XANTHOMA DISSEMINATUM

By Gary M. White, MD

Xanthoma Disseminatum Courtesy Department of Dermatology, University of California, Irvine


Xanthoma disseminatum (XD), also known as Montgomery syndrome, is a rare, benign, mucocutaneous xanthomatosis which represents a non-Langerhans cell histiocytosis.

Clinical

Xanthoma disseminatum presents as orange to red to mahogany-brown papules and nodules symmetric in both axilla and on the flanks typically in a young adult. Lesions are symmetric and the preferred sites of involvement in decreasing order are axilla, eyelids, groin, neck, trunk, and face. Perianal tumors may interfere with defecation.

Treatment

This disease is benign but the course is chronic. Most therapies fail. Although the lipid profile is normal, clofibrate has been used with partial success. Oral corticosteroids and antimitotic drugs may be tried but usually fail.  Cyclophosphamide was beneficial in one patient. Multiple cycles of cladribine and arabinoside (Ara-C) were successful in another patient [British J Dermatology 2014 May]. In another case report, a patient was treated with a combination of rosiglitazone 4 mg daily, simvastatin 10 mg daily, and fenofibrate 200 mg daily treatment. Within a year, his lesions improved 50% [Ann Dermatol. 2012 Aug;24(3):380-382].

Additional Pictures

Courtesy Department of Dermatology, University of California, Irvine
Xanthoma Disseminatum

References

A 62-year-old man presented with skin changes on the face, flexors, and trunk for 30 years. Exam showed hundreds of red to brown papules were distributed symmetrically on axilla, genitalia, poplitea, trunk and nasal mucosa. Case Reports in Dermatologic Medicine

A 37-year-old Asian male presented with 5-year history of numerous asymptomatic yellow-brownish papules distributed on face and flexural areas. His lipid levels were normal. He also had diabetes insipidus. Ann Dermatol. 2012 Aug;24(3):380-382

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