The exposure of skin to cold results in the release of histamine, leukotrienes, and pro-inflammatory mast cell mediators which leads to the appearance of pruritic wheals with or without angioedema and in turn the onset of urticaria. According to the etiology, cold urticaria can be classified into acquired cold urticaria (ACU) and familial cold urticaria (FCU). This study aimed to overview the acquired and FCU. The articles incorporated in writing this review were obtained through searching the scientific databases and using various related keywords. All types of articles were eligible and those written in English language. The current subject was discussed in two main titles, ACU and FCU. Each title overviewed the related condition, diagnosis, and management. There is confusion regarding the classification of urticaria; ACU and FCU are two forms of urticaria based on etiology. However, there is little known about FCU, especially regarding its management. Correct and prompt diagnosis is the key factor to avoid severe consequences of these two conditions.
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