Crumpled hands after water contact

Question

Dear expert team,
it seems to be that in CF-patients, hands get much faster crumpled in case of water contact then in healthy people (11 min).
Keyword: Aquagenic Wrinkling of the Palms.
Many children with CF are diagnosed troublesomely after many pneumonias, because a simple, for everybody recognizable sign of the illness is lacking, which is obviously present here.
Would it not be desireable that this is gonig to be more public?
Should this be passed as an "advice under parents"?
Would parents be taken seriously who go to the pediatrician because their thin child, which is often ill, shows crumpled hands in water after 2 minutes?
Many thanks in advance,

Answer

Dear questioner,
the symptom of the crumpled hands is indeed a hint for the diagnose of CF, like also very recent publications confirm (see below). The phenomenon, that the palms get crumpled in case of water contact can be found more frequently in CF patients as well as in carriers of CF-mutations, however much stronger in the first ones. The changes appear to be stronger in patients, who carry a CF-mutation (DF508) on both chromosomes.
In my opinion, the phenomenon of the fast crumpling hands can be consulted as an additional symptom in order to confim the suspected diagnose. In case of further hints for CF, an immediate clarification should be initiated, and if necessary, the finding of the diagnose with the performance of a sweat test according to the classic diagnostic guidelines as well as a molecular genetic investigation. In case this phenomenon is the only symptom, I would rather be restrictive with an extended investigation.
It would be desirable and important, that the diganose of CF would be made fast, this especially as long as there is no newborn-screening available. Therefore, parents should mention symptoms of suspicion readily, even with the danger of not being taken for serious. Doctors should look for other symptoms and in case of clinical evidence, initiate a quick clarification.
Yours sincerely,
Dr. H. Ellemunter

Gild R, Clay C, Morey S. Aquagenic Wrinkling Of The Palms in Cystic Fibrosis And The Cystic Fibrosis Carrier State - A Case Control Study. Br J Dermatol. 2010 Jun 18. [Epub ahead of print]

Berk DR, Ciliberto HM, Sweet SC, Ferkol TW, Bayliss SJ. Aquagenic wrinkling of the palms in cystic fibrosis: comparison with controls and genotype-phenotype correlations. Arch Dermatol. 2009 Nov;145(11):1296-9.

Stewart LC, Doe SJ, Bourke SJ, Leech S. Aquagenic palmar wrinkling as a presenting feature of cystic fibrosis gene dysfunction. Clin Exp Dermatol. 2009 Dec;34(8):e647-9.

Gild R, Clay CD. Aquagenic wrinkling of the palms in a cystic fibrosis carrier. Australas J Dermatol. 2008 Feb;49(1):19-20.

21.09.2010