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囊性纤维化性皮炎关节炎综合征:4例病例系列

Cystic fibrosis dermatitis arthritis syndrome: A series of four cases.

作者信息

Schwartzman Gabrielle, Zlotoff Barrett J, Gru Alejandro A, Guffey Darren J

机构信息

Department of Dermatology, University of Virginia, Charlottesville, Virginia, USA.

出版信息

Pediatr Dermatol. 2025 Jan-Feb;42(1):133-135. doi: 10.1111/pde.15727. Epub 2024 Aug 8.

Abstract

Dermatologic manifestations of cystic fibrosis (CF) include nutrient deficiency dermatoses, vasculitis, transient reactive papulotranslucent acrokeratodema, digital clubbing, and increased rates of atopy and drug reactions. Few cases of a characteristic eruption in patients with episodic arthritis of CF have been described with prior reports primarily occurring outside of the dermatology literature. We report four cases consistent with this presentation to add to the literature and propose a new and unifying name to recognize this entity as cystic fibrosis dermatitis arthritis syndrome (CF-DAS). Clinical suspicion should remain high in young female patients with cystic fibrosis presenting with episodic joint pain and rash, independent of pulmonary exacerbations.

摘要

囊性纤维化(CF)的皮肤表现包括营养缺乏性皮肤病、血管炎、短暂性反应性丘疹半透明肢端角化病、杵状指,以及特应性和药物反应发生率增加。CF发作性关节炎患者出现特征性皮疹的病例很少,先前的报告主要发表在皮肤科文献之外。我们报告了4例符合该表现的病例以补充文献,并提出一个新的统一名称,将这一实体称为囊性纤维化皮炎关节炎综合征(CF-DAS)。对于患有囊性纤维化且出现发作性关节疼痛和皮疹的年轻女性患者,应高度怀疑CF-DAS,无论是否有肺部加重情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/50ae/11781019/e36f1093f2a8/PDE-42-133-g002.jpg

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