title: Prevalence of inherited ichthyosis in France: a study using capture-recapture method.
abstract: 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.
  
  
  METHODS
  Capture - recapture method was used for this study. According to statistical requirements, 3 different lists (reference/competence centres, French association of patients with ichthyosis and internet network) were used to record such patients. The study was conducted in 5 areas during a closed period.
  
  
  RESULTS
  The prevalence was estimated at 13.3 per million people (/M) (CI95%, [10.9 - 17.6]). With regard to autosomal recessive congenital ichthyosis, the prevalence was estimated at 7/M (CI 95% [5.7 - 9.2]), with a prevalence of lamellar ichthyosis and congenital ichthyosiform erythroderma of 4.5/M (CI 95% [3.7 - 5.9]) and 1.9/M (CI 95% [1.6 - 2.6]), respectively. Prevalence of keratinopathic forms was estimated at 1.1/M (CI 95% [0.9 - 1.5]). Prevalence of syndromic forms (all clinical forms together) was estimated at 1.9/M (CI 95% [1.6 - 2.6]).
  
  
  CONCLUSIONS
  Our results constitute a crucial basis to properly size the necessary health measures that are required to improve patient care and design further clinical studies.
authors.affiliation: Reference Centre for Rare Skin Diseases, Dermatology Department, Larrey Hospital, CHU Toulouse, Toulouse, France. dreyfus.i@chu-toulouse.fr.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Dreyfus
authors.forename: Cécile
authors.initials: C
authors.lastname: Chouquet
authors.forename: Khaled
authors.initials: K
authors.lastname: Ezzedine
authors.forename: Sophie
authors.initials: S
authors.lastname: Henner
authors.forename: Christine
authors.initials: C
authors.lastname: Chiavérini
authors.forename: Aude
authors.initials: A
authors.lastname: Maza
authors.forename: Sandrine
authors.initials: S
authors.lastname: Pascal
authors.forename: Lauriane
authors.initials: L
authors.lastname: Rodriguez
authors.forename: Pierre
authors.initials: P
authors.lastname: Vabres
authors.forename: Ludovic
authors.initials: L
authors.lastname: Martin
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Mallet
authors.forename: Sébastien
authors.initials: S
authors.lastname: Barbarot
authors.forename: Jérôme
authors.initials: J
authors.lastname: Dupuis
authors.forename: Juliette
authors.initials: J
authors.lastname: Mazereeuw-Hautier
mesh_terms: D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D007057:Ichthyosis / Q000453:epidemiology*; D008297:Male; D015995:Prevalence