Aquagenic cutaneous disorders

Abstract

SummaryAquagenic cutaneous disorders, which include aquagenic urticaria, aquagenic pruritus, and aquagenic acrokeratoderma, are a group of rare diseases characterized by skin lesions or discomfort induced by brief contact with water. Aquagenic urticaria is characterized by pruritic wheals that occur at the sites of water contact, either immediately or within minutes, and disappear within 30-60 min after water removal. Aquagenic pruritus presents with severe pruritus or a stinging, tingling, or burning sensation without any visible skin changes. These symptoms occur immediately or within minutes after the skin is exposed to water, lasting for one hour or longer. Aquagenic acrokeratoderma usually presents with whitish papules and plaques, edema, and hyperwrinkling within 2-20 min after brief exposure to water; symptoms disappear within minutes to one hour after drying. The pathomechanisms of these diseases are not yet fully understood, and their diagnosis is primarily based on clinical features. Treatment remains a challenge. Herein, we review the literature regarding these unusual disorders.

Hereditary palmoplantar keratodermas. Part II: syndromic palmoplantar keratodermas – Diagnostic algorithm and principles of therapy

“…Sporadic AWP individuals, described as affected with non-classic CF or without CF, were shown to carry monoallelic or biallelic mutations in the CFTR. [114][115][116][117] Nevertheless, some non-CF AWP cases resulted negative for the most common CFTR mutations 118,119 or even did not go through CFTR genotyping. 107,109,120,121 The exact pathogenetic mechanism for AWP is still not understood.…”

Aquagener Pruritus als Warnzeichen eines inneren Tumorleidens – eine Fallvorstellung und Literaturübersicht

“…Zusätzlich beobachteten die Autoren einen Anstieg mononukleärer Zellen und in geringerem Ausmaß eosinophiler Granulozyten in der Haut Abb. 1 Übersicht über Formen des aquagenen Pruritus[53]. (MPN = myeloproliferative Neoplasien) MPN PMF PV ET andere CML BCR-ABL (-) BCR-ABL (+) ▶ Abb.…”

Atypical familial aquagenic syringeal acrokeratoderma

“…These lesions, however, are not typically confused with ASA due to their distinctly different presentations. 12 Several water-independent entities should also be considered when considering ASA. Symmetrical acral keratoderma, palmoplantar keratoderma, and acanthosis nigricans may be considered in the right clinical setting.…”