Multicentric Reticulohistiocytosis ( 2 фото )

A 32-year-old man presented to the rheumatology clinic with a 7-year history of skin nodules, along with swelling, pain, and morning stiffness in his fingers and knuckles. On physical examination, there were smooth nodules over the chest wall, auricles (Panel A), and dorsal surface of the distal fingertips (Panel B). There was also synovitis of the proximal and distal interphalangeal joints and metacarpophalangeal joints. Laboratory studies revealed normal inflammatory markers and negative results for rheumatoid factor and antibody against cyclic citrullinated peptide. Radiographs of the hands showed marginal erosions of multiple interphalangeal joints (Panel C, arrows). Histopathological examination of a skin-biopsy sample obtained from a chest nodule showed histiocytes and multinucleated giant cells with abundant eosinophilic granular ground-glass–like cytoplasm in the dermis, hyperplasia of fibrous tissue, and acute and chronic inflammatory-cell infiltration. A diagnosis of multicentric reticulohistiocytosis was made. This condition is a rare systemic form of non–Langerhans-cell histiocytosis that is characterized by erosive arthritis and skin lesions. It may be misdiagnosed as rheumatoid arthritis owing to the presence of inflammatory arthritis and periarticular skin nodules. Treatment with a tapering dose of prednisone, hydroxychloroquine, and methotrexate was initiated. At the 1-year follow-up, the patient’s joint pain and skin lesions had abated.

Authors: Fei Sun, M.D. , and Jie Zhang, M.D.Author Info & Affiliations
Published June 15, 2024
N Engl J Med 2024;390:2199
DOI: 10.1056/NEJMicm2313532
VOL. 390 NO. 23


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