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遗传性鱼鳞病和真菌感染:发病机制和治疗策略的最新进展。

Inherited ichthyosis and fungal infection: an update on pathogenesis and treatment strategies.

机构信息

Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.

出版信息

J Dtsch Dermatol Ges. 2021 Mar;19(3):341-350. doi: 10.1111/ddg.14389. Epub 2021 Jan 14.

Abstract

Inherited ichthyoses are a group of genodermatoses classified as either nonsyndromic or syndromic. Nonsyndromic ichthyoses and keratitis, ichthyosis and deafness (KID) syndrome predispose to fungal infection. The diagnosis and treatment of fungal infections underlying ichthyoses are challenging. In this review, we summarize reported cases of ichthyosis with fungal infection over the past 50 years. Atypical manifestations such as alopecia, papules and brittle nails occurred in patients with ichthyosis combined with fungal infection. Various pathogenic mechanisms have been implicated, including mutations of ichthyosis-related genes leading to disruption of the skin barrier via multiple pathways. Host immune disorders, including atopy and abnormal innate immunity also contribute to susceptibility. Specific fungi may escape the immune response. Extensive and recurrent fungal infections are not uncommon in patients with ichthyosis, making a cure more difficult and increasing the need for systemic antifungal therapy. Traditional and new ichthyosis treatments aiming to improve skin barrier function could help prevent fungal infection. In conclusion, the close relationship between ichthyosis and fungal infection is of vital importance in clinical practice and requires more attention from physicians. More studies are required to investigate the mechanisms and explore useful treatment strategies.

摘要

遗传性鱼鳞病是一组遗传性皮肤病,分为非综合征型或综合征型。非综合征型鱼鳞病和角膜炎、鱼鳞病和耳聋(KID)综合征易发生真菌感染。鱼鳞病相关真菌感染的诊断和治疗具有挑战性。在这篇综述中,我们总结了过去 50 年来报道的鱼鳞病伴真菌感染病例。鱼鳞病合并真菌感染的患者出现脱发、丘疹和脆甲症等非典型表现。多种发病机制被认为与鱼鳞病相关基因的突变有关,这些突变通过多种途径破坏皮肤屏障。宿主免疫紊乱,包括特应性和先天免疫异常,也会导致易感性。特定的真菌可能逃避免疫反应。鱼鳞病患者广泛和反复的真菌感染并不少见,这使得治愈更加困难,并增加了全身抗真菌治疗的需求。旨在改善皮肤屏障功能的传统和新型鱼鳞病治疗方法可能有助于预防真菌感染。总之,鱼鳞病与真菌感染的密切关系在临床实践中至关重要,需要医生给予更多关注。需要更多的研究来探讨机制并探索有用的治疗策略。

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