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14-year-old girl presents with ‘raisin hands’
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A 14-year-old girl presented to the pediatric dermatology clinic with a complaint of “raisin hands.” For the previous month, she had noted that the skin on the fingers and palms of her hands would “wrinkle” and “hurt” following exposure to water for several minutes or longer. Her palms are occasionally pruritic, and the patient stated that her condition seemed to worsen with bathing and handwashing.
The patient had attempted to treat her palms with 20% aluminum chloride solution but experienced even worse pain and peeling of the skin. Her past medical history is significant for recently diagnosed mild asthma. The patient occasionally takes ibuprofen, but is not taking daily medications. She has not experienced any unintended weight loss and appears otherwise healthy.
On physical exam, the patient is noted to be athletic and without constitutional symptoms. She has no lymphadenopathy. Her hair, nails and skin – including her palms – all appear normal. After 45 seconds of contact with running water, however, direct observation reveals that she develops excessive wrinkling of the skin on her palms and volar fingers with mild superficial peeling and tenderness. She notes, “This is the prune skin I was talking about.”
Figure 1. Affected right hand after brief exposure to water vs. unaffected left hand
Figure 2. Up-close photo of affected right hand demonstrating wrinkling and edema after brief and directly observed exposure to water.
Source:Karkowski AC
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Case Discussion
Diagnosis: Cystic fibrosis (B)
This patient has a condition known as “aquagenic wrinkling of the palms.” This has also been termed “transient reactive papulotranslucent acrokeratoderma” and “aquagenic syringeal keratoderma.”
Emily Osier
Andrew C. Krakowski
Aquagenic wrinkling of the palms occurs after even mild exposure to water, as quickly as a few seconds but on average after 2 minutes of immersion in water —compared with the normal wrinkling response that occurs after, approximately, 11.5 minutes of water exposure in unaffected individuals. Affected patients usually experience excessive wrinkling of the palmar skin and, less frequently, plantar skin. Some patients have also reported experiencing the development of translucent papules, desquamation, or symptoms of pain, pruritus, tingling or burning.
Since 1974, aquagenic wrinkling has been linked to the diagnosis of cystic fibrosis or, at least, carrier status. Other reported related conditions include atopic dermatitis, hyperhidrosis, marasmus, and medication side effect (eg, angiotensin- converting enzyme inhibitors, selective cyclooxygenase inhibitors, antibiotics and NSAIDs). Aquagenic wrinkling has also been reported in healthy individuals.
An Italian study evaluated patients with a confirmed diagnosis of cystic fibrosis (CF) and compared their incidence of aquagenic wrinkling of the palms with CF carriers and healthy controls. They found that 53% of patients with CF had aquagenic wrinkling, compared with 9% of carriers and no patients in the control group. The CFTR protein that is affected in CF also regulates aquaporins; one hypothesis is that patients with CF have increased epithelial water uptake due to abnormal keratinocyte aquaporin expression. Another theory postulates that the higher sweat chloride content increases water retention of keratinocytes or that sympathetic stimulation of nerve fibers increases water retention; the latter may also help explain the development of paresthesias that affects many patients with aquagenic wrinkling.
A patient with suspected aquagenic wrinkling of the palms should have the diagnosis confirmed with a directly observed, in-office water submersion test of the hands and feet. Wrinkling that occurs in under 3 minutes is highly suspicious for CF and wrinkling at >7 minutes suggests CF carrier status. Workup of patients with aquagenic wrinkling of the palms should include evaluation for CF, as a mildly affected patient may not have been diagnosed as having CF and because “late presentations” have been reported. Although newborn screening programs currently test for CF, false-negatives do occur, and a history of a normal newborn screen should not preclude providers from performing diagnostic testing. As CF carrier status has obvious family planning implications, genetic counseling may also be warranted.
The patient’s family grew increasingly concerned about the potential diagnosis of CF after it was discovered that a first cousin had died from the condition. They have since established contact with a genetic counselor and have opted to pursue genetic testing of the 28 most common CF mutations with reflex testing for known rarer mutations.
- References:
- Chinazzo C. et al. Dermatology. 2014; doi: 10.1159/000355183.
- Weibel L., et al. N Engl J Med. 2012; doi: 10.1056/NEJM1CM1103833.
- Park L. et al. Pediatr Dermatol. 2012; doi: 10.111/j.1525-1470, 2011, 01609.x.
- For more information:
- Emily Osier, MD, is a clinical research fellow in pediatric dermatology at Rady Children’s Hospital, San Diego. She can be reached at 8010 Frost St., Suite 602, San Diego, CA 92123; email: ejosier@gmail.com.
- Andrew C. Krakowski, MD, is an attending physician at Rady Children’s Hospital, San Diego.
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