Notably, of the 19 patients with HAVCR2 p.Y82C variant, 3 homozygotes (15.8%) had an episode of idiopathic HLH/HLH-like systemic illnesses before the appearance of panniculitis-like skin lesions with recurrent HLH/HLH-like systemic illnesses. This evidence concerns the gene HAVCR2 and hemophagocytic syndrome.