Multicentric reticulohistiocytosis (lipoid dermato-arthritis). An erosive polyarthritis with distinctive clinical, roentgenographic and pathologic features.

Detailed analysis of the progressive roentgenographic changes found in complete serial skeletal surveys of 2 men with multicentric reticulohistiocytosis (MR) have been correlated with pathologic finds and compared to features preivously reported. The lesions of MR predominate in skin and synovium and result in the following characteristic features: (1) sharply circumscribed, rapidly progressive, strikingly bilaterally symmetric erosions spreading from joint margins to articular surfaces; (2) interphalangeal joint predominance; (3) early and severe atlanto-axial involvement; (4) disproportion between severity of joint destruction and mildness of symptoms; (5) absent or minimal periosteal reaction; (6) absent or disproportionately mild osteoporosis compared to the severity of erosions; and (7) prominent, uncalcified soft tissue nodules. Since arthritis often precedes cutaneous manifestations, careful roentgenologic evaluation may play key role in early diagnosis.