R Gild1, C D Clay, S Morey. 1. Department of Dermatology, Princess Alexandra Hospital, Woolloongabba, Brisbane, Qld 4102, Australia. osroch@graduate.uwa.edu.au
Abstract
BACKGROUND: Aquagenic wrinkling of the palms (AWP) is hyperwrinkling occurring within 3 min of exposure to water. It is associated with cystic fibrosis (CF) and has been reported in a CF carrier. OBJECTIVES: To ascertain if AWP is a sign of the CF carrier state and to test for an association between cystic fibrosis transmembrane regulator (CFTR) protein function and time to wrinkling. METHODS: Twenty-one 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 CF status as the independent variable. Patients with a time to wrinkling of ≤ 3 min were defined as having AWP. A test of proportions was performed to assess if the proportion of patients with AWP varied between groups. RESULTS: Mean time to wrinkling was 11 min in controls, 7 min in carriers and 2 min in patients with CF. AWP was not seen in controls, but occurred in 80% of patients with CF and 25% of carriers. There was a significant difference between groups (P < 0·001). CONCLUSIONS: The study demonstrated that AWP is a sign of both CF and the carrier state. It suggests that time to wrinkling decreases with decreased CFTR protein function. Patients presenting with AWP should be offered screening for both CF and the carrier state.
BACKGROUND: Aquagenic wrinkling of the palms (AWP) is hyperwrinkling occurring within 3 min of exposure to water. It is associated with cystic fibrosis (CF) and has been reported in a CF carrier. OBJECTIVES: To ascertain if AWP is a sign of the CF carrier state and to test for an association between cystic fibrosis transmembrane regulator (CFTR) protein function and time to wrinkling. METHODS: Twenty-one 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 CF status as the independent variable. Patients with a time to wrinkling of ≤ 3 min were defined as having AWP. A test of proportions was performed to assess if the proportion of patients with AWP varied between groups. RESULTS: Mean time to wrinkling was 11 min in controls, 7 min in carriers and 2 min in patients with CF. AWP was not seen in controls, but occurred in 80% of patients with CF and 25% of carriers. There was a significant difference between groups (P < 0·001). CONCLUSIONS: The study demonstrated that AWP is a sign of both CF and the carrier state. It suggests that time to wrinkling decreases with decreased CFTR protein function. Patients presenting with AWP should be offered screening for both CF and the carrier state.
Authors: Leah R Reznikov; Qian Dong; Jeng-Haur Chen; Thomas O Moninger; Jung Min Park; Yuzhou Zhang; Jianyang Du; Michael S Hildebrand; Richard J H Smith; Christoph O Randak; David A Stoltz; Michael J Welsh Journal: Proc Natl Acad Sci U S A Date: 2013-02-04 Impact factor: 11.205