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掌跖脓疱病的临床特征、遗传学、合并症及治疗:台湾某单中心66例病例的回顾性分析

Clinical characteristics, genetics, comorbidities and treatment of palmoplantar pustulosis: A retrospective analysis of 66 cases in a single center in Taiwan.

作者信息

Huang Chang-Ming, Tsai Tsen-Fang

机构信息

Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan.

Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.

出版信息

J Dermatol. 2020 Sep;47(9):1046-1049. doi: 10.1111/1346-8138.15470. Epub 2020 Jul 1.

Abstract

We retrospectively analyzed 66 patients with palmoplantar pustulosis (PPP) from January 1994 to September 2019 in our department. Interleukin-36 receptor antagonist gene (IL36RN) intron 3 c.115+6T>C mutation was present in two out of 27 patients (7.4%). Both cases developed generalized pustular psoriasis and/or acrodermatitis continua of Hallopeau later. Topical medications and phototherapy were used in 93.9% and 28.8% of patients, respectively, while 60.6% received systemic agents. The majority of patients (60.6%) responded to treatment, but episodes of flare-up existed. The demographic data of our patients with PPP showed female predominance (59.1%), middle-age onset (44.2 years old) and current smokers (62.1%). Generalized pustular psoriasis initially presenting as palmoplantar lesions may be misdiagnosed as PPP, and the presence of IL36RN mutation may serve to predict or confirm the diagnosis of future generalized pustular psoriasis or acrodermatitis continua of Hallopeau. To our knowledge, this is the largest demographic study of PPP in Taiwan.

摘要

我们回顾性分析了1994年1月至2019年9月在我科就诊的66例掌跖脓疱病(PPP)患者。27例患者中有2例(7.4%)存在白细胞介素-36受体拮抗剂基因(IL36RN)内含子3 c.115+6T>C突变。这两例患者后来均发展为泛发性脓疱型银屑病和/或Hallopeau连续性肢端皮炎。分别有93.9%和28.8%的患者使用了局部用药和光疗,而60.6%的患者接受了全身用药。大多数患者(60.6%)对治疗有反应,但存在病情复发情况。我们的PPP患者人口统计学数据显示女性占优势(59.1%),发病年龄为中年(44.2岁),且当前吸烟者占(62.1%)。最初表现为掌跖损害的泛发性脓疱型银屑病可能会被误诊为PPP,而IL36RN突变的存在可能有助于预测或确诊未来的泛发性脓疱型银屑病或Hallopeau连续性肢端皮炎。据我们所知,这是台湾地区关于PPP的最大规模人口统计学研究。

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