{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["10(27)"],"submitter":["Schmauch E"],"pubmed_abstract":["Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease with a poorly understood pathogenesis. Through a molecularly driven precision medicine approach and an extensive mechanistic pathway analysis in PRP skin samples, compared to psoriasis, atopic dermatitis, healed PRP, and healthy controls, we identified IL-1β as a key mediator, orchestrating an NF-κB-mediated IL-1β-CCL20 axis, including activation of CARD14 and NOD2. Treatment of three patients with the IL-1 antagonists anakinra and canakinumab resulted in rapid clinical improvement and reversal of the PRP-associated molecular signature with a 50% improvement in skin lesions after 2 to 3 weeks. This transcriptional signature was consistent with in vitro stimulation of keratinocytes with IL-1β. With the central role of IL-1β "],"journal":["Science advances"],"pagination":["eado2365"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11221491"],"repository":["biostudies-literature"],"pubmed_title":["Targeting IL-1 controls refractory pityriasis rubra pilaris."],"pmcid":["PMC11221491"],"pubmed_authors":["Kahlenberg JM","Mellett M","Snijder B","Gudjonsson JE","Satyam A","Nilsson J","Xing X","Navarini A","Johansen P","Tsoi LC","Tsokos GC","Kolios AGA","Sarkar MK","Kellis M","Nobbe S","French LE","Severin Y","Kaikkonen MU","Linna-Kuosmanen S","Contassot E","Mangold A","Conrad C","Schmauch E"],"additional_accession":[]},"is_claimable":false,"name":"Targeting IL-1 controls refractory pityriasis rubra pilaris.","description":"Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease with a poorly understood pathogenesis. Through a molecularly driven precision medicine approach and an extensive mechanistic pathway analysis in PRP skin samples, compared to psoriasis, atopic dermatitis, healed PRP, and healthy controls, we identified IL-1β as a key mediator, orchestrating an NF-κB-mediated IL-1β-CCL20 axis, including activation of CARD14 and NOD2. Treatment of three patients with the IL-1 antagonists anakinra and canakinumab resulted in rapid clinical improvement and reversal of the PRP-associated molecular signature with a 50% improvement in skin lesions after 2 to 3 weeks. This transcriptional signature was consistent with in vitro stimulation of keratinocytes with IL-1β. With the central role of IL-1β ","dates":{"release":"2024-01-01T00:00:00Z","publication":"2024 Jul","modification":"2026-06-02T21:18:35.078Z","creation":"2025-04-04T12:21:35.411Z"},"accession":"S-EPMC11221491","cross_references":{"pubmed":["38959302"],"doi":["10.1126/sciadv.ado2365"]}}