French national protocol for the management of congenital ichthyosis

IF 3.1 4区医学 Q2 DERMATOLOGY

Annales De Dermatologie Et De Venereologie Pub Date : 2024-03-01 DOI:10.1016/j.annder.2024.103247

M. Severino-Freire , C. Granier Tournier , C. Chiaverini , A. Audouze , F. Morice-Picard , H. Texier , I. Dreyfus , A.-C. Bing-Lecointe , S. Mallet , C. Bodemer , J. Fischer , N. Jonca , J. Mazereeuw-Hautier , On behalf of the French Pediatric Dermatology Research Group

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引用次数: 0

Abstract

Congenital ichthyoses (CI) comprise a heterogeneous group of monogenic genetic skin diseases characterized by diffuse scaling, often associated with skin inflammation. Diagnosis of the individual form of ichthyosis is complex and is guided by clinical expertise. CI usually has a major impact on quality of life (QOL) and thus requires lifelong treatment. To date, there are no curative therapies, although various symptomatic treatment options exist.

The present protocol for the management of CI has been drawn up in accordance with the recommendations published in 2012 by the French National Authority for Health, based on a literature review, with the help and validation of members of the French network for rare skin diseases (FIMARAD). It provides a summary of evidence and expert-based recommendations and is intended to help clinicians with the management of these rare and often complex diseases.

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法国国家先天性鱼鳞病治疗方案

先天性鱼鳞病（CI）是一组异质性的单基因遗传性皮肤病，以弥漫性鳞屑为特征，常伴有皮肤炎症。鱼鳞病的诊断非常复杂，需要临床专业知识的指导。CI 通常会对生活质量（QOL）产生重大影响，因此需要终生治疗。本CI治疗方案是根据法国国家卫生局2012年发布的建议制定的，以文献综述为基础，并得到了法国罕见皮肤病网络（FIMARAD）成员的帮助和认可。它提供了证据摘要和专家建议，旨在帮助临床医生管理这些罕见且复杂的疾病。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Annales De Dermatologie Et De Venereologie 医学-皮肤病学

CiteScore

1.40

自引率

0.00%

发文量

审稿时长

81 days

期刊介绍： Les Annales de dermatologie sont le rendez-vous mensuel incontournable de toute la dermatologie francophone, grâce à leur comité de rédaction qui assure une sélection rigoureuse des articles selon les normes de l''édition scientifique internationale. Une revue didactique, véritable aide à la pratique médicale quotidienne Pour compléter et enrichir la partie scientifique, la rubrique Formation médicale continue propose aux lecteurs des textes didactiques et interactifs (Cas pour diagnostic, Notes de pharmacovigilance, la Question du praticien, Dermatologie chirurgicale, la Sélection bibliographique du mois...) qui les font bénéficier d''une formation post-universitaire diversifiée et de qualité. La revue consacre également un espace pour la publication de questions des lecteurs auxquelles des experts apportent une réponse.