Necrobiotic xanthogranuloma

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

“…Clinical presentation of xanthomas resembles that seen in general hyperlipidemic forms and is usually not suggestive of the presence of an associated MIg, except for the nodules and plaques, sometimes with an inflammatory aspect, that are characteristic features of the so-called NXG: MIg is found in ϳ 80% of patients with this presentation. 8 Although we lack sufficient data to precisely assess the frequency of xanthomatosis and MIg association, it would seem reasonable to recommend performing at least a serum electrophoresis when faced with a xanthoma lesion, with the exception of isolated xanthelasma, particularly in the absence of abnormalities of serum lipid tests. Indeed, although NX is usually much less frequent than HX, it was reported more often in association with MIg, both in our series and in the literature.…”

“…6 Few data are available for NXG, but an interaction between the MIg and a lipoprotein and activation of circulating monocytes has been described in 1 case each. 8,9 Assuming a common physiopathology of the 3 forms of xanthoma with MIg, the different clinical presentation might be explained by a different target of the MIg or by host-dependent variable responses to the lipoprotein deposits in monocytes and macrophages.…”

“…46,[64][65][66][67] Extracutaneous lesions were reported in 23 cases (17.6%), including 6 patients with several localizations. They were pulmonary (n ϭ 7), 8 cardiac (n ϭ 6), 8,37 oral cavity (n ϭ 4), 38,65 orbital (n ϭ 3), 8 hepatic (n ϭ 2), 8,37 spleen (n ϭ 2), 8 medullar (n ϭ 1), 68 genital (penis; n ϭ 1), 34 mastoid (n ϭ 1), 32 facial nerve (n ϭ 1), 32 and renal (n ϭ 1). 69 In addition, splenomegaly was noted in 22 patients (17%) and hepatomegaly in 17 (13%), without any further information.…”

“…We reviewed 216 well-described cases of xanthomatosis associated with MIg in the literature, including 37 cases of HX, 3 63 of NX, 6,9-30 and 116 of NXG 4,8,[31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47] (Tables 3 and 4). The main patient characteristics are listed in Tables 3 and 4.…”

“…3,6,7 The physiopathology of NXG remains much less well understood than the 2 other forms. 8 Therapeutic options for patients with xanthoma lesions and associated MIg, which depend on the demonstration of a relationship between the 2 disorders, are currently poorly defined. With a view to gaining further knowledge of the association of xanthoma and MIg, we analyzed a personal series of 24 patients with respect to clinical, biologic, pathologic, and therapeutic parameters and compared our findings with existing data from the literature.…”