Aquagenic Wrinkling of the Palms: A Potential Sign of Systemic Treatment Response to Elexacaftor-Tezacaftor-Ivacaftor

Abstract

Cystic Fibrosis is a life altering disease resulting from a mutated gene and ion channel. It is a systemic condition that affects multiple body systems including the skin. Aquagenic wrinkling of the palms (AWP) is the rapid formation of white plaques on the palms after exposure to water and is commonly seen in patients with cystic fibrosis. Numerous theories exist to explain this pathophysiology however no one explanation has been able to characterize it completely. Our patient is a 25-year-old with a past medical history of cystic fibrosis and AWP who was started on Trikafta® (elexacaftor-tezacaftor-ivacaftor). Shortly after she started treatment she observed near total resolution of her AWP. This is the second documented case of this phenomenon and may be a marker for systemic improvement and response to this drug.