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Aquagenic Wrinkling of Palms

Question
My daughter (5 y.o.; F508del/CFTR dele2,3) seems to have Aquagenic wrinkling of Palms. What does this condition mean? IS it somehow dangerous? Should it be treated?
Answer
Dear questioner,
in order to answer your question, I collected information from a former question on this topic asked on the French ECORN-CF site, answered by Dr. Gilles Rault in May, 2011. I hope to have helped you with this,

Best regards,
Dr. Daniela d'Alquen (coordiantor of the central English Archive)

"Hello,
The mentioned “Aquagenic wrinkling of Palms” have been reported in a few publications under various names: Aquagenic palmoplantar keratoderma (APPK), also called Aquagenic wrinkling of the palms, Transient reactive papulotranslucent acrokeratoderma, Aquagenic syringeal keratoderma or Transient aquagenic hyperwrinkling ... Diagnosis can easily be confirmed by the immersion of hands in a bucket of fresh water (cold or warm) for 3 minutes. This test aims to highlight what is called simply the "Sign of the bucket”. This dermatosis is characterised by oedema, whitish papules, hyperwrinkling and sometimes desquamation of palms and/or soles . It appears after contact with water for a few seconds to ten minutes; however, symptoms most often present after two to three minutes of water immersion. It can also trigger pruritus, tingling, burning or even pain.
These symptoms have been observed for the first time in a patient with cystic fibrosis in 1970. Fifty identical cases have been reported so far. This symptomatology is not related to the severity of the disease since it can be found in healthy carriers of a single mutation of the CFTR gene or in individuals with no CFTR mutation. The sign of the bucket was positive in 11 of 27 patients with cystic fibrosis (41%), in a recent study carried out in our center (Roscoff Center, France) and published in 2010 in the British Journal of Dermatology. It is therefore a usual sign of cystic fibrosis whose frequency is very underassessed because the attention of teams and patients is primarily focused towards more severe symptoms. This symptom is the manifestation of an increase of salt (sodium and chloride) concentration in the different layers of the skin resulting in water hyperabsorption. This pathophysiological mechanism explains that the symptoms can be observed also in other medical conditions than cystic fibrosis or as a side effect of some drugs that inhibit cyclo-oxygenase 2 (rofecoxib, Relecoxib and Aspirin). The frequency of this syndrome in the general population is not known, it can also be "idiopathic" i.e. isolated and unrelated to a particular disease.
Usually light, this condition may sometimes be perceived as unpleasant or painful, then the symptoms are treated. They can be improved by avoiding predisposing conditions (occlusion by hands gloves, inhibitors of cyclo-oxygenase 2), and with application of moisturizers.
With my cordial greetings,

Dr. Gilles Rault

Reference quoted : Frequency of aquagenic Palmoplantaris keratoderma in cystic fibrosis: a new sign of cystic fibrosis? Br J Dermatol, 2010 Jul; 163 (1) :162-6. Male N-Michel et al."

10.03.2014