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多发性网状组织细胞增生症伪装为皮肤结缔组织疾病。

Multicentric reticulohistiocytosis masquerading as cutaneous connective tissue disease.

机构信息

Department of Dermatology, HCA Healthcare/USF Morsani College of Medicine Largo, FL.

出版信息

Dermatol Online J. 2021 Mar 15;27(3):13030/qt37h8p3rp.

PMID:33865276
Abstract

Multicentric reticulohistiocytosis (MRH) is a rare type of non-Langerhans cell histiocytosis characterized by coral-toned papules with predilection for dorsal surfaces in addition to severe arthropathy. It sometimes proves difficult to differentiate these joint and skin findings clinically from certain rheumatologic diseases, primarily dermatomyositis. Herein, we present an 82-year-old woman who presented with the clinical findings described above and was subsequently diagnosed with MRH after biopsy and review of relevant clinical history. Because about 25% of patients diagnosed with MRH have an underlying occult malignancy, our patient underwent a complete malignancy workup that was negative. She was treated with systemic corticosteroids and methotrexate, which resulted in an improvement of the arthritis and constitutional symptoms. This case demonstrates that in patients with both rheumatologic and dermatologic symptoms, particularly on acral surfaces, MRH must be a diagnostic consideration. Identifying this disease early in its course can prevent negative consequences for the patients, specifically arthritis mutilans and upper airway involvement.

摘要

多发性骨肥大性网状组织细胞增生症(MRH)是一种罕见的非朗格汉斯细胞组织细胞增生症,其特征是珊瑚色丘疹,除严重关节炎外,还偏爱背部表面。这些关节和皮肤表现临床上有时很难与某些风湿病(主要是皮肌炎)区分开来。在此,我们介绍一位 82 岁的女性患者,其临床表现如上所述,在活检和回顾相关临床病史后被诊断为 MRH。由于约 25%的 MRH 患者存在潜在隐匿性恶性肿瘤,因此我们对该患者进行了全面的恶性肿瘤检查,结果为阴性。她接受了全身皮质类固醇和甲氨蝶呤治疗,关节炎和全身症状均有所改善。该病例表明,对于同时存在风湿病和皮肤病表现(特别是在肢端表面)的患者,必须考虑 MRH 的诊断。早期识别这种疾病可以防止对患者造成负面影响,特别是关节炎破坏性和上呼吸道受累。

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Multicentric reticulohistiocytosis masquerading as cutaneous connective tissue disease.多发性网状组织细胞增生症伪装为皮肤结缔组织疾病。
Dermatol Online J. 2021 Mar 15;27(3):13030/qt37h8p3rp.
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Multicentric reticulohistiocytosis: a case report of an atypical presentation in a 2-year-old.多中心性网状组织细胞增生症:一名2岁患儿非典型表现的病例报告
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Multicentric reticulohistiocytosis with dermatomyositis-like features: a more common disease presentation than previously thought.多中心性网状组织细胞增生症伴皮肌炎样特征:比以往认为的更常见的疾病表现。
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