When Cold Causes Rash and Many Complaints

Cryopyrin-associated periodic syndromes (CAPS) are among the monogenic autoinflammatory diseases. These diseases are characterized by recurring episodes of inflammation, usually with fever. Cold as a trigger of disease flare-ups, urticarial exanthemas, eye inflammations (conjunctivitis, episcleritis, uveitis), inner ear hearing loss, chronic aseptic meningitis, and skeletal abnormalities are suspicious for CAPS. A genetic investigation should be carried out. Two patients with different phenotypes of the disease are presented.