Painful Wrinkles in the Bathtub: Association with Hyperhidrosis and Cystic Fibrosis

Abstract

Transient aquagenic palmar hyperwrinkling (TAPH) is a rarely reported cutaneous condition clinically characterized by swelling and hyperwrinkling of the palms, and associated with burning sensations after briefly immersing the hands in water. Upon the withdrawal of water, the symptoms rapidly disappear. We report on a 10-year-old boy with type I diabetes, who developed the typical symptoms of TAPH concomitantly with the onset of palmar hyperhidrosis. Determination of the sodium chloride concentration of the sweat revealed elevated levels. Subsequent screening for the most common mutations of the gene responsible for cystic fibrosis (CF) was negative. Review of the literature shows that TAPH may develop in conditions of increased water absorption due to an increased sweat quantity, such as hyperhidrosis, or an increased sweat electrolyte concentration, such as CF. In the majority of reported cases TAPH has been associated with CF; therefore, it is a cutaneous sign, which should be recognized by dermatologists, and patients should be referred for evaluation of the sodium chloride concentration of the sweat.

Aquagenic Wrinkling of the Palms and Cystic Fibrosis: An Italian Study with Controls and Genotype-Phenotype Correlations

“…The association with hyperhidrosis [17] is also suggestive. Hyperhidrosis provokes an increase in the salt concentration of perspiration, because the primary sweat spends less time in the ducts, and less salt is reabsorbed.…”

“…The defective stratum corneum barrier function observed in atopy would lead to the occlusion of the eccrine duct ostia, and to the weakness of the eccrine duct wall [7,11]. Furthermore, the latter would produce the dilation of the sweat glands, leading to a mechanical wrinkling [17]. Recent histopathological findings may provide a support to this theory including the presence of hyperkeratosis with metachromasia of the stratum corneum, associated with dilated sweat duct ostia and dermal eccrine ducts, sometimes with accumulation of eosinophilic secretion [6].…”

“…Moreover, AWP has been reported even in otherwise healthy individuals as well as in patients with marasmus [10], atopic dermatitis, hyperhidrosis and in patients taking selective cyclooxygenase (COX-2) inhibitors, angiotensin-converting enzyme inhibitors, nonsteroidal anti-inflammatory drugs and antibiotics [12,17,18,19,20]. …”

Unilateral aquagenic keratoderma treated with botulinum toxin A

“…2 The "hand-in-the bucket" sign, when patients submerge their hands in water to demonstrate the lesions, constitutes a clue to diagnosis. 3 Patients may report tightness, pruritus, mild pain, 4 Bellotch et al (1 case) 13 Katz et al (2 cases) 14 Neri et al (1 case) 15 Suchak et al (1 case) 16 Kocatürk et al (1 case) 17 Seitz et al (1 case) 9 Katz et al (1 case) 14 Baldwin et al (1 case) 18 Flann et al (1 case) 19 Kabashina et al (1 case) 20 Diba et al (1 case) 11 Bagazgoita et al (1 case) 6 Our case The last 3 cases responded to botulinum toxin injections…”

“…A third theory, based on association of AK with cystic fibrosis 9 and intake of aspirin 5 or cyclo-oxygenase-2 inhibitors, 10 suggests that AK could be due to increased sweat salt concentration in the stratum corneum. 2,9 Spontaneous resolution has been reported in 4 cases and some cases clear after a few years. 8,11,12 Table 1 shows a list of all the cases of AK reported in the medical literature and their response to treatment.…”