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1 Laboratory for Investigative Dermatology, Rockefeller University, New York City, New York, USA.
2 University of Michigan, Ann Arbor, Michigan, USA.
3 Icahn School of Medicine at Mount Sinai, New York, New York, USA.
4 Nagoya City University, Nagoya, Japan.
5 Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
6 Dalhousie University, Halifax, Nova Scotia, Canada.
7 Dermatology Centre, Northern Care Alliance NHS Foundation Trust & Division of Musculoskeletal and Dermatological Sciences, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom.
8 Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
9 Yale University School of Medicine, New Haven, Connecticut, USA and Central Connecticut Dermatology Research, Cromwell, Connecticut, USA.
10 School of Infection and Immunity, University of Glasgow, Glasgow, United Kingdom.
11 School of Medicine, Second Affiliated Hospital, Zhejiang University, Hangzhou, Zhejiang, China.
12 Mayo Clinic, Scottsdale, Arizona, USA.
13 Washington University School of Medicine, St. Louis, Missouri, USA.
14 St. John's Institute of Dermatology, King's College London, London, United Kingdom.
15 UT Southwestern Medical Center, Dallas, Texas, USA.
1 Laboratory for Investigative Dermatology, Rockefeller University, New York City, New York, USA.
2 University of Michigan, Ann Arbor, Michigan, USA.
3 Icahn School of Medicine at Mount Sinai, New York, New York, USA.
4 Nagoya City University, Nagoya, Japan.
5 Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
6 Dalhousie University, Halifax, Nova Scotia, Canada.
7 Dermatology Centre, Northern Care Alliance NHS Foundation Trust & Division of Musculoskeletal and Dermatological Sciences, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom.
8 Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
9 Yale University School of Medicine, New Haven, Connecticut, USA and Central Connecticut Dermatology Research, Cromwell, Connecticut, USA.
10 School of Infection and Immunity, University of Glasgow, Glasgow, United Kingdom.
11 School of Medicine, Second Affiliated Hospital, Zhejiang University, Hangzhou, Zhejiang, China.
12 Mayo Clinic, Scottsdale, Arizona, USA.
13 Washington University School of Medicine, St. Louis, Missouri, USA.
14 St. John's Institute of Dermatology, King's College London, London, United Kingdom.
15 UT Southwestern Medical Center, Dallas, Texas, USA.
People with generalized pustular psoriasis experience underlying skin inflammation, even in the absence of flares. Spesolimab treatment helps control the inflammation and prevent future flares.
Conflict of interest: The conflict-of-interest statement is available in the Supplemental Material.
Figures
Figure 1. Changes in proinflammatory gene expression…
Figure 1. Changes in proinflammatory gene expression during the study period.
Alternative biopsies are indicated…
Figure 1. Changes in proinflammatory gene expression during the study period.
Alternative biopsies are indicated by asterisks. The graph shows the gene expression of proinflammatory mediators in all 18 patients who participated in the biomarker study at all time points, including flare and postflare treatment and at the end of the study (week 48). Patients who had a complete series of biopsies throughout the studies are shown (Patients 1–7). One patient had more than 1 biopsy (Patient 6). DEFB4A, defensin β 4A; EoT, end of trial; LD, loading dose; NCF, neutrophil cytosolic factor; q12w, every 12 weeks; q4w, every 4 weeks; TNF, tumor necrosis factor.
Bachelez H, et al. Inhibition of the interleukin-36 pathway for the treatment of generalized pustular psoriasis. N Engl J Med. 2019;380(10):981–983. doi: 10.1056/NEJMc1811317.
-
DOI
-
PubMed
Morita A, et al. Efficacy and safety of subcutaneous spesolimab for the prevention of generalised pustular psoriasis flares (Effisayil 2): an international, multicentre, randomised, placebo-controlled trial. Lancet. 2023;402(10412):1541–1551. doi: 10.1016/S0140-6736(23)01378-8.
-
DOI
-
PubMed
Tauber M, et al. IL36RN mutations affect protein expression and function: a basis for genotype-phenotype correlation in pustular diseases. J Invest Dermatol. 2016;136(9):1811–1819. doi: 10.1016/j.jid.2016.04.038.
-
DOI
-
PubMed
Jordan CT, et al. Rare and common variants in CARD14, encoding an epidermal regulator of NF-kappaB, in psoriasis. Am J Hum Genet. 2012;90(5):796–808. doi: 10.1016/j.ajhg.2012.03.013.
-
DOI
-
PMC
-
PubMed
Mahil SK, et al. AP1S3 mutations cause skin autoinflammation by disrupting keratinocyte autophagy and up-regulating IL-36 production. J Invest Dermatol. 2016;136(11):2251–2259. doi: 10.1016/j.jid.2016.06.618.
-
DOI
-
PMC
-
PubMed