Frequency of aquagenic palmoplantar keratoderma in cystic fibrosis: a new sign of cystic fibrosis?

Garçon-Michel, N.; Roguedas-Contios, A.-M.; Rault, G.; Bihan, Jean Le; Ramel, Stig; Revert, K.; Dirou, Armel; Miséry, Laurent

doi:10.1111/j.1365-2133.2010.09764.x

Cited by 55 publications

(59 citation statements)

References 39 publications

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“…We found a 41% incidence in our adult CF population. Thus, our data is in accordance with the recent publications by Berk et al [6] and Garcon-Michel et al [7] and suggests that the incidence of AWP is likely to be much higher than that previously reported. It has been stated that patients with CF, or their parents, do not think it important to report the symptoms of AWP as, at clinic visits, assessment and treatment of the chest and gastrointestinal disease are the most pressing factors [13].…”

Section: Discussionsupporting

confidence: 77%

“…There have been only 2 reported population-based studies in the literature regarding AWP and CF [6,7]. Prior to this, AWP had been described to be a rare phenomenon with only 37 reported cases, 19 of these occurring in CF patients.…”

Section: Discussionmentioning

confidence: 99%

“…Until recently there had been no population-based studies regarding AWP and CF. Two recent reports have found a high prevalence of AWP in an American CF population [6] and a French CF population [7]. AWP has been described under numerous synonyms including ‘transient reactive papulotranslucent keratoderma’ [8,9,10], ‘aquagenic palmoplantar keratoderma’ [1], ‘aquagenic keratoderma’ [11], ‘aquagenic syringeal acrokeratoderma’ [12], ‘transient palmar hyperwrinkling’ [4] and ‘wrinkling of the skin’ [2,13].…”

Section: Introductionmentioning

confidence: 99%

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Aquagenic Wrinkling of the Palms in an Adult Cystic Fibrosis Population

Tolland

Boyle²,

Hall³

et al. 2010

Dermatology

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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.

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Section: Discussionsupporting

confidence: 77%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Aquagenic Wrinkling of the Palms in an Adult Cystic Fibrosis Population

Tolland

Boyle²,

Hall³

et al. 2010

Dermatology

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“…The causative immersion may last from a few seconds to 10 min, but most often the lesions become manifest after 2-3 min [7,8]. Warm water is more rapidly effective than cold water [9].…”

Section: Introductionmentioning

confidence: 99%

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

Chinazzo

Alessandri²,

Menoni

et al. 2014

Dermatology

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Aims: The primary aim was to determine the prevalence of aquagenic wrinkling of the palms (AWP) in patients with cystic fibrosis (CF) compared to controls, and secondarily to evaluate genotype-phenotype correlations among CF subjects found to have AWP. Methods: Fifty-eight patients with CF underwent a hand immersion test in tap water. Twenty-three of their CF carrier relatives and 7 subjects with a negative genetic test for CF were recruited as controls. Secondary analyses explored associations with genotype, pulmonary function, and sweat electrolyte levels in all subjects with and without AWP. Additional information about atopic diathesis, hyperhidrosis of the palms and drug intake were also collected. Results: Thirty-one of the patients with CF (53.4%) exhibited AWP, in contrast to only 2 carriers (8.7%) and none in the control group. No correlation was found between CF genotype and AWP score severity. Twenty-three (39.7%) CF patients reported a history of hyperhidrosis, and in 17 of them (74%) AWP had been provoked. No correlation with history of atopy and lung function was noted. The difference between CF patients with hyperhidrosis and those without was highly significant (p = 0.016). Salt concentrations were significantly higher in patients with AWP. Conclusions: AWP is linked to CF and its diagnosis should lead the patients to a genetic or sweat test for CF. We found a significant association with hyperhidrosis and sweat electrolytes which supports the ‘hyperconcentrated sweat' pathogenetic theory of AWP.

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“…Aquagenic wrinkling of the palms is considered a clinical sign of cystic fibrosis and is seen in as many as 80% of people with the disease. 2,3 It has also been reported in people who are cystic fibrosis carriers, although the sign takes longer to manifest following water immersion in this population. 4 Genetic testing should be performed in patients who present with aquagenic wrinkling of the palms.…”

mentioning

confidence: 96%