NECROBIOTIC XANTHOGRANULOMA WITH PARAPROTEINEMIA - AN EVOLVING PRESENTATION

Necrobiotic xanthogranuloma with paraproteinemia is a progressive and destructive process that is often confused both clinically and histolo gically with other granulomatous and xanthomatous entities, It was fir st described by Kossard and Winkelmann in 1980, Prior to this, the ent ity was reported under a variety of names such as atypical multicentri c reticulohistiocytosis with paraproteinemia,(1,2) atypical xanthoma d isseminatum,(1,2) and atypical necrobiosis lipoidica.(1,2) A 69-year-o ld woman experienced slightly pruritic and painful papules, plaques, a nd nodules, Initial biopsy specimens showed a granulomatous process co nsistent with granuloma annulare, Later biopsy specimens demonstrated histologic changes indicative of necrobiosis lipoidica, The most recen t histologic findings are those of necrobiotic xanthogranuloma, with r esults of laboratory studies revealing a coexistent IgG kappa paraprot einemia. Patients with necrobiotic xanthogranuloma who demonstrate a b enign monoclonal proteinemia and are evaluated for several years show a 9 to 11 percent risk of myeloma, amyloidosis, or macroglobulinemia.( 1) Multiple treatment regimens have been attempted, none of which are curative, We propose that this entity may be part of an evolutionary p rocess that may start as a granulomatous entity and culminate in a xan thogranulomatous process with an accompanying paraproteinemia.