Cardiac engagement in multicentric reticulohistiocytosis: report of a case with fatal outcome and literature review
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Multicentric reticulohistiocytosis is a rare systemic disorder of unknown etiology characterized by severe destructive arthritis, development of cutaneous nodules and is in many cases associated with malignancy.
In multicentric reticulohistiocytosis polyarticular, symmetric, erosive arthritis is common and progresses to mutilans arthritis in 45% of cases. Skin lesions are characterized by typical nodular manifestations and plaques containing lipid-laden (Periodic Acid Schiff positive) histiocytes and multinucleated giant cells .
In multicentric reticulohistiocytosis involvement of other organs (such as heart, lungs, liver, kidney, thyroid, bone marrow and muscles) and systemic symptoms (like fever and weight loss) have been described less commonly .
We report a case of multicentric reticulohistiocytosis with mutilans arthritis, later cardiac engagement and fatal outcome in spite of therapy.
In August 2000 a 37-year-old woman was referred to our Rheumatology Unit. She manifested...
KeywordsPrimary Biliary Cirrhosis Pericardial Effusion Multinucleated Giant Cell Histiocytosis Hemophagocytic Lymphohistiocytosis
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