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Successful Treatment of Necrobiotic Xanthogranuloma With Intravenous Immunoglobulin

2010; American Medical Association; Volume: 146; Issue: 9 Linguagem: Inglês

10.1001/archdermatol.2010.236

1538-3652

Christine Hallermann,

Infectious Disease Case Reports and Treatments

Necrobiotic xanthogranuloma (NXG) is a rare systemic disease first described by Kossard and Winkelmann in 1980, and about 100 cases have been reported since then. It is clinically characterized by indurated yellowish to red-orange or brown papules or nodules that grow into larger and in some cases very extensive plaques covering the face (especially with periorbital distribution), trunk, and extremities. Lesions are nearly always asymptomatic, but secondarily they may become ulcerated. Moreover, pathologic changes in internal organs have been documented, including giant-cell myocardial disease. Because involvement of the heart seems to be relatively frequent, routine echocardiography and dynamic cardiac imaging are recommended in all patients. Necrobiotic xanthogranuloma also may involve other organs including the lung, larynx, pharynx, skeletal muscle, kidney, spleen, ovary, and intestine. Histopathologically, NXG is characterized by a granulomatous inflammation in the dermis extending into the subcutaneous fat. Dense infiltrates of macrophages with some foreign body–type giant cells and foamy macrophages are accompanied by areas of degenerated collagen and a moderate lymphocytic infiltrate in some cases with plasma cells. Mucin deposition or cholesterol clefts can be found. The underlying pathogenesis of the disease remains unknown, although in up to 80% of the patients, an association with paraproteinemia, especially monoclonal IgGproteinemia, can be found. Less often, bone marrow examination shows multiple myeloma. No first-line NXG therapy has been established. The recommended therapies of corticosteroids (intralesional and/or systemic), alkylating agents (such as cyclophosphamide, melphalan, or chlorambucil), interferon alfa, antimetabolites, antimicrobial treatment, and plasmapheresis have shown inconsistent success. To our knowledge, this is the first report of successful treatment of NXG with intravenous immunoglobulin (IVIg).