Author Affiliations: Department of Dermatology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
A 25-year-old atopic Turkish woman presented with a 6-month history of widespread, slightly elevated, pruritic red plaques over her face, trunk, and extremities occurring each time after prolonged contact with water of any temperature. Skin biopsy findings were consistent with urticaria. Bathing exceeding 20 to 30 minutes always resulted in syncope followed by spontaneous recovery in 30 minutes. Angioedema or respiratory symptoms did not occur. While sweating, producing tears, drinking water, or washing hands for a short duration did not provoke lesions, washing dishes by hand induced localized lesions. No spontaneous attacks of syncope were reported. None of the family members were similarly affected.
Focal and systemic infections and autoimmune and other systemic diseases were ruled out. The human immunodeficiency virus antibody test result was negative. Neurologic and cardiovascular investigations revealed no other causes of syncope. Challenge tests with hot, ice-cold, and room-temperature water led to urticarial plaques within 20 minutes (Figure 1A). Exposure to heated air from an electric hair dryer at 40 °C for 2 minutes resulted in localized urticarial plaque within 10 minutes. Cold urticaria (tested with an ice cube inside a plastic bag for 10 minutes), cholinergic urticaria, and dermatographism were absent. Combined treatments with antihistamines (ie, oral hydroxyzine, desloratadine, rupatadine, or parenteral pheniramine at high doses), narrowband UV-B, shortening the bathing time to less than 5 minutes, and alkalization of bath water (pH 8-9) by adding sodium bicarbonate (500 g/50 L) were partially effective (Figure 1B and Figure 2).
Figure 1. Clinical images of the forearm. A, Positive challenge tests with hot (40 °C), ice-cold, and room-temperature water on the forearm within 20 minutes. B, Bicarbonated water (500 g/50 L) immersion test led to pruritic and burning edematous plaque within 2 hours.
Figure 2. Delayed onset of pruritic erythema on the trunk (A), upper extremities (B), and lower extremities (C). Reactions occurred 2 hours after a 5-minute bath with bicarbonated water during combined therapy with oral antihistamines and narrowband UV-B. Attacks of syncope could be prevented by combined intervention.
Thyroid function test results and autoimmune markers were normal. Thyroid ultrasonography revealed a 12-mm isoechoic, solid nodule in the left lobe of the thyroid gland. Fine-needle aspiration biopsy findings were consistent with papillary carcinoma of the thyroid. The patient underwent total thyroidectomy. Only mild erythema occurred following the first postoperative bathing. Treatment with all medications was stopped, and the patient was free of lesions at 6-month follow-up.
Large urticarial plaques, involvement of the entire body, and accompanying heat-induced urticaria as in this case are extremely rare in aquagenic urticaria. Aquagenic urticaria leading to syncope is a very unusual finding reported in infants and children.1 - 2 Bronchospasm might occur.3 Hematologic, lymphoproliferative, infectious, and neoplastic diseases have been reported mainly in conjunction with aquagenic pruritus,4 a condition in the same spectrum as aquagenic urticaria. However, the etiologic role of the underlying disease often remains obscure.
Recently, 4 adult cases of chronic urticaria with occult papillary carcinoma of the thyroid have been reported.5 Similar to our case, prompt resolution of the urticarial lesions was achieved following total thyroidectomy. To our knowledge, this is the first report of an adult case of aquagenic urticaria and syncope associated with occult papillary thyroid carcinoma showing dramatic response to total thyroidectomy. Despite the lack of any clinical or laboratory hints for performing a thyroid ultrasonography, its role in unraveling this challenging case was of inestimable worth.
Correspondence: Dr Özkaya, Department of Dermatology, Istanbul University, 34093 Istanbul, Turkey (profeo@istanbul.edu.tr).
Financial Disclosure: None reported.
Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature
Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal
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