Aquagenic Wrinkling of the Palms in an Adult Cystic Fibrosis Population

Abstract

Background: Aquagenic wrinkling of the palms (AWP) is a rare condition characterised by the development of oedema and excessive wrinkling of the palms following exposure to water. It has frequently been associated with cystic fibrosis (CF). Early reports of AWP have only been case reports or small case series; there has only been one reported prevalence study of AWP in a CF population. Objective: To determine the incidence and characteristics of AWP in the adult CF population in Northern Ireland. Methods: 105 CF patients were interviewed. The patients were asked whether they noticed excess wrinkling of the hands when exposed to water. If they answered ‘yes’, further questions were asked regarding clinical characteristics. The atopic status, CF genotype and drug history were recorded for each patient. Formal testing of 7 patients was carried out. Results: Out of the 105 patients who were interviewed, 43 (41%) described AWP. Of the 43 patients with AWP, 20 were male and 23 were female. There was no association of AWP with genotype, atopy or concomitant drug intake. Conclusion: AWP appears to have an equal sex incidence, and the high number of cases in the population studied would suggest that this condition is underreported.

Aquagenic Wrinkling of the Palms: Morphological Changes in Reflectance Confocal Microscopy and High-Definition Optical Coherence Tomography

“…It has been proposed that in patients with CF and hyperhidrosis, the elevated epidermal sodium content might increase the absorption of water above levels of healthy persons [9,10]. In general, it is assumed that in AWP patients structural changes in the stratum corneum result in a malfunction following water contact, and an aberrant expression of AQP5, which belongs to the aquaporin water channel family, was shown in the sweat glands of AWP patients.…”

Aquagenic Wrinkling of the Palms and Cystic Fibrosis: An Italian Study with Controls and Genotype-Phenotype Correlations

“…Berk et al [14] demonstrated that CF patients who were ΔF508 homozygous had severer wrinkling than those with other CF genotypes. In addition, a survey-based study showed that 41% of CF patients admitted to having AWP [15]. Finally, Arkin et al [16] found that 84% of CF patients demonstrated AWP, in contrast to none of the controls.…”

“…The prevalence of AWP in CF is however unclear (it varies from 41 to 84%) as the methods of evaluation are neither homogeneous nor validated in large, stringent case-control studies [15,16]. Moreover, AWP has been reported even in otherwise healthy individuals as well as in patients with marasmus [10], atopic dermatitis, hyperhidrosis and in patients taking selective cyclooxygenase (COX-2) inhibitors, angiotensin-converting enzyme inhibitors, nonsteroidal anti-inflammatory drugs and antibiotics [12,17,18,19,20].…”

Dermatoscopy as an Adjunctive Diagnostic Tool in Aquagenic Syringeal Acrokeratoderma

“…A recent study highlighted a 41% incidence of aquagenic wrinkling of the palms in 105 patients with CF. The authors suggested that homozygosity for the F508del CFTR (cystic fibrosis transmembrane conductance regulator) mutation may predispose to aquagenic wrinkling of the palms [8]. A clinical trial showing the decreased time interval for aquagenic wrinkling using the water immersion test in patients with CF (2 min) compared with a heterozygous mutation (carrier) group (7 min) and control group (14 min) also strengthens the relationship between ASA and CF [9].…”