Verruciform Xanthoma: Background, Pathophysiology, Epidemiology

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Sections Verruciform Xanthoma
Overview
Presentation
- History
- Physical
- Causes
- Complications
- Show All
DDx
Workup
Treatment
References

Overview

Background

Verruciform xanthoma is an uncommon lesion with a predilection for the oral mucosa of middle-aged persons or on the scrotum of middle‒aged-to-elderly Japanese men. The most common site for verruciform xanthoma is the oral mucosa. Extraoral verruciform xanthoma is extremely uncommon; it has been reported on the anogenital skin such as the vulva, scrotum, penis, and extremities.^{[1, 2]}Lesions on the perineum or on the skin often have some predisposing factor, such as lymphedema^[3]or an epidermal nevus.^{[4, 5]}Verruciform xanthoma can also occur in association with CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) syndrome.

Most commonly, the lesion has a verruciform appearance, but it may appear polypoid, papillomatous, or sessile. Rarely, verruciform xanthomas have been found to occur in cysts. The primary distinguishing feature of verruciform xanthoma is the presence of large numbers of lipid-laden foamy histiocytes in, and essentially limited to, the connective-tissue papillae in the lesion.

Note the image below.

Xanthelasma. Courtesy of Duke University Medical Center.

Pathophysiology

The pathophysiology of verruciform xanthoma remains unknown.

Frequency

Verruciform xanthoma is uncommon, with a frequency of 0.025-0.095%.^{[6, 7, 8]}To date, at least 300 cases have been reported in the oral cavity,^{[8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18]}seven on the lip,^{[19, 20, 21]}24 on the penis,^{[1, 2, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43]}20 on the vulva,^{[29, 44, 45, 46, 47, 48, 49, 50, 51, 52]}one in the anal region,^[53]three on the nose or nasal vestibule,^{[54, 55]}two on the ear,^[56]four on the distal extremities (including three on the feet),^[57]and individual cases elsewhere on the skin. At least 23 cases have been reported on the scrotum, almost all of them in Japanese men.^{[58, 59, 60]}Three cases of esophageal verruciform xanthoma have been reported.^{[29, 61, 62, 63]}A single case of multiple verruciform xanthoma lesions has been reported in internal organs; the lesions were in the upper aerodigestive tract of a child with a systemic lipid storage disease.^[64]

Race

Most cases of verruciform xanthoma are reported in whites, but blacks are also affected.^[65]Most scrotal cases of verruciform xanthoma occur in middle‒aged-to-elderly Japanese men. This may be caused by chronic pressure associated with the Japanese tradition of sitting on the floor.^[59]

Sex

Verruciform xanthoma has a slight male predominance,^{[6, 66]}and it also has a tendency to occur on the scrotum of middle‒aged-to-elderly Japanese men.

Age

Most oral cases of verruciform xanthoma occur in middle-aged persons (mean age, 40-60 y), and most scrotal cases occur in middle‒aged-to-elderly Japanese men (mean age, 65 y). However, the age at occurrence varies widely; verruciform xanthoma can occur in persons aged 2.5-89 years.^[51]

Verruciform xanthoma is exceedingly rare in children aged 10 years or younger, except in epidermal nevi or in the context of CHILD syndrome.

Prognosis

The prognosis for verruciform xanthoma is excellent after local surgical excision, and verruciform xanthoma typically does not require medical, chemical, or radiological treatment after surgery.^[67]Recurrence is rare.

Patient Education

Patients should be reassured after adequate excision and accurate diagnosis that verruciform xanthoma is cured.

Clinical Presentation

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Media Gallery

Xanthelasma. Courtesy of Duke University Medical Center.

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Author

W Clark Lambert, MD, PhD Clinical Professor of Pathology, Clinical Professor of Medicine (Professor of Dermatology), Rutgers New Jersey Medical School; Professor of Pathology, Rutgers Graduate School of Biomedical Sciences

W Clark Lambert, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American Dermatological Association, American Federation for Clinical Research, American Society of Dermatopathology, College of American Pathologists, Dermatological Society of New Jersey, Institute of Electrical and Electronics Engineers, International Academy of Pathology, International Federation of Pigment Cell Societies, International Society of Dermatopathology, Medical Society of New Jersey, Sigma Xi, Society for Investigative Dermatology, Xeroderma Pigmentosum Society

Disclosure: Nothing to disclose.

Coauthor(s)

Peter C Lambert, MS St George's University School of Medicine, Grenada

Disclosure: Nothing to disclose.

Hong Li, MD Pathologist and Dermatopathologist

Hong Li, MD is a member of the following medical societies: American Society for Clinical Pathology, College of American Pathologists

Disclosure: Nothing to disclose.

Fernanda Salgado Rutgers New Jersey Medical School

Fernanda Salgado is a member of the following medical societies: American Medical Association, Latino Medical Student Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD.

Sections Verruciform Xanthoma
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Presentation
- History
- Physical
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DDx
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