title: Aquagenic wrinkling of the palms and cystic fibrosis: an Italian study with controls and genotype-phenotype correlations.
abstract: OBJECTIVE
  The primary aim was to determine the prevalence of aquagenic wrinkling of the palms (AWP) in patients with cystic fibrosis (CF) compared to controls, and secondarily to evaluate genotype-phenotype correlations among CF subjects found to have AWP.
  
  
  METHODS
  Fifty-eight patients with CF underwent a hand immersion test in tap water. Twenty-three of their CF carrier relatives and 7 subjects with a negative genetic test for CF were recruited as controls. Secondary analyses explored associations with genotype, pulmonary function, and sweat electrolyte levels in all subjects with and without AWP. Additional information about atopic diathesis, hyperhidrosis of the palms and drug intake were also collected.
  
  
  RESULTS
  Thirty-one of the patients with CF (53.4%) exhibited AWP, in contrast to only 2 carriers (8.7%) and none in the control group. No correlation was found between CF genotype and AWP score severity. Twenty-three (39.7%) CF patients reported a history of hyperhidrosis, and in 17 of them (74%) AWP had been provoked. No correlation with history of atopy and lung function was noted. The difference between CF patients with hyperhidrosis and those without was highly significant (p = 0.016). Salt concentrations were significantly higher in patients with AWP.
  
  
  CONCLUSIONS
  AWP is linked to CF and its diagnosis should lead the patients to a genetic or sweat test for CF. We found a significant association with hyperhidrosis and sweat electrolytes which supports the 'hyperconcentrated sweat' pathogenetic theory of AWP.
authors.affiliation: Section of Dermatology, DISSAL, University of Genoa, Genoa, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Chinazzo
authors.forename: A
authors.initials: A
authors.lastname: De Alessandri
authors.forename: S
authors.initials: S
authors.lastname: Menoni
authors.forename: G
authors.initials: G
authors.lastname: Romanisio
authors.forename: A
authors.initials: A
authors.lastname: Rebora
authors.forename: F
authors.initials: F
authors.lastname: Rongioletti
mesh_terms: D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000235:genetics* / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D006225:Hand / Q000503:physiopathology*; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D006945:Hyperhidrosis / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D007101:Immersion / Q000503:physiopathology*; D007558:Italy; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010641:Phenotype; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D012965:Sodium Chloride / Q000032:analysis; D013542:Sweat / Q000737:chemistry; D014867:Water; D055815:Young Adult
chemical_list: D014867:Water; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D012965:Sodium Chloride