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

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.
Competing Interests: Declaration of competing interest Felix Ratjen received research grants from Vertex Pharmaceuticals on the original ivacaftor study from which patients were recruited. There was no direct funding for this research. Melinda Solomon received consultation fees from Vertex Pharmaceuticals. Parts of this study were supported by investigator-initiated study grants from Vertex Pharmaceuticals to Tanja Gonska and Hartmut Grasemann. The remaining authors have nothing to disclose.
(Copyright © 2022 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)