Aquagenic Wrinkling: It burns…

A 16 year-old female presented with a two month history of painful, burning wrinkling of her palms after taking a pottery class.  Within 5 minutes of water exposure she noted the onset of wrinkling with sharp pains like paper cuts.  The pain would resolve within 5-10 minutes after drying her hands.  She denied taking any medications except for zyrtec on occasion for allergies.  She denied any family history of cystic fibrosis.

On clinical examination after warm water exposure her palmar skin became pebbly, slightly edematous with characteristic whitish edema around palmar pores, as shown on the images above.  Wrinkling was not prominent with this short exposure.  The patient noted the pain symptoms as described.

The patient was treated with drysol and instructed to follow up in a few weeks.  Of interest is that she recently had her tonsils and adenoids removed and since that time her palmar symptoms have improved if not resolved.

Aquagenic wrinkling (AW) of the palms is a rare disorder affecting females more often than males.  It was first described by Elliot in patients with cystic fibrosis, which has been described as being distinct from normal aquagenic water associated wrinkling.  Cystic fibrosis patients develop a form of chronic aquagenic wrinkling of the palms (AWP).  AW may be idiopathic and has been reported to be associated with marasmus, atopic dermatitis or hyperhidrosis.  In addition, several drugs have been reported to induce AW, such as nonsteroidal anti-inflammatory agents, angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers.

ETIOLOGY:  The pathophysiology underlying AW is unknown.  A defect in the stratum corneum altering water retention has been proposed, but not proven.  Some studies on the F508 mutation in the cystic fibrosis transmembrane receptor (CFTR) gene in a homozygous state correlate with the degree of transepidermal water loss.  Sweat chloride levels appear unrelated.  Aberrant expression of aquaporin-5 may also contribute to AWP…suggesting a multifactorial pathogenesis.

TREATMENT:  A number of treatments have been attempted, directed at creating a barrier against water influx into the stratum corneum.  Aluminum chloride, salicylic acid 20% in petrolatum, botulinum toxin injections, tacrolimus, antihistamines, and inotophoresis have been utilized with variable results.  It is recommended that patients be screened for the cystic fibrosis gene.  To read more about AW click HERE, HERE, and HERE.