| Summary points: | The authors present the case of a 62-year-old Caucasian woman with severe cold intolerance with an urticarial rash and oropharyngeal angio-oedema upon cold exposure since early childhood. Physical examination, ice cube test gene sequencing, extensive ancillary investigations, and other tests are described. The authors describe the first reported case of anakinara treatment in idiopathic cold urticaria with good response. |