Cutaneous lesions and articular manifestations in multicentric reticulohistiocytosis: a diagnostic challenge.

IF 2.9 3区 医学 Q2 RHEUMATOLOGY
Carlos Marques-Gomes, Inês Rodrigues, Catarina Rua, Rúben Costa, Mariana Lopes, Gilberto Rosa, Sofia Magina, José Manuel Lopes, Iva Brito
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Abstract

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.

多中心网状组织细胞增多症的皮肤病变和关节表现:一个诊断挑战。
56岁女性,一年多关节痛和皮肤病变病史。检查发现多关节炎和指间关节(IF)红褐色结节。组织病理学证实为多中心网状组织细胞增多症(MRH),特征性组织细胞和多核巨细胞PAS、PASD、CD45和CD68阳性。影像学显示关节周围骨质减少和糜烂。强的松龙(PDN)和甲氨蝶呤(MTX)的免疫抑制治疗在两个月内显著改善了症状。MRH是一种罕见的非朗格汉斯细胞组织细胞增多症,以丘疹样皮肤病变和对称多关节炎为特征,具有潜在的侵蚀性。它可能与风湿病有关,需要进行恶性肿瘤筛查。由于缺乏特定的生物标志物,诊断基于临床和组织学结果。免疫抑制治疗仍然是治疗的基石。
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来源期刊
Clinical Rheumatology
Clinical Rheumatology 医学-风湿病学
CiteScore
6.90
自引率
2.90%
发文量
441
审稿时长
3 months
期刊介绍: Clinical Rheumatology is an international English-language journal devoted to publishing original clinical investigation and research in the general field of rheumatology with accent on clinical aspects at postgraduate level. The journal succeeds Acta Rheumatologica Belgica, originally founded in 1945 as the official journal of the Belgian Rheumatology Society. Clinical Rheumatology aims to cover all modern trends in clinical and experimental research as well as the management and evaluation of diagnostic and treatment procedures connected with the inflammatory, immunologic, metabolic, genetic and degenerative soft and hard connective tissue diseases.
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