Aquagenic wrinkling of the palms in cystic fibrosis patients treated with ivacaftor

•Aquagenic wrinkling of palms (AWP) is common in people with CF and gating mutations.•Ivacaftor therapy results in an improvement in AWP over time.•CFTR dysfunction contributes to development of AWP.•AWP may serve as a simple screening test for CF. Aquagenic wrinkling of palms (AWP) in cystic fibrosis (CF) patients and common CFTR mutations is recognized as a frequent symptom of the disease. The long-term effect of CFTR targeting therapy on AWP has not been studied. AWP was monitored in 16 CF patients (8 children and 8 adults) before and for 6 months after initiation of ivacaftor therapy. Thirteen (81.3%) patients had at least mild and 8/16 (50%) moderate-to-severe AWP at baseline. AWP improved with ivacaftor therapy. This observation suggests that AWP is also common in individuals with CF and relatively rare mutations and is directly related to CFTR function.