Recently, selective blockade of the IL-1 pathway in HS with anakinra (recombinant IL-1R antagonist) has been investigated in an open-label pilot study (NCT01516749), demonstrating a clinical improvement in six patients with moderate-to-severe HS, as evidenced by a significant reduction in the modified Sartorius score and improvement in quality of life [54]. Here, IL1R1 is linked to histiocytic sarcoma.