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口和生殖器溃疡伴软骨炎症综合征:病例报告及已发表文献综述

Mouth and Genital Ulcers with Inflamed Cartilage Syndrome: Case Report and Review of the Published Work.

作者信息

Kaneko Yuka, Nakai Noriaki, Kida Takashi, Kawahito Yutaka, Katoh Norito

机构信息

Department of Dermatology, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan.

Department of Inflammation and Immunology, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan.

出版信息

Indian J Dermatol. 2016 May-Jun;61(3):347. doi: 10.4103/0019-5154.182463.

Abstract

Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome are disease that fulfilled criteria for diagnosis of Behcet's disease (BD) and relapsing polychondritis (RP). We report a 22-year-old Japanese woman presented with MAGIC syndrome and we described the clinicopathological characteristics of MAGIC syndrome based on a review of published cases from July 1985 to December 2015. In our case, the patient with oral aphthae, erythema nodosum, acne-like eruptions, uveitis, and polyarthritis fulfilled criteria for diagnosis of incomplete form of BD. The patient with uveitis, polyarthritis, and histological confirmation of chondritis also fulfilled criteria for diagnosis of RP. The patient was successfully treated with oral colchicine followed by prednisolone. The symptoms of MAGIC syndrome gradually disappeared, and the prednisolone dosage was gradually decreased and stopped. She has been in remission without active medication for a further 8 months. In the previous reports, some authors suggested that MAGIC syndrome was not a disease entity and might be RP occurring secondary to BD, another association of an autoimmune disease, or vasculitis with RP. However, the pathogenic association between MAGIC syndrome, BD, and RP is still unclear, and the number of reported cases of MAGIC syndrome is insufficient to establish a clear explanation. Therefore, further accumulation of data and careful observation of the clinical course are required to improve the understanding of MAGIC syndrome.

摘要

口生殖器溃疡伴软骨炎(MAGIC)综合征是一种符合白塞病(BD)和复发性多软骨炎(RP)诊断标准的疾病。我们报告了一名患有MAGIC综合征的22岁日本女性,并基于对1985年7月至2015年12月已发表病例的回顾描述了MAGIC综合征的临床病理特征。在我们的病例中,该患者有口腔溃疡、结节性红斑、痤疮样皮疹、葡萄膜炎和多关节炎,符合不完全型BD的诊断标准。该患者有葡萄膜炎、多关节炎且软骨炎经组织学证实,也符合RP的诊断标准。患者口服秋水仙碱后接着使用泼尼松龙治疗成功。MAGIC综合征的症状逐渐消失,泼尼松龙剂量逐渐减少并停用。在未进行积极药物治疗的情况下,她又缓解了8个月。在既往报告中,一些作者认为MAGIC综合征不是一个独立的疾病实体,可能是继发于BD的RP、另一种自身免疫性疾病的关联情况或伴有RP的血管炎。然而,MAGIC综合征、BD和RP之间的致病关联仍不清楚,且MAGIC综合征的报告病例数不足以得出明确解释。因此,需要进一步积累数据并仔细观察临床病程,以增进对MAGIC综合征的了解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d07/4885202/483d440c26c7/IJD-61-347a-g001.jpg

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