History

Patients usually present with a history of an asymptomatic or tender lesion on the skin or mucosa.

Physical Examination

The clinical appearance of a verruciform xanthoma is not diagnostic; the diagnosis is almost always made at histologic examination. Depending on the nature of the individual lesion, verruciform xanthoma may clinically resemble any verrucous, papillary, or lichenoid oral lesion, particularly any such lesion that is also hyperkeratotic. It is frequently misdiagnosed at clinical examination as a papilloma.

The lesion may appear as a verrucous, papillary, or flat-to-lichenoid process varying from 0.2-2 cm in diameter, although one lesion 4 cm in diameter has been reported. A giant verruciform xanthoma measuring 15 cm x 20 cm was reported in a 54-year old man with developmental delay.^[78]Depending on the degree of keratinization, the xanthoma may appear gray to reddish pink. The process may be pedunculated but usually is sessile. At least two cases occurring in cysts have been reported.

Although almost any part of the mouth may be involved, the alveolar ridge, hard palate, and gingiva have been noted as preferential sites.^[79]Because of this predilection, inflammation caused by mild trauma has been proposed to play a role in the etiopathogenesis.^{[28, 41]}A second hypothesis suggests that the verrucous and papillary epithelial architecture may be secondary to the presence of foamy cells, which affect the metabolism of the epithelial cells, leading to hyperkeratotic change.^{[28, 80]}

The lesions that occur on the oral mucosa or scrotum are usually not associated with any predisposing disorder.

The lesions that occur on the feet are often associated with lymphedema.

The skin lesions that occur outside of the perineum or those that are multiple are often associated with another disease process.

Examples include several cases of epidermal nevi and congenital hemidysplasia with ichthyosiform erythroderma and limb defect (CHILD) syndrome,^{[81, 82]}in which verruciform xanthomata may be the only ostensible sign,^[83]and one case each of chronic eczema with ichthyosis, chronic severe sun damage, recessive dystrophic epidermolysis bullosa,^[84]and discoid lupus erythematosus.^[85]Lesions have also been found in the oral cavity in association with lichen planus,^{[59, 86]}pemphigus vulgaris,^[87]oral bullae, carcinoma in situ,^[88]or frank squamous cell carcinoma^{[46, 89]}; the lesions were also found in a bone marrow transplant recipient.^[90]

Complications

Verruciform xanthoma can be easily cured by surgical excision without complications. However, if an underlying disease is present, such as the CHILD syndrome (see above), this must be managed. Two cases of verruciform xanthoma associated with squamous cell carcinoma have been reported, so careful scrutiny of the histopathology for associated keratinocyte atypia is warranted, especially in lesions associated with penile phimosis.^{[1, 46]}

Differential Diagnoses

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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, The Scientific Research Honor Society, Society for Investigative Dermatology, Xeroderma Pigmentosum Society

Disclosure: Nothing to disclose.

Coauthor(s)

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 income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>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 these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

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<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

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

Disclosure: Nothing to disclose.

Verruciform Xanthoma Clinical Presentation

History

Physical Examination

Complications

References

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