慢性蕁麻疹的致病因很多，若未找出並有效治療，患者的生活品質常受嚴重影響。鑑別診斷時，首先要和蕁麻疹性血管炎及自體發炎疾病做鑑別，其次要考慮是否為食物引起的非過敏、藥物造成的偽過敏，或是誘發性蕁麻疹，若皆不是就得要探查是否和自體免疫相關，本文提供自體免疫性慢性蕁麻疹的診斷及治療經驗。兩個慢性蕁麻疹案例，一位合併自體免疫甲狀腺疾病，另一位合併硬皮症及修格連氏症候群。多數指引建議採升階式來治療慢性蕁麻疹，然而自體免疫性慢性自發性蕁麻疹的治療仍未有定論，案例一由抗組織胺開始，症狀未明顯改善即加上免疫抑制劑hydroxychloroquine，案例二直接使用抗組織胺，合併免疫抑制劑hydroxychloroquine及azathioprine，皆有效改善。

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.