Management of congenital ichthyoses: guidelines of care: Part one: 2024 update

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.

Congenital ichthyoses are a group of rare skin diseases. In 2019, clinical experts released guidelines for treating them. The team of experts updates these guidelines every 5 years. This ensures they include advancements in treatments and understanding of the diseases. An international team of experts carried out the latest update in 2023, in two parts. In this Part one, we focus on providing recommendations for different treatments options. These treatments include both oral and injected medications. The team reports Part two separately. Part one also covers future treatment options. These include managing the psychological and social impacts of living with a rare skin disease. This part also covers telemedicine, and how to communicate diagnoses and genetic information. Finally, this part addresses genetic testing before birth and before implantation.

Published by The British journal of dermatology, 2025. The guideline itself is at the source linked here.

doi.org/10.1093/bjd/ljaf076