Multicentric Reticulohistiocytosis


Multicentric reticulohistiocytosis is a rare syndrome of adulthood characterized by the rapid appearance of multiple papules and nodules arising in conjunction with a severe, mutilating arthritis. When occurring as part of the syndrome, the reticulohistiocytomas are found on mucosal surfaces in up to 50% of cases (1–2). Visceral reticulohistiocytomas involving lymph nodes, bone marrow, lungs, and endocardium have also been described in patients with the syndrome (3,4). Cardiac involvement may result in cardiac failure in extreme cases. Xanthomatous lesions may also be present. The destructive osteoarthritis tends to be symmetrical and involves primarily the joints in the hands. The knees and wrists are also involved in greater than 50% of cases. The arthritis may precede, occur concurrently with, or follow the appearance of the cutaneous lesions but is the presenting sign in most cases. After a period of rapid deterioration, the arthritis usually stabilizes, but does not improve. In approximately 30% of cases, the constellation of multiple reticulohistiocytomas and destructive osteoarthritis has been associated with visceral malignancies (3,5,6).


Mucinous Adenocarcinoma Cervical Squamous Cell Carcinoma Slight Female Predominance Visceral Malignancy Express S100 Protein 
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