Musculoskeletal Imaging

Multicentric reticulohistiocytosis

a systemic disease of unknown cause characterized by proliferation of histiocytes in the numerous tissues (skin, mucosa, subcutaneous tissues, synovia and occasionally bone and periosteum). Multiple joints are involved, including the interphalangeal joints of the fingers, knees, shoulders, wrists, hips, ankles, feet, elbows, spine and temporomandibular joints. Patients with multicentric reticulohistiocytosis may also have xanthelasma related to presence of hypercholesterolaemia. Other manifestations are tendon sheath swelling, lymphadenopathy, erythema, joint hypermobility and pathologic fractures.

Radiographically, well circumscribed erosions of the interphalangeal joints of the hand and foot, early and severe involvement of the atlantoaxial joint, erosive arthritis beginning at the margins of the joint and spreading centrally, lack of periarticular osteoporosis or periosteal bone formation, and uncalcified nodules of skin, subcutaneous tissues, and tendon sheaths are observed. The joint space may be widened or narrowed. Arthritis mutilans may develop in progressive disease. The glenohumeral and acromioclavicular joints, hip, knee, elbow and ankle may also reveal abnormalities on radiographs. Changes in the sacroiliac joint include erosion with bony ankylosis of the articular space, and in the cervical spine, apophyseal joint erosions and severe destruction may be encountered.

DR