This condition has been described at different times and by various authors under different names, such as transient reactive papulotranslucent acrokeratoderma, acquired aquagenic papulotranslucent acrokeratoderma, aquagenic wrinkling of the palms, aquagenic syringeal keratoderma, and transient aquagenic palmar hyperwrinkling. However, most researchers now agree that they all represent the same disorder.
The condition occurs between the ages of 6 and 45 years, with the average age being 20-30 years, and it predominantly affects women. The pathogenesis is unclear, and the majority of cases are sporadic.
Aquagenic keratoderma occurs in 44-80% of patients with cystic fibrosis (mucoviscidosis) who have been found to have homozygous or heterozygous δF508 mutation in the CFTR gene, which is responsible for regulating electrolyte transport. The mutation leads to reduced chloride and sodium reabsorption across epithelial membranes, resulting in increased sodium levels in sweat and enhanced water exchange capacity of the stratum corneum due to high salt content. This likely leads to visible changes on the skin upon contact with water or during excessive sweating.
Aquagenic keratoderma can also occur with the use of certain medications such as aspirin, celecoxib, rofecoxib, and tobramycin, which inhibit the enzyme cyclooxygenase-2, leading to electrolyte concentration in sweat.
Some cases of the condition have been associated with focal hyperhidrosis and Raynaud's phenomenon, suggesting a link to the autonomic nervous system and vasoconstriction.The condition is characterized by the development of edema, wrinkling of the skin, semi-translucent gray-white papules 1-2 mm in diameter, sometimes accompanied by scaling, on the palms and less frequently on the soles, occurring within minutes of contact with water or excessive sweating.
Duration of water exposure and water temperature influence the speed and intensity of lesion development. The papules coalesce into plaques with a "cobblestone" surface appearance, with dilated orifices of eccrine sweat glands visible as small dots in the center. Erythema is absent.
Several cases have been reported with unusual localization of the eruption, such as on the heel, dorsum of the fingers, and wrists. The eruptions are always accompanied by itching, burning, or even pain and resolve spontaneously within 2-30 minutes, leaving inconspicuous small areas of hyperkeratosis in the central part of the palms.The diagnosis is based on the characteristic clinical presentation after a water test.
Dermoscopy shows dilated openings of eccrine sweat glands. Examination with a Wood's lamp may reveal inconspicuous areas of hyperkeratosis outside of episodes following water exposure. In patients with newly diagnosed disease, it is necessary to exclude cystic fibrosis or carrier status of the heterozygous defective CFTR gene.- Trench foot (immersion foot syndrome)
- Hereditary palmoplantar keratoderma
- Irritant contact dermatitis of the palms and soles
- Diffuse palmoplantar keratoderma, Bothnian type
- Aquagenic urticaria
- Aquagenic pruritus
- Application of a 15%-20% solution of aluminum chloride hexahydrate.
- Keratolytic agents such as creams or ointments containing urea and salicylic acid.
- Oxybutynin chloride 2.5mg twice daily orally
- Subcutaneous injections of botulinum toxin.