Chronic urticarial, a disease with multiple etiology, is not only a gravely disturbing condition for patients but also a challenge to primary care physicians in terms of its differential diagnosis and treatment. Here, we present two cases of autoimmune chronic spontaneous urticaria, an important subtype of chronic urticaria. The first case had autoimmune thyroid disease, and the second case scleroderma and Sjögren’s syndrome.
In the diagnosis of autoimmune related chronic urticaria, the first step is to differentiate it from urticarial vasculitis and auto-inflammatory disorders. Second, it is necessary to consider whether it is a non-allergic condition caused by food, pseudo-allergic one caused by drugs, or allergic one caused by inducible urticaria.
While a stepwise approach has been widely adopted to treat chronic urticaria, the treatment algorithm for autoimmune chronic spontaneous urticaria remains inconclusive. In this report, the first case was initially treated with antihistamines with hydroxychloroquine added as symptoms persisted. The second case was treated with antihistamines, hydroxychloroquine and azathioprine simultaneously. Both cases were successfully treated with marked improvement of symptoms.