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      Two Cases of Aquagenic Wrinkling of the Palms in Korean Healthy Women: Easily Overlooked Disease

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          Abstract

          Aquagenic wrinkling of the palms (AWP) is a rare condition, which is characterized by appearance of whitish papules and plaques and an excessive wrinkling and swelling of the palmar skin after exposure to water. Herein, we report two cases of AWP. A 17-year-old woman presented prickling focal various sized whitish papules, plaques with wrinkles on both palms after water contact for 6 months ago. Histologic findings were consistent with AWP. A 26-year-old woman presented asymptomatic multiple various sized whitish papules, plaques with wrinkles on both palms after water contact for 1 year ago. Both patients underwent a cystic fibrosis transmembrane conduct receptor test but were negative and improved without any treatment.

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          Most cited references15

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          A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

          Increasing the activity of defective cystic fibrosis transmembrane conductance regulator (CFTR) protein is a potential treatment for cystic fibrosis. We conducted a randomized, double-blind, placebo-controlled trial to evaluate ivacaftor (VX-770), a CFTR potentiator, in subjects 12 years of age or older with cystic fibrosis and at least one G551D-CFTR mutation. Subjects were randomly assigned to receive 150 mg of ivacaftor every 12 hours (84 subjects, of whom 83 received at least one dose) or placebo (83, of whom 78 received at least one dose) for 48 weeks. The primary end point was the estimated mean change from baseline through week 24 in the percent of predicted forced expiratory volume in 1 second (FEV(1)). The change from baseline through week 24 in the percent of predicted FEV(1) was greater by 10.6 percentage points in the ivacaftor group than in the placebo group (P<0.001). Effects on pulmonary function were noted by 2 weeks, and a significant treatment effect was maintained through week 48. Subjects receiving ivacaftor were 55% less likely to have a pulmonary exacerbation than were patients receiving placebo, through week 48 (P<0.001). In addition, through week 48, subjects in the ivacaftor group scored 8.6 points higher than did subjects in the placebo group on the respiratory-symptoms domain of the Cystic Fibrosis Questionnaire-revised instrument (a 100-point scale, with higher numbers indicating a lower effect of symptoms on the patient's quality of life) (P<0.001). By 48 weeks, patients treated with ivacaftor had gained, on average, 2.7 kg more weight than had patients receiving placebo (P<0.001). The change from baseline through week 48 in the concentration of sweat chloride, a measure of CFTR activity, with ivacaftor as compared with placebo was -48.1 mmol per liter (P<0.001). The incidence of adverse events was similar with ivacaftor and placebo, with a lower proportion of serious adverse events with ivacaftor than with placebo (24% vs. 42%). Ivacaftor was associated with improvements in lung function at 2 weeks that were sustained through 48 weeks. Substantial improvements were also observed in the risk of pulmonary exacerbations, patient-reported respiratory symptoms, weight, and concentration of sweat chloride. (Funded by Vertex Pharmaceuticals and others; VX08-770-102 ClinicalTrials.gov number, NCT00909532.).
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            Unilateral aquagenic wrinkling of the palms associated with aspirin intake.

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              Aberrant aquaporin 5 expression in the sweat gland in aquagenic wrinkling of the palms.

              Aquagenic wrinkling of the palms (AWP) is an uncommon disease characterized by the rapid and transient formation of edematous whitish plaques on the palms on exposure to water. Although this disease is occasionally accompanied by hyperhidrosis, the pathophysiology of AWP remains unknown. Herein we describe a patient with AWP. The location of wrinkling was limited to the areas positive for iodine-starch test after water exposure, which suggests that AWP is etiologically related to hyperhidrosis. Histologic examination revealed hyperplastic and papillated eccrine glandular epithelium with the enlarged diameter of eccrine coils. Immunohistochemically, while aquaporin 5 (AQP5), one of the water channel AQP families, was present exclusively in the dark cells of sweat glands of healthy donors, an aberrant AQP5 staining, extending to the clear cells, was found in the patient with AWP. The hyperplastic glandular epithelium and aberrant AQP5 staining in the patient's sweat glands suggest that AWP stems from dysregulation of sweating.
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                Author and article information

                Journal
                Ann Dermatol
                Ann Dermatol
                AD
                Annals of Dermatology
                The Korean Dermatological Association; The Korean Society for Investigative Dermatology
                1013-9087
                2005-3894
                April 2020
                11 March 2020
                : 32
                : 2
                : 151-154
                Affiliations
                Department of Dermatology, Chosun University Medical School, Gwangju, Korea.
                Author notes
                Corresponding author: Bong Seok Shin, Department of Dermatology, Chosun University Medical School, 365 Pilmun-daero, Dong-gu, Gwangju 61453, Korea. Tel: 82-62-220-3130, Fax: 82-62-222-3215, inho-yoga@ 123456hanmail.net
                Author information
                https://orcid.org/0000-0001-8514-3550
                https://orcid.org/0000-0002-9166-4050
                https://orcid.org/0000-0002-8102-6653
                https://orcid.org/0000-0001-5259-5382
                https://orcid.org/0000-0001-9618-1763
                Article
                10.5021/ad.2020.32.2.151
                7992540
                33911727
                9445214d-2046-4e11-b2a1-2fe24c238971
                Copyright © 2020 The Korean Dermatological Association and The Korean Society for Investigative Dermatology

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 11 September 2018
                : 10 December 2018
                : 02 January 2019
                Categories
                Case Report

                Dermatology
                aquagenic wrinkling of the palms,cystic fibrosis
                Dermatology
                aquagenic wrinkling of the palms, cystic fibrosis

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