In 2019, a group of experts published the first European guidelines for the management of congenital ichthyoses after a multidisciplinary expert meeting held in 2016. An update of these guidelines and literature search was planned every 5 years, given the clinical, molecular and therapeutic advances, including the use of biologic therapies. We present here updated guidelines that have been developed by a reorganized multidisciplinary group of international experts. The evidence is based on a systematic review of recent literature, discussions and consensus reached at an expert conference held in June 2023. The guidelines provide summarized evidence and expert-based recommendations that aim to guide clinicians in the management of these rare and often complex diseases. These guidelines consist of two sections. This Part one covers topical and systemic therapies (including oral retinoids, biologics and Janus kinase inhibitors), future therapeutic approaches, psychosocial management, telemedicine, communicating the diagnosis and genetic counselling, prenatal diagnosis and preimplantation genetic testing.

2019年，一个专家小组在2016年举行的多学科专家会议后发布了首部欧洲先天性鱼鳞病管理指南。鉴于临床、分子及治疗领域的进展（包括生物制剂疗法的应用），原计划每5年对这些指南进行更新和文献检索。本文呈现的是由重组后的国际多学科专家小组制定的更新版指南。证据基于对近期文献的系统性回顾、讨论以及2023年6月专家会议达成的共识。该指南通过总结证据和提供基于专家共识的建议，旨在指导临床医生管理这类罕见且通常复杂的疾病。

本指南包含两个部分。此第一部分涵盖局部和全身治疗（包括口服维A酸类药物、生物制剂及Janus激酶抑制剂）、未来治疗方向、社会心理管理、远程医疗、诊断沟通与遗传咨询、产前诊断及胚胎植入前遗传学检测。