Definition

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A Chronic Inducible Urticaria

Cold urticaria (CU) clinically manifests as a papular, erythematous, and pruritic rash that occurs during exposure to cold and subsides within less than 2 hours after warming, without leaving scars or pigmentation. It can be superficial (dermal edema) or deep (dermo-hypodermic edema or angioedema). It belongs to the spectrum of chronic inducible urticarias (CIU), for which one or more triggering factors can be identified, unlike chronic spontaneous urticaria (CSU). In CIU, several triggering factors are possible: friction, pressure, heat, vibrations, etc.

CSU and CIU can occur synchronously or not in the same patient.

Its prevalence is estimated to be around 0.05% in Central Europe [Möller, 1996]; in France, no precise figures have been published. CU is the second most frequent CIU after dermographism. In a recent monocentric study in Barcelona conducted between 2002 and 2016, CU represented more than 28% of CIU cases.

There is no consensus in France among practitioners managing CU regarding the definition of the disease, risk factors for severe forms, the role of paraclinical investigations, or the appropriate therapeutic strategy.

Expert Summary

Cold urticaria is a rare condition with a chronic course, and spontaneous remission occurs after several months or years in the vast majority of cases. It can occur at any age, with a clear female predominance in adults.

An association with an atopic background is observed in just over half of adult patients and in two-thirds of a strictly pediatric series. The presence of another form of urticaria, particularly inducible, is common. The most frequently associated inducible urticarias are dermographism and cholinergic urticaria.

Classification

The classification used is based on the severity of clinical signs during previous cold urticaria episodes:

The 3 Degrees of Severity of Cold Urticaria (only for primary typical or atypical cold urticarias; secondary cold urticarias are excluded):

• Type Ⅰ: Urticaria and/or localized angioedema in the area of cold contact (without localized asphyxiating laryngeal edema)
• Type Ⅱ: Generalized urticaria and/or angioedema without hypotension
• Type Ⅲ: Severe systemic reaction or asphyxiating pharyngo-laryngeal edema

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