• David R Berk, Heather M Ciliberto, Stuart C Sweet, Thomas W Ferkol, and Susan J Bayliss.
• Department of Internal Medicine and Pediatrics, Division of Dermatology, Washington University School of Medicine and St Louis Children's Hospital, Campus Box 8123, 4921 Parkview Place, St Louis, MO 63110, USA. dberk@dom.wust.edu
• Arch Dermatol. 2009 Nov 1;145(11):1296-9.

ObjectiveTo determine the prevalence of aquagenic wrinkling of the palms (AWP) in patients with cystic fibrosis (CF) compared with control patients, and evaluate for genotype-phenotype correlations. Since its first description over 30 years ago, AWP has frequently been anecdotally associated with CF, but this association has not been confirmed in a rigorous prospective case-control study.DesignBlinded comparison.SettingThe CF and dermatology clinics at St Louis Children's Hospital.ParticipantsForty-four individuals with CF from a CF clinic and 26 controls from a dermatology clinic. Intervention Participants were tested for AWP using 3 minutes of water immersion with room-temperature tap water. Main Outcome Measure The degree of AWP was scored from 0 (no wrinkling) to 4 (severe wrinkling) by 3 blinded physicians. For genotype-phenotype correlations, patients with CF were divided into those homozygous for the DeltaF508 mutation and those with other genotypes.ResultsThe mean AWP score of the CF group was significantly higher than the mean score of the control group (1.5 vs 0.6; P < .001). Patients with CF who were homozygous for the DeltaF508 mutation (n = 27) had significantly higher scores than patients with CF who were not homozygous for the DeltaF508 mutation (n = 17) (1.7 vs 1.1; P = .02). The 17 patients with CF who were not homozygous for the DeltaF508 mutation still had higher scores than the control group (1.1 vs 0.6; P = .03). There was no correlation between sweat chloride concentrations measured at the time of diagnosis and AWP score.ConclusionsOur results confirm the association between AWP and CF. Among patients with CF, greater AWP occurs in those who are homozygous for the DeltaF508 mutation.

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