Background:

Hypersensitivity to specific stimulus presenting as pruritic wheals is pathognomonic of urticaria, a common malady worldwide, but such vulnerability to water is a rare and a distressing phenomenon requiring considerable lifestyle modifications. Aquagenic urticaria, a rare subtype of urticaria is most probably an allergic response to water. Though histamine is the most potent mediator of the phenomenon, a few reports implied that acetylcholine and genetic predisposition also plays a crucial role in the pathogenesis. Till date only a limited number of case reports are available worldwide with indefinite etio-pathogenesis and treatment guidelines. Therefore it is of utmost importance to summarize the available theranostics to provide guidance for the management of the condition and explore the future possibilities in light of recent advancements in understanding the pathophysiology of the disease. We attempt to describe the pathogenesis, case reports to the best of our knowledge and available treatment options from the literature.

Keywords: Aquagenic urticaria, water allergy, urticaria, contact dermatitis