Abstract:
Multicentric reticulohistiocytosis (MRH) is a rare, class IIb non-Langerhans cell histiocytosis associated with skin and joint involvement. It is more prevalent (80%) in Caucasian females in their fifth to sixth decade of life. Patients usually demonstrate symptoms and signs of symmetric polyarthritis and papulonodular cutaneous lesions. In addition to skin and joints, multiple organs can be involved, such as the lung (pleural effusion, interstitial fibrosis, hilar lymphadenopathy), heart (pericardial effusion, myocarditis), gastrointestinal system, and urogenital system (genital tract and kidney). Pericardial involvement is a rare manifestation, and around three cases have been reported in the literature so far. Our case report is a valuable contribution to the literature, which aids clinicians in contemplating MRH as one of the differentials among patients presenting with pericardial effusion. We described the characteristics of MRH along with its differentiating features from other autoimmune conditions and management.
摘要:
多中心网状组织细胞增生症(MRH)是一种罕见的,与皮肤和关节受累相关的IIb类非朗格汉斯细胞组织细胞增生症。它在高加索女性中在他们的第五到第六十年中更普遍(80%)。患者通常表现出对称性多关节炎和丘疹性皮肤病变的症状和体征。除了皮肤和关节,可能涉及多个器官,如肺(胸腔积液,间质纤维化,肺门淋巴结病),心脏(心包积液,心肌炎),胃肠系统,和泌尿生殖系统(生殖道和肾脏)。心包受累是一种罕见的表现,到目前为止,文献中已经报道了大约三例。我们的病例报告是对文献的宝贵贡献,这有助于临床医生将MRH视为存在心包积液的患者之间的差异之一。我们描述了MRH的特征及其与其他自身免疫性疾病和管理的区别特征。