Chronic urticaria in infants as the first manifestation of autoinflammatory disease.

We report two unrelated infants in whom chronic urticaria was the first clinical manifestation of cryopyrin-associated periodic syndrome, which should be suspected in infants with early-onset chronic urticaria, especially if there is a neutrophil-rich infiltrate in the skin biopsy. Early diagnosis of cryopyrin-associated periodic syndrome may lead to early and successful treatment with anti-interleukin-1 medications.