Abstract
urticarial rash is a hallmark symptom of systemic autoinflammatory diseases such as Schnitzler's syndrome (SchS). Clinically, the urticarial rash cannot be distinguished from wheals in chronic spontaneous urticaria (CSU). Thus, it is commonly misdiagnosed as CSU.1 SchS is characterized by monoclonal gammopathy, long-term complications including the development of lymphoma, and systemic inflammation.
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