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.