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 <i>NRLP3</i> 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. Conclusion: 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.