Aquagenic palmar wrinkling

Again, I think we as a CF community are so fortunate to have Mandy and all of her amazing research around treatments for CF. I have learned so much from her and this post is another testament to her dedication to the lives of all CFers.

As many of you know, I have been drinking green smoothies in an attempt to get more greens in my diet - I truly eat like a crazy teenager, which I know is not good for my health. I really love the smoothies and they taste wonderful.

A benefit I didn't realize I could expect from these wonderful smoothies is my aquagenic palmar wrinkling has disappeared. It took about 4 weeks of drinking the smoothies everyday, but it worked!

Now what exactly the health consequences are of this, we aren't sure. But we do know that those with CF and those who are CFTR carriers wrinkle much faster when submerged in water.

I'll continue to keep you all posted on any other exciting changes!

Arch Dermatol. 2009 Nov;145(11):1296-9.

Aquagenic wrinkling of the palms in cystic fibrosis: comparison with controls and genotype-phenotype correlations.

Berk DR, Ciliberto HM, Sweet SC, Ferkol TW, Bayliss SJ.

Department of Internal Medicine and Pediatrics, Division of Dermatology, Washington University School of Medicine and St Louis Children's Hospital, Campus Box 8123, 4921 Parkview Place, St Louis, MO 63110, USA. dberk@dom.wust.edu

OBJECTIVE: To determine the prevalence of aquagenic wrinkling of the palms (AWP) in patients with cystic fibrosis (CF) compared with control patients, and evaluate for genotype-phenotype correlations.

Since its first description over 30 years ago, AWP has frequently been anecdotally associated with CF, but this association has not been confirmed in a rigorous prospective case-control study.

DESIGN: Blinded comparison.

SETTING: The CF and dermatology clinics at St Louis Children's Hospital.

PARTICIPANTS: Forty-four individuals with CF from a CF clinic and 26 controls from a dermatology clinic. Intervention Participants were tested for AWP using 3 minutes of water immersion with room-temperature tap water.

Main Outcome Measure The degree of AWP was scored from 0 (no wrinkling) to 4 (severe wrinkling) by 3 blinded physicians. F

or genotype-phenotype correlations, patients with CF were divided into those homozygous for the DeltaF508 mutation and those with other genotypes.

RESULTS: The mean AWP score of the CF group was significantly higher than the mean score of the control group (1.5 vs 0.6; P < .001). Patients with CF who were homozygous for the DeltaF508 mutation (n = 27) had significantly higher scores than patients with CF who were not homozygous for the DeltaF508 mutation (n = 17) (1.7 vs 1.1; P = .02). The 17 patients with CF who were not homozygous for the DeltaF508 mutation still had higher scores than the control group (1.1 vs 0.6; P = .03). There was no correlation between sweat chloride concentrations measured at the time of diagnosis and AWP score.

CONCLUSIONS: Our results confirm the association between AWP and CF. Among patients with CF, greater AWP occurs in those who are homozygous for the DeltaF508 mutation.

Aquagenic palmar wrinkling as a presenting feature of cystic fibrosis gene dysfunction.

Clin Exp Dermatol. 2009 Jun 22. [Epub ahead of print]

Aquagenic palmar wrinkling as a presenting feature of cystic fibrosis gene dysfunction.

Stewart LC, Doe SJ, Bourke SJ, Leech S.

Department of Dermatology, Royal Victoria Infirmary, Newcastle upon Tyne, Newcastle, UK.

Aquagenic palmar wrinkling (APW) is characterized by the rapid and transient oedematous wrinkling of the palms after brief immersion in water. APW has been associated with cystic fibrosis (CF). Since the discovery of the CF gene, the clinical spectrum of CF has broadened from classic severe CF to include milder 'atypical CF' and 'CF-related disorders'. We report an unusual case in which APW occurred in a patient with no lung disease, and in whom investigations showed evidence of CF gene dysfunction. APW may be a presenting feature of a CF-related disorder and should prompt investigation of CF gene dysfunction.

PMID: 19548945 [PubMed - as supplied by publisher]