Successful Treatment of Generalized Pustular Psoriasis With the Interleukin-1-Receptor Antagonist Anakinra: Lack of Correlation With IL1RN Mutations

Letters6 July 2010Successful Treatment of Generalized Pustular Psoriasis With the Interleukin-1-Receptor Antagonist Anakinra: Lack of Correlation With IL1RN MutationsManuelle Viguier, MD, PhD, Philippe Guigue, Cécile Pagès, MD, Asma Smahi, PhD, and Hervé Bachelez, MD, PhDManuelle Viguier, MD, PhDFrom INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôpital Necker-Enfants Malades, 75015 Paris, France; INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôital Necker-Enfants Malades, 75015 Paris, France; and INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France., Philippe GuigueFrom INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôpital Necker-Enfants Malades, 75015 Paris, France; INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôital Necker-Enfants Malades, 75015 Paris, France; and INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France., Cécile Pagès, MDFrom INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôpital Necker-Enfants Malades, 75015 Paris, France; INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôital Necker-Enfants Malades, 75015 Paris, France; and INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France., Asma Smahi, PhDFrom INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôpital Necker-Enfants Malades, 75015 Paris, France; INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôital Necker-Enfants Malades, 75015 Paris, France; and INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France., and Hervé Bachelez, MD, PhDFrom INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôpital Necker-Enfants Malades, 75015 Paris, France; INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôital Necker-Enfants Malades, 75015 Paris, France; and INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-153-1-201007060-00030 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Generalized pustular psoriasis (GPP) is a multisystemic inflammatory disease with disseminated pustular skin involvement that often responds poorly to currently available treatments, including tumor necrosis factor (TNF)-α inhibitors (1). The recent identification of mutations in the IL1RN gene coding for the interleukin (IL)-1 receptor antagonist in an autoinflammatory syndrome with pustular skin involvement provides a rationale to investigate the contribution of the IL-1β pathway to the pathogenesis of GPP (2).Objective: To report the efficacy of the recombinant IL-1 receptor antagonist anakinra and to search for IL1RN gene mutations in 2 patients with GPP.Methods: The IL1RN isoform-1 gene ...References1. Umezawa Y, Ozawa A, Kawasima T, Shimizu H, Terui T, Tagami H, et al. Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity. Arch Dermatol Res. 2003;295 Suppl 1 S43-54. [PMID: 12677432] CrossrefMedlineGoogle Scholar2. Aksentijevich I, Masters SL, Ferguson PJ, Dancey P, Frenkel J, van Royen-Kerkhoff A, et al. An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist. N Engl J Med. 2009;360:2426-37. [PMID: 19494218] CrossrefMedlineGoogle Scholar3. Barksby HE, Lea SR, Preshaw PM, Taylor JJ. The expanding family of interleukin-1 cytokines and their role in destructive inflammatory disorders. Clin Exp Immunol. 2007;149:217-25. [PMID: 17590166] CrossrefMedlineGoogle Scholar4. Mee JB, Cork MJ, di Giovine FS, Duff GW, Groves RW. Interleukin-1: a key inflammatory mediator in psoriasis? Cytokine. 2006;33:72-8. [PMID: 16460958] CrossrefMedlineGoogle Scholar5. Blumberg H, Dinh H, Trueblood ES, Pretorius J, Kugler D, Weng N, et al. Opposing activities of two novel members of the IL-1 ligand family regulate skin inflammation. J Exp Med. 2007;204:2603-14. [PMID: 17908936] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: From INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôpital Necker-Enfants Malades, 75015 Paris, France; INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France; INSERM U780, Assistance Publique-Hôpitaux de Paris Hôital Necker-Enfants Malades, 75015 Paris, France; and INSERM U976, Université Denis-Diderot (Paris 7), Assistance Publique-Hôpitaux de Paris Hôpital Saint-Louis, 75010 Paris, France.Disclosures:Consultancies: M. Viguier (Janssen-Cilag), H. Bachelez (Abbott, Centocor, Janssen-Cilag, Merck Serono, Schering-Plough, Wyeth Pharmaceuticals). Grants received: H. Bachelez (Roche). 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