Generalized pustular psoriasis: overview of the status quo and results of a panel discussion.
Journal
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
ISSN: 1610-0387
Titre abrégé: J Dtsch Dermatol Ges
Pays: Germany
ID NLM: 101164708
Informations de publication
Date de publication:
06 2022
06 2022
Historique:
received:
19
08
2021
accepted:
12
02
2022
pubmed:
9
6
2022
medline:
22
6
2022
entrez:
8
6
2022
Statut:
ppublish
Résumé
Generalized pustular psoriasis (GPP) is a rare, severe, potentially life-threatening, autoinflammatory, neutrophilic skin disease that may be accompanied by fever and leukocytosis. This paper describes the current state of knowledge on GPP in terms of classification, (differential) diagnosis and prevalence. We present a comparison of the genetics and pathoimmunology of GPP and psoriasis vulgaris with the central mechanisms of autoimmunology and autoinflammation. The currently available therapeutic options, expert recommendations for therapy, and data from early clinical trials investigating targeted therapies will be summarized. We present the results of our discussion with 13 experts for psoriasis vulgaris and GPP and give an integrated overview of indication and therapy based on our personal experience and present an outlook on further research questions. Collectively, this article highlights the high unmet need in GPP, as there exists no satisfactory method of diagnosis or treatment to date and new treatment options will be of great therapeutic benefit to those affected.
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
753-771Informations de copyright
© 2022 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.
Références
Kharawala S, Golembesky AK, Bohn RL, Esser D. The clinical, humanistic, and economic burden of generalized pustular psoriasis: a structured review. Expert Rev Clin Immunol 2020; 16: 239-52.
Bachelez H. Pustular psoriasis and related pustular skin diseases. Br J Dermatol 2018; 178: 614-8.
Baker H, Ryan TJ. Generalized pustular psoriasis. A clinical and epidemiological study of 104 cases. Br J Dermatol 1968; 80: 771-93.
Choon SE, Lai NM, Mohammad NA et al. Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol 2014; 53: 676-84.
Marrakchi S, Guigue P, Renshaw BR et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011; 365: 620-8.
Zelickson BD, Muller SA. Generalized pustular psoriasis. A review of 63 cases. Arch Dermatol 1991; 127: 1339-45.
Gooderham MJ, Van Voorhees AS, Lebwohl MG. An update on generalized pustular psoriasis. Expert Rev Clin Immunol 2019; 15: 907-19.
Ryan TJ, Baker H. The prognosis of generalized pustular psoriasis. Br J Dermatol 1971; 85: 407-11.
Roth PE, Grosshans E, Bergoend H. [Psoriasis: development and fatal complications]. Ann Dermatol Venereol 1991; 118: 97-105.
Akiyama M, Takeichi T, McGrath JA, Sugiura K. Autoinflammatory keratinization diseases. J Allergy Clin Immunol 2017; 140: 1545-47.
Boehner A, Navarini AA, Eyerich K. Generalized pustular psoriasis - a model disease for specific targeted immunotherapy, systematic review. Exp Dermatol 2018; 27: 1067-77.
Navarini AA, Burden AD, Capon F et al. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol 2017; 31: 1792-9.
Bone RC, Balk RA, Cerra FB et al. Definitions for sepsis and organ failure and guidelines for the use of innovative therapies in sepsis. The ACCP/SCCM Consensus Conference Committee. American College of Chest Physicians/Society of Critical Care Medicine. Chest 1992; 101: 1644-55.
Fujita H, Terui T, Hayama K et al. Japanese guidelines for the management and treatment of generalized pustular psoriasis: The new pathogenesis and treatment of GPP. J Dermatol 2018; 45: 1235-70.
Sidoroff A, Dunant A, Viboud C et al. Risk factors for acute generalized exanthematous pustulosis (AGEP)-results of a multinational case-control study (EuroSCAR). Br J Dermatol 2007; 157: 989-96.
Feldmeyer L, Heidemeyer K, Yawalkar N. Acute generalized exanthematous pustulosis: pathogenesis, genetic background, clinical variants and therapy. Int J Mol Sci 2016; 17: 1214.
Kardaun SH, Kuiper H, Fidler V, Jonkman MF. The histopathological spectrum of acute generalized exanthematous pustulosis (AGEP) and its differentiation from generalized pustular psoriasis. J Cutan Pathol 2010; 37: 1220-9.
Bachelez H, Choon SE, Marrakchi S et al. Inhibition of the interleukin-36 pathway for the treatment of generalized pustular psoriasis. N Engl J Med 2019; 380: 981-3.
Fredriksson T, Pettersson U. Severe psoriasis-oral therapy with a new retinoid. Dermatologica 1978; 157: 238-44.
Saeki H, Nakagawa H, Nakajo K et al. Efficacy and safety of ixekizumab treatment for Japanese patients with moderate to severe plaque psoriasis, erythrodermic psoriasis and generalized pustular psoriasis: Results from a 52-week, open-label, phase 3 study (UNCOVER-J). J Dermatol 2017; 44: 355-62.
Morita A, Yamazaki F, Matsuyama T et al. Adalimumab treatment in Japanese patients with generalized pustular psoriasis: Results of an open-label phase 3 study. J Dermatol 2018; 45: 1371-80.
Ghoreschi K, Balato A, Enerbäck C, Sabat R. Therapeutics targeting the IL-23 and IL-17 pathway in psoriasis. Lancet 2021; 397: 754-66.
Hawkes JE, Yan BY, Chan TC, Krueger JG. Discovery of the IL-23/IL-17 signaling pathway and the treatment of psoriasis. J Immunol 2018; 201: 1605-13.
Schön MP. Adaptive and innate immunity in psoriasis and other inflammatory disorders. Front Immunol 2019; 10: 1764.
Rood JE, Behrens EM. Inherited autoinflammatory syndromes. Annu Rev Pathol 2022; 17: 227-49.
Berki DM, Liu L, Choon SE et al. Activating CARD14 mutations are associated with generalized pustular psoriasis but rarely account for familial recurrence in psoriasis vulgaris. J Invest Dermatol 2015; 135: 2964-70.
Sugiura K, Muto M, Akiyama M. CARD14 c.526G>C (p.Asp176His) is a significant risk factor for generalized pustular psoriasis with psoriasis vulgaris in the Japanese cohort. J Invest Dermatol 2014; 134: 1755-57.
Onoufriadis A, Simpson MA, Pink AE et al. Mutations in IL36RN/IL1F5 are associated with the severe episodic inflammatory skin disease known as generalized pustular psoriasis. Am J Hum Genet 2011; 89: 432-7.
Twelves S, Mostafa A, Dand N et al. Clinical and genetic differences between pustular psoriasis subtypes. J Allergy Clin Immunol 2019; 143: 1021-6.
Zhou J, Luo Q, Cheng Y et al. An update on genetic basis of generalized pustular psoriasis (Review). Int J Mol Med 2021; 47: 118.
Mössner R, Wilsmann-Theis D, Oji V et al. The genetic basis for most patients with pustular skin disease remains elusive. Br J Dermatol 2018; 178: 740-8.
Setta-Kaffetzi N, Simpson MA, Navarini AA et al. AP1S3 mutations are associated with pustular psoriasis and impaired Toll-like receptor 3 trafficking. Am J Hum Genet 2014; 94: 790-7.
Frey S, Sticht H, Wilsmann-Theis D et al. Rare loss-of-function mutation in SERPINA3 in generalized pustular psoriasis. J Invest Dermatol 2020; 140: 1451-55. e13.
Haskamp S, Bruns H, Hahn M et al. Myeloperoxidase modulates inflammation in generalized pustular psoriasis and additional rare pustular skin diseases. Am J Hum Genet 2020; 107: 527-38.
Vergnano M, Mockenhaupt M, Benzian-Olsson N et al. Loss-of-function myeloperoxidase mutations are associated with increased neutrophil counts and pustular skin disease. Am J Hum Genet 2020; 107: 539-43.
Han JW, Wang Y, Alateng C et al. Tumor necrosis factor-alpha induced protein 3 interacting protein 1 gene polymorphisms and pustular psoriasis in Chinese Han population. Chin Med J (Engl) 2016; 129: 1519-24.
Choon SE, Lebwohl MG, Marrakchi S et al. Study protocol of the global Effisayil 1 Phase II, multicentre, randomised, double-blind, placebo-controlled trial of spesolimab in patients with generalized pustular psoriasis presenting with an acute flare. BMJ Open 2021; 11: e043666.
Johnston A, Xing X, Guzman AM et al. IL-1F5, -F6, -F8, and -F9: a novel IL-1 family signaling system that is active in psoriasis and promotes keratinocyte antimicrobial peptide expression. J Immunol. 2011; 186: 2613-22.
Uppala R, Tsoi LC, Harms PW et al. “Autoinflammatory psoriasis”-genetics and biology of pustular psoriasis. Cell Mol Immunol 2021; 18: 307-17.
Catapano M, Vergnano M, Romano M et al. IL-36 promotes systemic IFN-I responses in severe forms of psoriasis. J Invest Dermatol 2020; 140: 816-26. e3.
Johnston A, Xing X, Wolterink L et al. IL-1 and IL-36 are dominant cytokines in generalized pustular psoriasis. J Allergy Clin Immunol 2017; 140: 109-20.
Arakawa A, Vollmer S, Besgen P et al. Unopposed IL-36 activity promotes clonal CD4(+) T-cell responses with IL-17A production in generalized pustular psoriasis. J Invest Dermatol 2018; 138: 1338-47.
Vigne S, Palmer G, Lamacchia C et al. IL-36R ligands are potent regulators of dendritic and T cells. Blood 2011; 118: 5813-23.
Ohkawara A, Yasuda H, Kobayashi H et al. Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background. Acta Derm Venereol 1996; 76: 68-71.
Augey F, Renaudier P, Nicolas JF. Generalized pustular psoriasis (Zumbusch): a French epidemiological survey. Eur J Dermatol 2006; 16: 669-73.
Strober B, Kotowsky N, Medeiros R et al. Unmet medical needs in the treatment and management of generalized pustular psoriasis flares: Evidence from a survey of corrona registry dermatologists. Dermatol Ther (Heidelb) 2021; 11: 529-41.
Borges-Costa J, Silva R, Goncalves L et al. Clinical and laboratory features in acute generalized pustular psoriasis: a retrospective study of 34 patients. Am J Clin Dermatol 2011; 12: 271-6.
Jin H, Cho HH, Kim WJ et al. Clinical features and course of generalized pustular psoriasis in Korea. J Dermatol 2015; 42: 674-8.
Bachelez H. Pustular psoriasis: The dawn of a new era. Acta Derm Venereol. 2020; 100: adv00034.
Robinson A, Van Voorhees AS, Hsu S et al. Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol 2012; 67: 279-88.
Kromer C, Loewe E, Schaarschmidt ML et al. Drug survival in the treatment of generalized pustular psoriasis: A retrospective multicenter study. Dermatol Ther 2021; 34: e14814.
Wang WM, Jin HZ. Biologics in the treatment of pustular psoriasis. Expert Opin Drug Saf 2020; 19: 969-80.
Ohata C, Tsuruta N, Yonekura K et al. Clinical characteristics of Japanese pustular psoriasis: A multicenter observational study. J Dermatol 2022; 49: 142-50.
Miyachi H, Konishi T, Kumazawa R et al. Treatments and outcomes of generalized pustular psoriasis: A cohort of 1516 patients in a nationwide inpatient database in Japan. J Am Acad Dermatol 2021; S0190-9622; 01108-7.
Ozawa A, Ohkido M, Haruki Y et al. Treatments of generalized pustular psoriasis: a multicenter study in Japan. J Dermatol 1999; 26: 141-9.
Torii H, Nakano M, Yano T et al. Efficacy and safety of dose escalation of infliximab therapy in Japanese patients with psoriasis: Results of the SPREAD study. J Dermatol 2017; 44: 552-9.
Post H, Magnolo N, Bohm M et al. [Successful treatment of generalized pustular psoriasis with certolizumab]. Hautarzt 2021; 72: 992-95.
Imafuku S, Honma M, Okubo Y et al. Efficacy and safety of secukinumab in patients with generalized pustular psoriasis: A 52-week analysis from phase III open-label multicenter Japanese study. J Dermatol 2016; 43: 1011-7.
Morita A, Okubo Y, Morisaki Y et al. Ixekizumab 80 mg every 2 weeks treatment beyond week 12 for Japanese patients with generalized pustular psoriasis and erythrodermic psoriasis. Dermatol Ther (Heidelb) 2022; 12(2): 481-94.
Yamasaki K, Nakagawa H, Kubo Y et al. Efficacy and safety of brodalumab in patients with generalized pustular psoriasis and psoriatic erythroderma: results from a 52-week, open-label study. Br J Dermatol 2017; 176: 741-51.
Sano S, Kubo H, Morishima H et al. Guselkumab, a human interleukin-23 monoclonal antibody in Japanese patients with generalized pustular psoriasis and erythrodermic psoriasis: Efficacy and safety analyses of a 52-week, phase 3, multicenter, open-label study. J Dermatol 2018; 45: 529-39.
A study to assess efficacy and safety of two different dose regimens of risankizumab administered subcutaneously in Japanese subjects with generalized pustular psoriasis or erythrodermic psoriasis. Available from: https://clinicaltrialsgov/ct2/show/NCT03022045, Last accessed February 4, 2022.
Hüffmeier U, Watzold M, Mohr J et al. Successful therapy with anakinra in a patient with generalized pustular psoriasis carrying IL36RN mutations. Br J Dermatol 2014; 170: 202-4.
Skendros P, Papagoras C, Lefaki I et al. Successful response in a case of severe pustular psoriasis after interleukin-1beta inhibition. Br J Dermatol 2017; 176: 212-5.
Mansouri B, Richards L, Menter A. Treatment of two patients with generalized pustular psoriasis with the interleukin-1beta inhibitor gevokizumab. Br J Dermatol 2015; 173: 239-41.
Bachelez H, Choon SE, Marrakchi S et al. Trial of spesolimab for generalized pustular psoriasis. N Engl J Med. 2021; 385: 2431-40.
A study to evaluate the efficacy and safety of ANB019 in subjects with generalized pustular psoriasis (GPP). Available from: https://clinicaltrialsgov/ct2/show/NCT03619902, Last accessed on February 4, 2022.
Mahil SK, Catapano M, Di Meglio P et al. An analysis of IL-36 signature genes and individuals with IL1RL2 knockout mutations validates IL-36 as a psoriasis therapeutic target. Sci Transl Med 2017; 9: eaan2514.
Cornelius V, Wilson R, Cro S et al. A small population, randomised, placebo-controlled trial to determine the efficacy of anakinra in the treatment of pustular psoriasis: study protocol for the APRICOT trial. Trials 2018; 19: 465.
Lutz V, Lipsker D. Acitretin- and tumor necrosis factor inhibitor-resistant acrodermatitis continua of hallopeau responsive to the interleukin 1 receptor antagonist anakinra. Arch Dermatol 2012; 148: 297-9.
Todorovic V, Su Z, Putman CB et al. Small molecule IL-36gamma antagonist as a novel therapeutic approach for plaque psoriasis. Sci Rep 2019; 9: 9089.
Iznardo H, Puig L. Exploring the role of IL-36 cytokines as a new target in psoriatic disease. Int J Mol Sci. 2021; 22: 4344.
Bassoy EY, Towne JE, Gabay C. Regulation and function of interleukin-36 cytokines. Immunol Rev 2018; 281: 169-78.
Gabay C, Towne JE. Regulation and function of interleukin-36 cytokines in homeostasis and pathological conditions. J Leukoc Biol 2015; 97: 645-52.
Ganesan R, Raymond EL, Mennerich D et al. Generation and functional characterization of anti-human and anti-mouse IL-36R antagonist monoclonal antibodies. MAbs 2017; 9: 1143-54.
Haefeli M, Elfering A. Pain assessment. Eur Spine J 2006; 15(Suppl 1): S17-24.
Hawker GA, Mian S, Kendzerska T, French M. Measures of adult pain: Visual Analog Scale for Pain (VAS Pain), Numeric Rating Scale for Pain (NRS Pain), McGill Pain Questionnaire (MPQ), Short-Form McGill Pain Questionnaire (SF-MPQ), Chronic Pain Grade Scale (CPGS), Short Form-36 Bodily Pain Scale (SF-36 BPS), and Measure of Intermittent and Constant Osteoarthritis Pain (ICOAP). Arthritis Care Res (Hoboken). 2011; 63(Suppl 11): S240-52.
Nestle FO, Kaplan DH, Barker J. Psoriasis. N Engl J Med 2009; 361: 496-509.