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May 2005

Aquagenic Wrinkling of the Palms in Patients With Cystic Fibrosis Homozygous for the ΔF508 CFTR Mutation

Author Affiliations

Author Affiliations: Department of Dermatology, University of Pennsylvania, Philadelphia (Dr Katz); Department of Pediatrics and Dermatology, Section of Pediatric Dermatology, Children’s Hospital of Philadelphia and the University of Pennsylvania School of Medicine (Dr Yan); and Dermatology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Md (Dr Turner).

Arch Dermatol. 2005;141(5):621-624. doi:10.1001/archderm.141.5.621

Background  Aquagenic wrinkling of the palms (AWP) is a rare condition characterized by the rapid and transient formation of edematous whitish plaques on the palms on exposure to water (the so-called hand-in-the-bucket sign). The changes may be asymptomatic or accompanied by pruritic or burning sensations. First described in 1974 in patients with cystic fibrosis—and still primarily reported in these patients—this condition has been previously described only in females. Specific mutations in CFTR, the gene responsible for cystic fibrosis, have not been reported previously in patients with AWP.

Observations  We describe 2 patients with AWP, both of whom are homozygous for the ΔF508 mutation in CFTR: a 17-year-old boy—the first male reported to have this condition—who has had AWP for 3 years and a 13-year-old girl who has had AWP for 6 months.

Conclusions  Cystic fibrosis should be considered in patients with AWP, and patients with cystic fibrosis should be asked about symptoms of this condition. Although the etiology of AWP is unknown, the association with cystic fibrosis, and with marasmus and cyclooxygenase-2 inhibitors, suggests that exposure of the skin to abnormally high concentrations of salt may play a role in its pathogenesis.