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

Eyal Jacobi; Melinda Solomon; Julie Avolio; Michelle Shaw; Tanja Gonska; Felix Ratjen; Hartmut Grasemann

doi:10.1016/j.jcf.2022.01.005

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

J Cyst Fibros. 2022 Mar;21(2):e102-e105. doi: 10.1016/j.jcf.2022.01.005. Epub 2022 Jan 19.

Authors

Eyal Jacobi¹, Melinda Solomon¹, Julie Avolio¹, Michelle Shaw¹, Tanja Gonska², Felix Ratjen³, Hartmut Grasemann⁴

Affiliations

¹ Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
² Division of Gastroenterology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Translational Medicine, Research Institute, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
³ Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Translational Medicine, Research Institute, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
⁴ Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Translational Medicine, Research Institute, The Hospital for Sick Children, University of Toronto, Toronto, Canada. Electronic address: [email protected].

PMID: 35063397
DOI: 10.1016/j.jcf.2022.01.005

Abstract

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.

Keywords: Aquagenic Wrinkling; Cystic fibrosis; Ivacaftor.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Aminophenols / pharmacology
Aminophenols / therapeutic use
Child
Cystic Fibrosis Transmembrane Conductance Regulator / genetics
Cystic Fibrosis* / complications
Cystic Fibrosis* / drug therapy
Cystic Fibrosis* / genetics
Humans
Mutation
Quinolones
Skin Aging / drug effects
Skin Aging / genetics
Water / adverse effects

Substances

Aminophenols
Quinolones
Water
Cystic Fibrosis Transmembrane Conductance Regulator
ivacaftor