Muckle-Wells syndrome (MWS) is a rare autosomal dominant hereditary disorder characterized by chronic recurrent urticaria, arthralgia, sensorineural deafness, and in some cases nephropathy due to amyloidosis (AA type). We report a 21-year-old woman and her father, both suffering from this syndrome, in whom elevated serum levels of IL-6 could be documented during the flares of urticaria, and discuss the relevance of this finding for MWS.
Background: The etiology of Wells’ syndrome or eosinophilic cellulitis is unknown. Various triggering factors, such as myeloproliferative disorders, lymphoma, infections/infestations, insect bites and drugs have been reported. In 1979, Wells was the first who pointed out some common features of eosinophilic cellulitis and skin lesions in toxocariasis. Objective: We report 2 patients who exhibited the characteristic clinical and histological features of Wells’s syndrome together with elevated antibody titers to the excretory-secretory antigen of Toxocara canis. Results: In both patients, the skin lesions disappeared after oral albendazole treatment and no recurrences were observed. The clinical response was followed by a normalization of the Toxocara antibody titer. In contrast, a patient with eczematoid skin lesions, eosinophilia and an elevated Toxocara antibody titer did not benefit from albendazole treatment despite serological normalization. Conclusion: Taken together, these cases lend support to a causal relationship of Toxocara in selected patients with Wells’ syndrome.
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