Urticarial vasculitis can present in a variety of ways, ranging from a primarily cutaneous disease consisting of chronic urticaria to a lupus-like disease with severe cardiopulmonary disease. Low complement levels and positive anti-C1q antibodies are markers of more severe disease. Care must be taken to look for an underlying condition. The mainstay of therapy is treatment of any underlying condition. Therapies most often employed include corticosteroids, antihistamine, and dapsone, but many others have been utilized.