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Multicentric Reticulohistiocytosis Presenting with Papulonodular Skin Lesions and Arthritis Mutilans

DOI: 10.1155/2013/201563

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Abstract:

Multicentric reticulohistiocytosis is a rare multisystem disorder of unknown etiology that is characterized by erosive polyarthritis and papulonodular lesions on the skin, mucous membranes, and internal organs. We report the case of a 54-year-old female who was misdiagnosed as having rheumatoid arthritis and underwent numerous joint replacement surgeries for progressively destructive arthritis in her hands, shoulders, hips, and knees. The patient finally received a diagnosis of multicentric reticulohistiocytosis after histopathological examination of the patient’s left knee arthroplasty which revealed a diffuse histiocytic infiltrate, multinucleated giant cells, and finely granulated eosinophilic cytoplasm with a ground-glass appearance. 1. Introduction Multicentric reticulohistiocytosis (MRH) is a rare multisystem disorder of unknown etiology, characterized by erosive polyarthritis and papulonodular lesions on the skin, mucous membranes, and internal organs. MRH is the most destructive chronic inflammatory arthritis, manifesting more aggressively than either rheumatoid arthritis (RA) or psoriatic arthritis [1], and progressing to arthritis mutilans in 45% of cases [2]. The onset of the disease is usually in the forth decade of life, affecting females two to three times more often than males [3, 4], and presenting insidiously with joint symptoms preceding skin manifestations in the majority of patients. Herein, we describe the case of a patient found to have multicentric reticulohistiocytosis who was previously misdiagnosed as having rheumatoid arthritis for several years. 2. Description of Case A 54-year-old African-American female with a history of multiple joint replacements presented to clinic complaining of severe diffuse arthritis. She reported progressive morning stiffness and arthralgias in her knees that first started 10 years prior, followed shortly by the development of skin nodules on the dorsum of both hands. Since this time, multiple other joints became affected including her bilateral hands, hips, and shoulders. She was initially seen by a rheumatologist 10 years ago and diagnosed with RA. Over the course of her disease, she tried numerous therapies including ibuprofen, prednisone, etanercept, methotrexate, and adalimumab. However, she had a poor response to these medications, and her arthritis continued to worsen. She eventually underwent bilateral hip and knee replacement surgeries to improve her deteriorating functional ability. On physical exam, the patient had multiple nonpruritic reddish-brown papulonodular lesions on the dorsum of

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