Necrobiotic xanthogranuloma with paraproteinemia.
Eight patients had multiple xanthomatous plaques and subcutaneous nodules that had a predilection for the periorbital area, flexures, and trunk and that tended to ulcerate. Skin biopsy specimens showed a combination of xanthogranulomatous nodules with necrobiosis. All patients had an accompanying dysproteinemia, which was a monoclonal IgG paraprotein in six. Hyperlipidemia, low serum complement, and cryoglobulinemia were variable features. Five patients had leukopenia. Bone marrow examination showed myeloma in two patients, a lymphoproliferative process in one, and some atypical plasma cells in two. Cutaneous necrobiotic xanthogranuloma is a distinctive histologic pattern most frequently related to plasma cell dyscrasias, and it should be distinguished from normolipemic plane xanthoma and other necrobiotic granulomas.
D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001797:Blood Protein Electrophoresis; D005260:Female; D006099:Granuloma / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000209:etiology* / Q000473:pathology; D012867:Skin / Q000473:pathology; D014973:Xanthomatosis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
D000970:Antineoplastic Agents; D007074:Immunoglobulin G