Complete remission of severe idiopathic cold urticaria on interleukin-1 receptor antagonist (anakinra)

A 62-year-old patient had suffered from severe cold intolerance with an urticarial rash and oropharyngeal angio-oedema upon cold exposure since early childhood. This could be provoked by the ice cube test and by exposure in a cold room. Her family history was negative, and she did not carry any mutations in the NRLP3 gene. Treatment with IL-1 receptor antagonist anakinra resulted in complete resolution of these symptoms, with a radical beneficial change in her quality of life. In recent years this patient had developed progressive neurological symptoms leading to a diagnosis of amyotrophic lateral sclerosis (ALS), which seems unrelated to the idiopathic cold urticaria. The neurological symptoms did not respond to anakinra treatment and were eventually fatal.

We describe the first case of IL-1RA treatment in idiopathic cold urticaria with good response. Anakinra had no effect on the progression of her symptoms of ALS.