A 57-year-old woman developed joint pain, deformity and cutaneous nodules. Reticulohistiocytosis was diagnosed after skin biopsy. Nodule formation and progressive arthritis continued for almost 2 years but appeared to cease when treatment with cyclophosphamide began.Mutilating polyarthritis is the most significant manifestation of reticulohistiocytosis. Lipoid dermatoarthritis, giant-cell reticulohistiocytoma and multicentric reticulohisiocytic granuloma are among other names applied to this rare disorder of unknown etiology (1-3). Arthritis is the initial complaint in most of the cases reported. Skin nodules are a less common mode of onset, but both nodules and arthritis usually occur. Involvement of other organs has been noted at autopsy (4).Severe disability may accompany joint involvement. Nodule formation in the synovium is responsible for articular damage. A case is reported in which cyclophosphamide therapy may have been responsible for arresting the development of nodules and preventing further disability.
CASE REPORTJT was generally well until December 1968, when, at age 57, she developed pain in her knees associated with walking and pain with motion of her shoulders. At about the same time, she noted the appearance of light-brown skin nodules. These began under the skin and gradually elevated. They occurred on the hands, usually the dorsum. extensor forearms and bridge of the nose. There were none on the trunk or legs. Two months later, she underwent an hysterectomy with postoperative cobalt radiation for carcinoma of the cervix. She endured the skin lesions and rheumatic symptoms for the next year and a half before being seen by an orthopedic surgeon, who hospitalized her for evaluation and biopsy in .July 1970.Positive physical findings on admission were limited to the joints and skin. There was pain with rotation of the shoulders; wrist motion was limited to 30" of flexion and extension, but there was no active synovitis. There was slight swelling of the metacarpophalangeal and interphalangeal joints, with 50% of normal fist formation. The knees demonstrated mild synovial thickening and pain, with flexion beyond 90". Other joints were normal. Skin nodules were present on the dorsum of the hands (Figure l), the forearms and bridge of the nose.Laboratory studies demonstrated mild normochromic anemia with hemoglobin of 11.0 g%. The leukocyte count was 3800/cu mm with a normal differential; the sedimentation rate was 37 mm/hr (Westergren); tests for rheumatoid factor and antinuclear antibodies were negative; protein electrophoresis was normal; and a series of blood chemistries revealed a fasting glucose of 160 mg/100 ml, but other studies, including cholesterol, were normal. Serology was negative.X-rays of the hands revealed marked erosive changes in the interphalangeal joints, both proximal and distal. Erosions were present but less widespread in the metacarpophalangeal joints. There were some localized erosions in the wrist, especially involving the navicular and greater multangular bones. The knees d...
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