Indian Journal of Dermatology
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Year : 2014  |  Volume : 59  |  Issue : 6  |  Page : 632
Aquagenic syringeal acrokeratoderma

Department of Dermatology, Sifa University, Basmane, Izmir, Turkey

Date of Web Publication30-Oct-2014

Correspondence Address:
Belkiz Uyar
35240 172/2 Fevzipaşa Bulvar? Basmane/Izmir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.143578

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Aquagenic syringeal acrokeratoderma is a rare, transient, and usually bilaterally symmetric, palmoplantar keratoderma. Patients complain of tingling and pain in the hands starting a few minutes after exposure to water and lasting for 20-30 minutes after removal. Clinically, there is marked wrinkling with edematous white papules on the palms or, less often, the soles. We present the case of a 21-year-old woman who used spironolactone for polycystic ovary syndrome and had similar clinical features 2 weeks later, after withdrawing the drug.

Keywords: Aquagenic syringeal acrokeratoderma, aquagenic wrinkling, spironolactone

How to cite this article:
Uyar B. Aquagenic syringeal acrokeratoderma. Indian J Dermatol 2014;59:632

How to cite this URL:
Uyar B. Aquagenic syringeal acrokeratoderma. Indian J Dermatol [serial online] 2014 [cited 2023 May 28];59:632. Available from:

What was known?
Aquagenic syringeal acrokeratoderma (ASA) is an acquired or hereditary keratoderma of unknown etiology. Several pathogenic mechanisms have been proposed. Drug-induced cases of ASA of the palms have been reported, associated with rofecoxib, celecoxib, aspirin, and in one case, tobramycin used for cystic fi brosis.

   Introduction Top

Aquagenic syringeal acrokeratoderma (ASA) is a rare skin disorder of unknown etiology. Several pathogenic mechanisms have been proposed, including structural or functional defects of the horny layer during adolescence, primary disease of the sweat ducts, increased sodium concentration in the skin, thereby increasing the water-retention capacity of the horny layer or a reaction to drugs.

We present a case of a woman who used spironolactone for polycystic ovary syndrome (PCOS) and developed ASA 2 weeks later, after withdrawing the drug.

   Case Report Top

A 21-year-old woman presented to our outpatient clinic with complaints of palmar eruption. She noted wrinkling and edema of the skin on the palms after soaking in water for 5-10 min, 3 weeks earlier. The wrinkling and edema were accompanied by sensations of tightness, tingling, and pruritus. The condition became better 10-15 minutes after exposure to water had ceased, but did not completely resolve. These findings regressed gradually after drying the hands, but were not completely resolved.

On dermatological examination, whitish, keratodermic, macerated plaques, and dilated punctae were observed over the volar surface of the hands [Figure 1]. After soaking the hands in water for 5 min, the lesions became more translucent, noticeable, and symptomatic [Figure 2].
Figure 1: Before soaking the hands in water, whitish keratodermic macerated plaques and dilated punctae were observed

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Figure 2: After soaking the hands in water for 5 min, the lesions became more translucent, noticeable, and symptomatic

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From her medical history, we learned that she used spironolactone for PCOS up to 2 months earlier. Her lesions occurred 2 weeks later, after withdrawal of the spironolactone. She denied any concomitant hyperhidrosis, a personal or family history of cystic fibrosis, abnormal scalp hair, or atopic diathesis. Her cousin had similar lesions years ago, but her symptoms were completely resolved with treatment. The disease had been treated at another center with 19% aluminum hydrochloride cream with no improvement.

The laboratory findings, including sweat chloride concentrations were within normal limits, except for very small elevation of prolactin levels. A pelvic ultrasonographic examination revealed PCOS. We wanted to perform genetic testing to define cystic fibrosis transmembrane conductance regulator (CFTR) mutations, but she would not accept any further tests. We have not performed a biopsy because of typical clinical image.

The patient was treated with topical pomade containing 10% urea and salicylic acid twice a day and topical 10% urea lotion (Excipial Lipo ® ; Orva). The lesions notably improved after 2 weeks, but did not completely resolve.

   Discussion Top

ASA has been described in the literature under various names for similar conditions, patients with flat-topped, pitted, or translucent papules, with pebbly or white, prominent eccrine pores that are macerated in appearance and that appear on the hands and feet, exacerbated by water immersion.

The disease was first reported as a "hereditary papulotranslucent acrokeratoderma" in 1973 by Onwukwe et al. They reported a condition that appeared soon after puberty, demonstrated an autosomal dominant mode of inheritance and associated with fine-textured scalp hair and an atopic diathesis. [1]

Afterwards, in 1974, "aquagenic wrinkling" was reported by Elliot in a Letter to the Editor, describing an anecdotal observation in children with cystic fibrosis. [2] Subsequently, several reports presented by several names, including transient reactive papulotranslucent acrokeratoderma, ASA, [3] aquagenic keratoderma, transient aquagenic palmar hyperwrinkling, and early aquagenic wrinkling. Young women are most often affected.

Histopathological changes include orthohyperkeratosis with increased thickness and abnormal staining of the stratum corneum, dilated acrosyringia, and dermal eccrine ducts with hyperplasia of eccrine glands, clear cell changes and vacuolations, and increased capillaries around and adjacent to the eccrine glands. [4]

Although ASA is often related to cystic fibrosis, researchers have reported that it is not only specific for cystic fibrosis; it is also seen in different conditions, such as marasmus and nephrotic syndrome.

Drug-induced cases of ASA of the palms have been reported in single case reports, associated with rofecoxib, [5] celecoxib, [6] aspirin, [7] and, in one patient, with tobramycin [8] used for cystic fibrosis. The proposed mechanisms for the cases using rofecoxib, celecoxib, and aspirin involve an increase in the sodium retention of epidermal cells. In these cases, the proposed mechanism is cyclooxygenase-2 (COX-2) inhibition in epidermal cells, which may cause increased sodium reabsorption in a mechanism similar to the effect of COX-2 inhibitors on kidney cells. [5],[6],[7]

Spironolactone is an aldosterone antagonist and potassium-sparing diuretic. Adrenal steroids have recently been shown to play an important role in the regulation of renal COX-2 expression. According to Zhang et al., blockade of mineralocorticoid receptors with spironolactone leads to upregulation of renal cortical COX-2 expression. [9] In our patient, who was using spironolactone for PCOS for approximately 2 months, it may have led to upregulation of COX-2 expression. Withdrawing the drug may have caused increased sodium retention of epidermal cells.

During treatment, 20% aluminum chloride solution, botulinum toxin injections, antihistamines, pomade containing 5% salicylic acid, a mixture of mometasone furoate and petroleum jelly, and a cream containing 20% urea were used for treatment. Our patient had not improved with 19% aluminum hydrochloride cream, perhaps because she had not suffered from palmar hyperhidrosis.

   References Top

1.Onwukwe MF, Mihm MC Jr, Toda K. Hereditary papulotranslucent acrokeratoderma: A new variant of familial punctate keratoderma. Arch Dermatol 1973;108:108-10.  Back to cited text no. 1
2.Elliott RB. Letter: Wrinkling of skin in cystic fibrosis. Lancet 1974;2:108.  Back to cited text no. 2
3.MacCormak MA, Wiss K, Malhotra R. Aquagenic syringeal acrokeratoderma: Report of two teenage cases. J Am Acad Dermatol 2001;45:125-6.  Back to cited text no. 3
4.Rongioletti F, Tomasini C, Crovato F, Marchesi L. Aquagenic (pseudo) keratoderma: A clinical series with new pathological insights. Br J Dermatol 2012;167:575-82.  Back to cited text no. 4
5.Carder KR, Weston WL. Rofecoxib-induced instant aquagenic wrinkling of the palms. Pediatr Dermatol 2002;19:353-5.  Back to cited text no. 5
6.Vildosola S, Ugalde A. Celecoxib-induced aquagenic keratoderma. Actas Dermosifiliogr 2005;96:537-9.  Back to cited text no. 6
7.Khuu PT, Duncan KO, Kwan A, Hoyme HE, Bruckner AL. Unilateral aquagenic wrinkling of the palms associated with aspirin intake. Arch Dermatol 2006;142:1661-2.  Back to cited text no. 7
8.Ludgate MW, Patel D, Lamb S. Tobramycin-induced aquagenic wrinkling of the palms in a patient with cystic fibrosis. Clin Exp Dermatol 2009;34:e75-7.  Back to cited text no. 8
9.Zhang MZ, Harris RC, McKanna JA. Regulation of cyclooxygenase-2 (COX-2) in rat renal cortex by adrenal glucocorticoids and mineralocorticoids. Proc Natl Acad Sci USA 1999;96:15280-5.  Back to cited text no. 9

What is new?
Withdrawing spironolactone may have caused increased sodium retention of epidermal cells, thereby inducing ASA.


  [Figure 1], [Figure 2]

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