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Multicentric reticulohistiocytosis

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This 62 year old lady had a three year history of symmetrical erosive polyarthritis, initially diagnosed as rheumatoid. She then developed small, purplish nodules around her nailfolds, fingers, nose, and elbows (figure, upper panels). Histopathology of the skin nodules revealed multinucleate giant cells and histiocytes with granular “foamy” cytoplasm containing lipoid material. Distinctive features of multicentric reticulohistiocytosis (MRH) were present on the hand radiographs (figure), including erosions across the endplate, apparent widening of the joint space, predominance of interphalangeal involvement, and relatively retained bone density. MRH is characterised by destructive symmetrical polyarthritis and nodular skin lesions typically affecting middle aged women. The arthritis usually precedes the skin lesions by months or years and is comminly misdiagnosed as rheumatoid until the more classic radiographic features and skin lesions evolve. A mild acute phase response may be present but rheumatoid factor is usually absent. The cause is unclear. Although spontaneous remissions do occur, alkylating agents are the treatment of choice.1 Various malignancies have been reported to develop in up to 30% of cases.

Acknowledgments

Contributors:gary d wright, m doherty.City Hospital, Nottingham NG5 1PB, United Kingdom.

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