Familial Cold Urticaria

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Abstract
Eleven patients with familial cold urticaria were found among 19 subjects in three generations of a Dutch family. There was autosomal dominant inheritance. The symptoms, which started during the first years of life, were noticed one half to three hours after generalized exposure to cold air. The patients had erythematous plaques, urticarial lesions, petechiae, and edematous swellings of the exposed parts, often accompanied by fever, chills, and joint complaints. Symptoms ranged from slight to incapacitating. In two patients, the symptoms could be reproduced in the cold room. A leukocytosis preceded and accompanied the symptoms. During cold exposure, there was a sharp decrease in the level of chymotrypsin-inhibiting capacity in the plasma, which suggests that during the active phase of the disease a chymotrypsin-like enzyme is released.

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