Multicentric reticulohistiocytosis associated with primary biliary cirrhosis: successful treatment with cytotoxic agents

✍ Scribed by M. Doherty; M. F. R. Martin; P. A. Dieppe

• Publisher: John Wiley and Sons
• Year: 1984
• Tongue: English
• Weight: 422 KB
• Volume: 27
• Category: Article
• ISSN: 0004-3591
• DOI: 10.1002/art.1780270316

✦ Synopsis

Multicentric reticulohistiocytosis (MRH) is a rare systemic disease of unknown etiology characterized histologically by widespread infiltrations of multinucleate giant cells and lipid-laden histiocytes. Rapidly destructive polyarthritis and nodular skin lesions are the most significant manifestations that may result in mutilating deformity, particularly of the hands and face (1-3). Involvement of other tissues including muscle, bone, pleura, pericardium, and endocardium has also been reported (2,4). The rarity of the disease, with fewer than 50 cases reported in the literature, has resulted in uncertainty over the clinical course of the condition (2,5) and precluded any possibility of controlled treatment trials.

A case of MRH, presenting with polyarthritis and skin lesions, is reported in an HLA-A1,B8 positive woman, who in addition had vitiligo, primary biliary cirrhosis (PBC), and autoimmune thyroid disease. Marked improvement in her arthritis and skin lesions was observed during two 3-month periods while she received oral cyclophosphamide. Relapse followed treatment cessation on both occasions. Sub-~~