xanthoma

Contributors: Molly Weinberg MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Verruciform xanthoma (VX) is a xanthoma variant that commonly presents as a single verrucous, papillomatous, or keratotic plaque or nodule on the oral or anogenital mucosa. Other less common locations are the perioral area and the esophagus and other parts of the aerodigestive tract. The lesion is found most frequently in individuals between 40 and 60 years of age; however, there are exceptions. Men and women appear to be affected equally. VX is typically a painless, slow-growing lesion.

Oral VX has been seen in association with oral submucous fibrosis, lichen planus, leukoplakia, oral squamous cell carcinoma, oral chronic graft-versus-host disease, oral discoid lupus erythematosus, and oral pemphigus vulgaris. Extraoral VX has been recognized in lichen sclerosus, autosomal recessive dystrophic epidermolysis bullosa (RDEB), CHILD syndrome, lower extremity lymphedema, lymphangioma circumscriptum, and KID syndrome.

In CHILD syndrome, the cutaneous manifestations are termed CHILD nevi. Verruciform xanthomas have been reported to occur within CHILD nevi, especially those occurring within folds. Oral VX may also be seen. CHILD syndrome is caused by a mutation of the nicotinamide adenine dinucleotide phosphate steroid dehydrogenase-like (NSDHL) gene. This gene is needed for cholesterol synthesis and is the basis behind the theory of NSDHL gene mutations leading to VX lesions.

The cause of VX when not associated with CHILD syndrome is unclear. It has been suggested that epithelial compromise, secondary to local trauma or inflammation, leads to a release of epithelial lipids that are then taken up by macrophages. No evidence of human papillomavirus (HPV) has been found in VX.

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified
K13.4 – Granuloma and granuloma-like lesions of oral mucosa

SNOMEDCT:
254756007 – Verruciform xanthoma of skin

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Oral VX:
Anogenital VX:
Nongenital plaques:

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:03/06/2020
Last Updated:03/12/2020
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Verruciform xanthoma
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Verruciform xanthoma
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