Management of congenital ichthyoses: European guidelines of care, part one

Abstract

These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016 and a consensus on the discussions. They summarize evidence and expert-based recommendations and are intended to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part one, covering topical therapies, systemic therapies, psychosocial management, communicating the diagnosis and genetic counselling.

Management of congenital ichthyoses: European guidelines of care, part two

“…In clinical practice retinoids are recommended as a second‐line therapy in combination with topical agents, in order to reduce moderate‐to‐severe ectropion and prevent further worsening (LoE 3, GoR B). However, oral retinoids may induce ophthalmic side‐effects such as dry eyes (see part one) …”

Consensus, collaboration and care coordination

“…The essential part of these exercises is what ultimately has an impact on patient care. Certainly, gatherings of experts, such as in the case of the authors of the article in this issue of the BJD , with sharing of years of collective clinical experience and a careful review of published literature is important . However, ultimately, such work should also be guided by those patients and families who, in this case, ichthyosis has an impact on in addition to (later) evaluation of the outcome of such interventions.…”

“…In the case of complex conditions such as ichthyosis, interdisciplinary care with coordination between clinicians and the patients is key. This is stressed by the authors of the article . Unfortunately, so often we do not work this way and the care of the patient ultimately suffers.…”

“…The specific aspects of clinical care are well reviewed by the authors . Of importance, the true utility and consequences of such clinical interventions are ‘a work in progress’ in spite of years of experience.…”

“…Health care, overall, is evolving. This article is one such example of a careful examination of the management of ichthyosis . A major goal of this work, as noted above, is to assess these prospectively over several years.…”