Marques-Gomes Carlos, Rodrigues Inês, Rua Catarina, Costa Rúben, Lopes Mariana, Rosa Gilberto, Magina Sofia, Lopes José Manuel, Brito Iva
Rheumatology Department, Unidade Local de Saúde de São João, Alameda Prof. Hernâni Monteiro, 4200-319, Porto, Portugal.
Department of Medicine, Faculty of Medicine, University of Porto, Porto, Portugal.
Clin Rheumatol. 2025 Jul;44(7):3127-3129. doi: 10.1007/s10067-025-07522-9. Epub 2025 Jun 3.
A 56-year-old woman presented with a one-year history of polyarthralgia and skin lesions. Examination revealed polyarthritis and reddish-brown nodules on interphalangeal (IF) joints. Histopathology confirmed a diagnosis of multicentric reticulohistiocytosis (MRH) with characteristic histiocytes and multinucleated giant cells positive for PAS, PASD, CD45, and CD68. Imaging showed periarticular osteopenia and erosions. Immunosuppressive therapy with prednisolone (PDN) and methotrexate (MTX) led to significant improvement in symptoms within two months. MRH, a rare non-Langerhans cell histiocytosis, is marked by papulonodular skin lesions and symmetric polyarthritis, potentially erosive. It may be associated with rheumatic diseases and necessitates malignancy screening. Diagnosis is based on clinical and histological findings due to the absence of specific biomarkers. Immunosuppressive treatment remains the cornerstone of management.
一名56岁女性，有一年多的多关节疼痛和皮肤病变病史。检查发现多关节炎以及指间关节处有红棕色结节。组织病理学确诊为多中心网状组织细胞增生症（MRH），其特征性组织细胞和多核巨细胞对PAS、PASD、CD45和CD68呈阳性。影像学显示关节周围骨质减少和侵蚀。使用泼尼松龙（PDN）和甲氨蝶呤（MTX）进行免疫抑制治疗，两个月内症状显著改善。MRH是一种罕见的非朗格汉斯细胞组织细胞增生症，其特征为丘疹结节性皮肤病变和对称性多关节炎，可能具有侵蚀性。它可能与风湿性疾病相关，需要进行恶性肿瘤筛查。由于缺乏特异性生物标志物，诊断基于临床和组织学发现。免疫抑制治疗仍然是治疗的基石。
