Aquagenic urticaria associated with anaphylaxis, urticarial vasculitis, skin ulcerations, and systemic lupus erythematosus: A rare case study

Aquagenic urticaria is a very rare form of physical urticaria with less than 50 cases reported in the medical literature. The condition causes itchy and potentially painful hives to break out whenever the sufferer comes into contact with water, sweat, or tears. A relationship between chronic urticaria and systemic lupus erythematosus (SLE) has been suggested in the literature. However, aquagenic urticaria has rarely been reported in association with other systemic conditions and is not typically a life-threatening condition. There are no documented cases of aquagenic urticaria occurring together with SLE or small vessel vasculitis, or presenting with cutaneous lesions more serious than urticaric wheals. This report presents a rare case of a 43-year-old African-American woman with aquagenic urticaria, who developed unusually painful skin ulcerations and life-threatening wheezing after contact with water, remained misdiagnosed for over five years, and, subsequently developed several serious comorbidities, including SLE. The case seems to suggest that aquagenic urticaria, urticarial vasculitis, and SLE may be related disorders that potentially developed along one spectrum of disease. As diagnosis of the condition is challenging and the use of water intraorally is essential to performing most dental procedures, the article aims to raise awareness of the condition among dentists and physicians and provides tips and suggestions to help manage patients with aquagenic urticaria more effectively.