Necrobiotic Xanthogranuloma

Abstract

We reviewed 32 cases of necrobiotic xanthogranuloma and 16 cases from the world literature. Necrobiotic xanthogranuloma is a destructive dermal and subcutaneous xanthogranuloma that most frequently involves the face (periorbital region) and trunk. Monoclonal gammopathy is common (IgG-kappa in 23 cases and IgG-lambda in nine cases), and cryoglobulins have also been found. Low complement levels, anemia, and leukopenia are frequently present. Bone marrow examination shows plasma cell proliferation and, rarely, true myeloma. We describe mucosal, muscle, and systemic lesions, including xanthogranulomatous myocardial lesions. The clinical course is chronic and often progressive. Low-dose chlorambucil treatment is safe and effective, but individual patients have responded to treatment with corticosteroids, melphalan, local radiation, and plasma exchange.

Local Corticosteroid Treatment of Eyelid and Orbital Xanthogranuloma

“…17 More inconsistent results have been reported with sporadic use of intralesional corticosteroids for the treatment of periocular NXG. 11,12 Assessment of the efficacy of intralesional corticosteroid treatment in NXG is hampered by the lack of documentation with respect to type, dose, and depth and site of injection. In addition, the need for repeated treatment, which we found to be necessary in our cases, is not mentioned in any of the reports.…”

“…The accessibility of NXG lesions to injection has prompted some to treat them with intralesional corticosteroids, yielding inconsistent results. 11,12 We describe treatment of AXG and NXG with series of local injections of triamcinolone acetonide. This therapy controls the disease while avoiding the side effects of systemic or radiation treatment.…”

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

“…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 In contrast, NXG lesions are firm papules, nodules, or plaques, the color of which varies from violaceous to red-orange or yellow. 4 HX and NX are characterized by foam cell infiltration in the skin, whereas NXG is featured by the association of necrobiosis, giant cells (Touton type), asteroid bodies, cholesterol clefts, and foam cells. 5 It could be argued that the association of xanthomatosis with monoclonal gammopathy is fortuitous, particularly in the elderly because of the relatively high prevalence of monoclonal gammopathy in this population.…”

Intra-abdominal Xanthogranulomatoses Secondary to Postoperative Bile Leak

“…A variant of xanthogranulomatous inflammation called as necrobiotic xanthogranuloma has a chronic, indolent, and progressive course with predilection for periorbital region, trunk and extremities [13]. It is commonly associated with monoclonal gammopathy and multiple myeloma.…”