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Practice Guideline
. 2025 Jun 20;193(1):28-43.
doi: 10.1093/bjd/ljaf077.

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

Juliette Mazereeuw-Hautier  1 , Amy S Paller, Edel O'Toole, Isabelle Dreyfus  1 , Christine Bodemer, Masashi Akiyama, Andrea Diociaiuti, Maya El Hachem, Judith Fischer, Rogelio Gonzalez-Sarmiento, Carlos Gutiérrez-Cerrajero, Hagen Ott, Cristina Has, Nathalie Jonca, Céline Granier Tournier  1 , Ana Martinez, Heiko Traupe, Carmen Maria Salavastru, Matthias Schmuth, Eli Sprecher, Kathrin Giehl, Mandy Aldwin, Ruth Anton Morales, Saturnino Santos, Marie-Anne Morren, Anne Audouze, Raman Malhotra, Karin Veldman, Joanna Narbutt, Kira Süßmuth, Antoni Gostynski, Angela Hernandez-Martin
Affiliations
Practice Guideline

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

Juliette Mazereeuw-Hautier et al. Br J Dermatol. .

Abstract

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 after 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. Part one is reported elsewhere. Here, Part two covers the management of complications (eye, ear-nose-throat, pruritus, pain, cutaneous infections, vaccinations, growth failure and nutritional deficiency, hair and nail anomalies, reaction to hot and cold climates, physical limitations, comorbidities) and the particularities of the neonatal period and Netherton syndrome.

Plain language summary

Congenital ichthyoses (pronounced ‘ik-thee-oh-sis’) are a rare group of genetic skin disorders. In 2019, a group of experts published guidelines on how to treat these disorders. The guidelines are updated every 5 years to include treatment advances and new findings. The latest update was prepared in 2023 by a team of international experts. The updated guidelines are published in two parts. This paper covers the second part of the update. Part one is published separately. In Part two, we make recommendations for how to manage complications in these rare skin disorders. Complications can include issues with the eyes, ears, nose, throat, itching, pain, skin infections, growth, nutrition, hair, nails, climate sensitivity, physical limitations and other health problems. Part two also covers special considerations for newborn babies. We also discuss how to treat a rare subtype called Netherton syndrome.

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Conflict of interest statement

Conflicts of interest: JM-H has been an investigator for the last 5 years for companies relevant to ichthyosis: LEO Pharma, Lilly, Mayne, Sanofi and Timber Pharmaceuticals. ASP is an investigator for Biomendics, Janssen, Regeneron and Timber, and a consultant for BioCryst, Boehringer Ingelheim, LEO and Regeneron/Sanofi relevant to ichthyosis. ES is a consultant for Kamari, BiomX, Bayer, Amryt, Medison, SolGel, Krystal, BiondVax, Galmed, Laser Team, IDERM and PINCELL. AD has been a consultant for Amryt Pharma/Chiesi (not related with ichthyosis) and Novartis (not directly related with ichthyosis). MEH is a consultant for Chiesi and Novartis, not related to ichthyosis. AM has done consultancy work with Krystal Bio and Amryt Pharma. KS is medical advisor for Nia Health GmbH, has received a fund for ‘Innovative Medical Research’ from the University of Münster Medical School, has received a clinician scientist program grant from the Deutsche Dermatologische Gesellschaft (DDG) and Arbeitsgemeinschaft Dermatologische Forschung (ADF), has received honoraria for presentations for Julius Zorn GmbH (Juzo) and Sanofi, and is a member of the DDG, ADF and Arbeitsgemeinschaft Pädiatrische Dermatologie. AH-M is a consultant for Sanofi but has no conflicts of interest relevant to ichthyosis. The other authors have no conflicts of interest to declare.

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