Necrobiotic xanthogranuloma in a human T-lymphotropic virus type 1 carrier

A 7-Year History of Necrobiotic Xanthogranuloma following Asymptomatic Multiple Myeloma: A Case Report

“…Chlorambucil appears to be the most effective treatment for patients with extensive cutaneous lesions [15–17]. Other systemic agents have been employed with some improvement including systemic steroid [18, 19], chlorambucil plus systemic corticosteroids, [7] cyclophosphamide, [20] melphalan, [21, 22] melphalan plus systemic corticosteroids, [23–25] azathioprine plus systemic corticosteroids, [26] thalidomide [27], and interferon- α 2b [7, 28]. All treatments can produce remission of paraproteinemia as well as skin lesions but, unfortunately, cannot prevent the evolution to multiple myeloma.…”

Necrobiotic xanthogranuloma

“…Bone marrow biopsies were obtained for 33 of the 70 patients reported since 1992. These included eight with atypical or increased plasma cells 17,23,28,30,38,40,48,49 (ranging from 2% atypical plasma cells to 15% plasma cells), two with multiple myeloma, 44,50 two with known myeloproliferative or lymphoproliferative disease (chronic lymphocytic leukemia and non‐Hodgkin's lymphoma occurring in a single patient, 18 Hodgkin's lymphoma 7 ), and 21 with nonspecific 14,15,21,41,51 or normal bone marrow biopsies (Table 2).…”

“…1). Many cases report ulceration, [6][7][8][9][10][11][12][13][14][15][16][17][18] telangiectases, 8,9,11,17,[19][20][21][22][23][24][25][26][27] erythematous and/or violaceous borders, 6,9,11,14,15,[28][29][30][31] and atrophy. 11,21,22,24,25,27,32,33 Lesions are nearly always asymptomatic, but can be pruritic.…”

“…6,12,42 Melphalan was used more than any other alkylating agent. Ten of 12 patients improved with melphalan, 9,14,17,30,31,40,42,50,53 although one patient with multiple myeloma later showed recurrence of skin lesions, 44 and the duration of the remaining responses is unknown.…”

Interferon Alpha-2a Monotherapy for Necrobiotic Xanthogranuloma

“…NX is characterized clinically by indurated xanthomatous or violaceous nodules and plaques that occur mostly on the trunk and the periorbital area (2). Since, in some cases, involvement of internal organs has been clearly documented (3), NX should be regarded as a systemic disease.…”