We present a 17-year-old male with an eighteen-month history of severe
palm and finger wrinkling following brief (less than 5 minutes)
immersion in water. The hands assume normal appearance within 60
minutes. Narrative is provided primarily by his mother as the patient
has high-functioning autism. He denies associated burning or itching and
is on no oral medications. Personal and family history are negative for
atopic dermatitis, Raynaud’s disease, and cystic fibrosis. At
consultation his hands are normal (Figure 1). A photograph shown during
the visit (Figure 2) revealed markedly shriveled fingers and palms and a
multitude of 1mm whitish papules.
Rare cases have been reported since the first description of aquagenic
palmar wrinkling (APW) by English and McCullough in
1966.1 Subsequently, various reports have described
the condition as transient aquagenic hyperwrinkling, aquagenic
palmoplantar keratoderma, and transient reactive papulotranslucent
acrokeratoderma.2,3 As APW often resolves within a
short period of time it is not believed to be a true
keratoderma.4
APW has a predilection for affecting young adult
women.5,6 It is diagnosed clinically based on
characteristic findings of the disease. These include the transient
rapid development of yellow, white edematous papules and plaques, along
with excessive wrinkling and even desquamation after brief contact with
water.7 The findings are pathognomonic and referred to
as the “hand-in-the-bucket” sign.8 Additional
symptoms of pain, pruritis, and burning sensations localized to the
palms are also frequently associated with the condition.
In APW, wrinkling occurs within 3 minutes of water immersion, in
comparison to the normal physiologic wrinkling occurring roughly 11
minutes after water immersion.9 It is commonly
bilateral affecting the palmar surfaces with plantar involvement
infrequently observed.10 However, rare cases of
unilateral involvement and varying aspects of the hands excluding the
palms have been reported.11,12 Resolution of symptoms
occurs within 10-60 minutes of skin drying.13 While
the diagnosis is clinical, histopathological examination will reveal
compact orthokeratosis, dilatation of eccrine sweat glands, and
spongiosis.14
The exact etiology and pathogenesis remain unknown, although an
autosomal recessive mode of inheritance has been
suggested.1 There are several conditions associated
with APW including hyperhidrosis, atopic dermatitis, and Raynaud’s
phenomenon. The most described association of disease is cystic fibrosis
(CF).2 APW is considered a highly favorable screening
tool in CF patients, with reported occurrence in 40-80% of
patients.15
CF is an autosomal recessive condition caused by mutations in the gene
involved in trans-epithelial ion transport leading to high sweat sodium
concentrations. One proposed mechanism behind the prevalence in CF
patients is an increased salt content in sweat leading to enhanced
keratin water binding capacity of the epidermal
cells.16 The water also alters membrane stability
causing vasoconstriction that contributes to skin
wrinkling.17
APW is also associated with medications including cyclooxygenase 2
inhibitors, non-steroidal anti-inflammatory drugs, gabapentin,
spironolactone, and aminoglycoside antibiotics.18,19These drugs may alter cell-membrane water channels, known as aquaporins,
within the epidermis.17 Additionally, the association
with atopic disease supports the hypothesis of decreased barrier
function. Similar to atopy, a dysfunction in stratum corneum leads to an
increased ability of the skin to absorb water.5,16
Several treatments for APW have been identified. Topical therapies
including aluminum chloride, salicylic-acid based products, and
urea-containing creams targeted at reducing the hyperkeratosis and
hyperhidrosis have proven of benefit.6,20 Other
therapeutic options include oral antihistamines and botulinum toxin
injections. One case improved following topical treatment with the
calcineurin inhibitor, tacrolimus.13 Many cases
resolve spontaneously.21
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