Aquagenic Wrinkling Of The Palms in Cystic Fibrosis And The Cystic Fibrosis Carrier State - A Case Control Study.

Br J Dermatol. 2010 Jun 18.

Aquagenic Wrinkling Of The Palms in Cystic Fibrosis And The Cystic Fibrosis Carrier State - A Case Control Study.

Gild R, Clay C, Morey S.

Princess Alexandra Hospital, Department of Dermatology, 199 Ipswich Road, Woolloongabba, Brisbane, Queensland 4102, Australia.

Abstract

Abstract Background Aquagenic wrinkling of the palms is hyperwrinkling occurring within three minutes of exposure to water. It is associated with cystic fibrosis and has been reported in a cystic fibrosis carrier.

Objectives

To ascertain if aquagenic wrinkling of the palms is a sign of the cystic fibrosis carrier state and to test for an association between Cystic Fibrosis Transmembrane Regulator protein function and time to wrinkling. Patients/Methods 21 patients, 13 carriers and 15 controls were recruited. Hands were immersed in water and time to wrinkling was measured.

An analysis of variance was performed with mean time to wrinkling as the dependent variable and cystic fibrosis status as the independent variable. Patients with a time to wrinkling of less than or equal to three minutes were defined as having aquagenic wrinkling. A test of proportions was performed to assess if the proportion of patients with aquagenic wrinkling varied between groups.

Results

Mean time to wrinkling was 11 minutes in controls, 7 minutes in carriers and 2 minutes in cystic fibrosis patients. Aquagenic wrinkling of the palms was not seen in controls, but occurred in 80% of cystic fibrosis patients and 25% of carriers. There was a significant difference between groups. (p<0.0001) Conclusions The study demonstrated that aquagenic palmar wrinkling is a sign of both cystic fibrosis and the carrier state. It suggests that time to wrinkling decreases with decreased cystic fibrosis transmembrane regulator protein function. Patients presenting with aquagenic wrinkling should be offered screening for both cystic fibrosis and the carrier state.

PMID: 20560957 [PubMed - as supplied by publisher]