Unusual and memorable
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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,
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