A case of multicentric reticulohistiocytosis responsive to azathioprine in a patient with no underlying malignancy

Abstract

Multicentric reticulohistiocytosis (MRH), a rare histiocytic systemic condition characterized by mutilating arthritis and multiple cutaneous nodules, has been associated with malignancy including that of the breast, thyroid and colon. An unsubstantiated link with infectious agents such as mycobacterium tuberculosis has been described. Many treatments have been used with varying success. We describe the case of a 60-year-old man with MRH and no underlying malignancy who initially responded well to azathioprine and whose disease recurred upon cessation. A second course of azathioprine administered in conjunction with antituberculous treatment resulted in disease control. Broader associations and features of this rare disease are discussed.

Multicentric Reticulohistiocytosis Presenting with Papulonodular Skin Lesions and Arthritis Mutilans

“…These drugs have been used in various combinations with adjuvant nonsteroidal anti-inflammatory agents, hydroxychloroquine, and corticosteroids [14]. MRH has also shown a successful response to azathioprine [15] and leflunomide [16]. More recent data show excellent results achieved with antitumor necrosis factor (TNF α ) therapy including etanercept [17], infliximab [18], and adalimumab [19].…”

Multicentric Reticulohistiocytosis–A rare and disabling disease

“…Although MRH is not a paraneoplastic disease, solid organ especially breast and stomach carcinomas and hematological malignancies have been reported in 31% of cases, 5 therefore underlying malignancy should always be ruled out in a patient with MRH.…”