Management of congenital ichthyoses: guidelines of care: Part two: 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 after a systematic review of recent literature, discussions and consensus reached at an expert conference held in June 2023.

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.

cLFM-Qol: A specific quality of life measurement tool for children from 11 to 15 years with low-flow malformations.

Background: Low-flow malformations (LFMs) are rare diseases with a significant impact on health-related quality of life (HRQoL), especially in children. No disease-specific questionnaire is available for children with LFMs.

Objective: To develop and validate a specific HRQoL questionnaire for children from 11 to 15 years old suffering from LFMs.

Why is it so difficult for GPs to effectively manage patients with rare skin diseases?

Background: Rare diseases are defined by a prevalence of less than one out of 2000 persons. In clinical practice, their management is difficult, due to their diversity, their complexity and a lack of adapted physician training.

Objective: The aims of this study were to identify rare skin diseases in a reference center, to describe the difficulties encountered by general practitioners (GPs) in management of these uncommon cases, and to pinpoint the characteristics of the GPs having the most problems.

Childhood pilomatricomas: Associated anomalies.

Pilomatricoma is a common benign tumor in children. We present a review of the literature with the aim of helping clinicians manage these patients. A detailed review of the literature was performed in the PubMed database using an exhaustive list of Medical Subject Heading words.

Efficacy of an interventional educational programme in mitigating post-traumatic stress in parents who have witnessed a febrile seizure: a pilot before-and-after study.

Objectives: To measure post-traumatic stress in parents who have witnessed their child's first simple febrile seizure and to assess the impact of workshops where information is dispensed, proper reactions are demonstrated and dialogue is encouraged on the mitigation of parental stress.

Design: A pilot before-and-after study, with control group, using self-reported measures from the Impact of Event Scale-Revised.

Setting: Data from two French participating centres.

Alitretinoin reduces erythema in inherited ichthyosis.

Background: Acitretin is the main retinoid used to treat severe inherited ichthyosis. Alternatives may be considered if it results ineffective or there are side-effects, or for women of childbearing age. Our objective is evaluation of the effects and tolerance of alitretinoin.

Burden of inherited ichthyosis: a French national survey.

Moderate to severe ichthyosis is known to have a significant impact on quality of life. A French national survey was performed to describe in more detail how ichthyosis impacts the patients' lives. A questionnaire specifically dedicated to ichthyosis was distributed to patients followed in hospital expert centres or members of the French association of patients.

Prevalence and risk factors of vitamin D deficiency in inherited ichthyosis: a French prospective observational study performed in a reference center.

Background: To date, few studies have investigated serum vitamin D status in patients with inherited ichthyosis. The aim of this study was to determine the prevalence of vitamin D deficiency (defined as serum level <10 ng/mL) in a French cohort of patients and to identify associated risk factors.

Methods: This was a prospective observational study performed in a hospital reference center with expertise for rare skin diseases.

Prevalence of inherited ichthyosis in France: a study using capture-recapture method.

Background: Inherited ichthyoses represent a group of rare skin disorders characterized by scaling, hyperkeratosis and inconstant erythema, involving most of the tegument. Epidemiology remains poorly described. This study aims to evaluate the prevalence of inherited ichthyosis (excluding very mild forms) and its different clinical forms in France.

IQoL-32: a new ichthyosis-specific measure of quality of life.

Background: Inherited ichthyoses are associated with impaired quality of life (QoL).

Objectives: The aim of this study was to create and validate a QoL questionnaire specifically dedicated to patients with ichthyosis.

Methods: A prequestionnaire was drawn after selecting items from a verbatim transcript.

Desloratadine for the treatment of cypress pollen-induced allergic rhinitis.

Background: Few studies have been conducted to assess treatment options for patients with sensitivities to cypress pollens, important triggers of allergic rhinitis (AR) in the Mediterranean region.

Objective: To evaluate the effect of desloratadine, a second-generation antihistamine, on AR symptoms caused by cypress pollens native to France.

Methods: Adults (N=233) with symptomatic cypress pollen allergies were randomized to receive desloratadine, 5 mg, or placebo daily for 15 days during 2 consecutive cypress pollen seasons.

Efficacy and safety of desloratadine in adults with chronic idiopathic urticaria: a randomized, double-blind, placebo-controlled, multicenter trial.

Background And Objective: Chronic idiopathic urticaria (CIU), a condition characterized by pruritus and wheals, can cause patients physical and psychological distress. Desloratadine, a second-generation histamine H(1) receptor antagonist (antihistamine), is a first-line treatment option for CIU. The objective of this study was to evaluate the efficacy and safety of once-daily desloratadine 5mg versus placebo for the treatment of CIU symptoms and disease severity in adults.