title: Influence of meteorological data on sun tolerance in patients with erythropoietic protoporphyria in France.
abstract: BACKGROUND
  Erythropoietic protoporphyria (EPP) is a rare metabolic disorder, characterized by photosensitivity, caused by errors of the haem biosynthetic pathway. Avoidance of sun exposure is recommended; however, some patients suggested a paradoxical improvement of symptoms when they move to sunny areas.
  
  
  OBJECTIVE
  In a national French study, we sought to investigate the influence of sun exposure on EPP symptoms.
  
  
  METHODS
  We used a national transversal observational study by questionnaire. Patients were selected from the national record of the Centre Français des Porphyries (French Porphyrias referral centre). Sun exposure level by geographic area was assessed using climate data provided by the French national meteorological service (Météo France).
  
  
  RESULTS
  Eighty-nine patients were included. We notably observed that 40% of patients declared an improvement in their tolerance of sun exposure after repeated sun exposures. In the more sunny areas, the intensity of the pain was lower (r = -0·26) and the duration of the sun exposure responsible for flares was longer (r = 0·39) than in the areas that were less sunny (P < 0·05).
  
  
  CONCLUSIONS
  This study proposes a benefit of natural progressive sun exposure for patients with EPP.
authors.affiliation: Department of Dermatology, Centre Hospitalier Universitaire (CHU) Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093, Nantes Cedex 1, France.
authors.forename: S
authors.initials: S
authors.lastname: de Bataille
authors.affiliation: Department of Dermatology, Centre Hospitalier Universitaire (CHU) Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093, Nantes Cedex 1, France.
authors.forename: H
authors.initials: H
authors.lastname: Dutartre
authors.affiliation: Centre Français des Porphyries, Hôpital Louis-Mourier Assistance Publique Hôpitaux de Paris, Colombes, France.|INSERM U1149, Centre de Recherche sur l'inflammation, 16 rue Henri Huchard, 75018, Paris, France.|Laboratory of Excellence, GR-Ex, Paris, France.
authors.forename: H
authors.initials: H
authors.lastname: Puy
authors.affiliation: Centre Français des Porphyries, Hôpital Louis-Mourier Assistance Publique Hôpitaux de Paris, Colombes, France.
authors.forename: J-C
authors.initials: JC
authors.lastname: Deybach
authors.affiliation: Centre Français des Porphyries, Hôpital Louis-Mourier Assistance Publique Hôpitaux de Paris, Colombes, France.|INSERM U1149, Centre de Recherche sur l'inflammation, 16 rue Henri Huchard, 75018, Paris, France.|Laboratory of Excellence, GR-Ex, Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Gouya
authors.affiliation: Department of Biostatistics, CHU Nantes, Nantes, France.
authors.forename: E
authors.initials: E
authors.lastname: Raffray
authors.affiliation: French national meteorological service, Météo France, Toulouse, France.
authors.forename: M
authors.initials: M
authors.lastname: Pithon
authors.affiliation: Department of Dermatology, Centre Hospitalier Universitaire (CHU) Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093, Nantes Cedex 1, France.
authors.forename: J-F
authors.initials: JF
authors.lastname: Stalder
authors.affiliation: Department of Biostatistics, CHU Nantes, Nantes, France.
authors.forename: J-M
authors.initials: JM
authors.lastname: Nguyen
authors.affiliation: Department of Dermatology, Centre Hospitalier Universitaire (CHU) Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093, Nantes Cedex 1, France. sebastien.barbarot@chu-nantes.fr.
authors.forename: S
authors.initials: S
authors.lastname: Barbarot
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D003020:Clothing; D004781:Environmental Exposure / Q000706:statistics & numerical data; D005260:Female; D005602:France / Q000453:epidemiology; D006370:Heliotherapy / Q000379:methods; D006801:Humans; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000517:prevention & control; D010787:Photosensitivity Disorders / Q000453:epidemiology* / Q000517:prevention & control; D046351:Protoporphyria, Erythropoietic / Q000453:epidemiology* / Q000517:prevention & control; D013472:Sunlight; D013473:Sunscreening Agents / Q000627:therapeutic use; D014887:Weather; D055815:Young Adult
chemical_list: D013473:Sunscreening Agents