Necrobiotic xanthogranuloma (NXG) is an uncommon granulomatous disease involving the and extracutaneous tissues. In its typical clinical presentation, it consists of multipleindurate yellow–brownish plaques or nodules, usually located on the periorbital area. Thelesions often are bilateral and symmetric and frequently show tendency to atrophy and ulceration. Most cases are asymptomatic, and oral mucosal erosions are sometimespresent. Ophthalmologic complications are observed in many patients, and NXG mayinvolve other extracutaneous sites. Paraproteinemia is closely associated with NXG, demonstratinga monoclonal gammapathy, and other hematologic or lymphoproliferative disordersalso may appear in association with NXG. Large areas of degenerated collagenalternating with granulomatous inflammation, multinucleate giant cells, cholesterol clefts, and lymphoid follicles are the most outstanding histopathologic characteristics of NXG. The prognosis for NXG is good, but no curative treatment is available. Chemotherapy withalkylating agents is the most frequently administered treatment, but oral and intralesional corticosteroids, interferon alpha, plasmapheresis, surgical excision, and radiation therapy also have been used for treatment of this disorder, with variable results.
Semin Cutan Med Surg 26:108-113 © 2007 Elsevier Inc. All rights reserved.