Off-Label studies on anakinra in dermatology: a review

References

• Dinarello CA, Simon A, van der Meer JW. Treating inflammation by blocking interleukin-1 in a broad spectrum of diseases. Nat Rev Drug Discov. 2012;11(8):633–652.
   PubMed Web of Science ®Google Scholar
• Kineret (anakinra) [package insert]. Stockholm, Sweden: Swedish Orphan Biovitrum AB.; 2012.
   Google Scholar
• Ramirez J, Canete JD. Anakinra for the treatment of rheumatoid arthritis: a safety evaluation. Expert Opin Drug Saf. 2018;17(7):727–732.
   PubMed Web of Science ®Google Scholar
• Bettiol A, et al. Unveiling the efficacy, safety, and tolerability of anti-interleukin-1 treatment in monogenic and multifactorial autoinflammatory diseases. Int J Mol Sci. 2019;20(8):20.
   Web of Science ®Google Scholar
• Kullenberg T, Löfqvist M, Leinonen M, et al. Long-term safety profile of anakinra in patients with severe cryopyrin-associated periodic syndromes. Rheumatology (Oxford). 2016;55(8):1499–1506.
   PubMed Web of Science ®Google Scholar
• Youngstein T, Hoffmann P, Gül A, et al. International multi-centre study of pregnancy outcomes with interleukin-1 inhibitors. Rheumatology (Oxford). 2017;56(12):2102–2108.
   PubMed Web of Science ®Google Scholar
• FDA approves SOBI’s Kineret for the treatment of NOMID 2013; [updated 2013 January 8, cited 2020 March 16]. Available from: https://www.thepharmaletter.com/article/fda-approves-sobi-s-kineret-for-the-treatment-of-nomid.
   Google Scholar
• Ouzzani M, Hammady H, Fedorowicz Z, et al. Rayyan-a web and mobile app for systematic reviews. Syst Rev. 2016;5(1):210.
   PubMedGoogle Scholar
• Grayson PC, Yazici Y, Merideth M, et al. Treatment of mucocutaneous manifestations in Behcet’s disease with anakinra: a pilot open-label study. Arthritis Res Ther. 2017;19(1):69.
   PubMed Web of Science ®Google Scholar
• Tzanetakou V, Kanni T, Giatrakou S, et al. Safety and efficacy of anakinra in severe Hidradenitis suppurativa: a randomized clinical trial. JAMA Dermatol. 2016;152(1):52–59.
   PubMed Web of Science ®Google Scholar
• Leslie KS, Tripathi SV, Nguyen TV, et al. An open-label study of anakinra for the treatment of moderate to severe Hidradenitis suppurativa. J Am Acad Dermatol. 2014;70(2):243–251.
   PubMed Web of Science ®Google Scholar
• Kuemmerle-Deschner JB, Wittkowski H, Tyrrell PN, et al. Treatment of muckle-wells syndrome: analysis of two IL-1-blocking regimens. Arthritis Res Ther. 2013;15(3):R64.
   PubMed Web of Science ®Google Scholar
• Kuemmerle-Deschner JB, Tyrrell PN, Koetter I, et al. Efficacy and safety of anakinra therapy in pediatric and adult patients with the autoinflammatory muckle-wells syndrome. Arthritis Rheum. 2011;63(3):840–849.
   PubMed Web of Science ®Google Scholar
• Wendling D, Prati C, Aubin F. Anakinra treatment of SAPHO syndrome: short-term results of an open study. Ann Rheum Dis. 2012;71(6):1098–1100.
   PubMed Web of Science ®Google Scholar
• Miyamae T. Cryopyrin-associated periodic syndromes: diagnosis and management. Paediatr Drugs. 2012;14(2):109–117.
   PubMed Web of Science ®Google Scholar
• Leslie KS, Lachmann HJ, Bruning E, et al. Phenotype, genotype, and sustained response to anakinra in 22 patients with autoinflammatory disease associated with CIAS-1/NALP3 mutations. Arch Dermatol. 2006;142(12):1591–1597.
   PubMedGoogle Scholar
• Ma L, Lee S, Montieth A, et al. Cryopyrin-associated periodic syndrome-associated Uveitis and Papillitis. Retin Cases Brief Rep. 2018. DOI:https://doi.org/10.1097/ICB.0000000000000756
   Google Scholar
• Chan L, Campbell DE, Ming AG. Erythema nodosum in an adolescent patient with cryopyrin-associated periodic syndrome. Clin Case Rep. 2018;6(7):1241–1245.
   PubMed Web of Science ®Google Scholar
• Sathishkumar D, Al-Abadi E, Nicklaus-Wollenteit I, et al. Early-onset urticaria: a marker of cryopyrin-associated periodic syndrome. Clin Exp Dermatol. 2017;42(5):579–581.
   PubMed Web of Science ®Google Scholar
• Herbert VG, Ahmadi-Simab K, Reich K, et al. Neutrophilic urticarial dermatosis (NUD) indicating Cryopyrin-associated periodic syndrome associated with a novel mutation of the NLRP3 gene. J Eur Acad Dermatol Venereol. 2016;30(5):852–853.
   PubMed Web of Science ®Google Scholar
• Imaizumi R, Ishii Y, Miki K, et al. A case of cryopyrin-associated periodic syndrome with kidney transplant failure. CEN Case Rep. 2015;4(1):1–5.
   PubMed Web of Science ®Google Scholar
• Posch C, Kaulfersch W, Rappersberger K. Cryopyrin-associated periodic syndrome. Pediatr Dermatol. 2014;31(2):228–231.
   PubMed Web of Science ®Google Scholar
• Broderick L, Tremoulet AH, Burns JC, et al. Prolonged urticaria and fever in a toddler. Allergy Asthma Proc. 2012;33(3):297–301.
   PubMed Web of Science ®Google Scholar
• Dutra LA, Jesus AA, Vasconcellos M, et al. Cryopyrin associated periodic syndrome with neurological involvement in a 50-year-old patient. Eur J Neurol. 2014;21(3):e27–8.
   PubMed Web of Science ®Google Scholar
• Ramos E, Aróstegui JI, Campuzano S, et al. Positive clinical and biochemical responses to anakinra in a 3-yr-old patient with cryopyrin-associated periodic syndrome (CAPS). Rheumatology (Oxford). 2005;44(8):1072–1073.
   PubMed Web of Science ®Google Scholar
• Maksimovic L, Stirnemann J, Caux F, et al. New CIAS1 mutation and anakinra efficacy in overlapping of Muckle-Wells and familial cold autoinflammatory syndromes. Rheumatology (Oxford). 2007;47(3):309–310.
   Web of Science ®Google Scholar
• Wittkowski H, Kuemmerle-Deschner JB, Austermann J, et al. MRP8 and MRP14, phagocyte-specific danger signals, are sensitive biomarkers of disease activity in cryopyrin-associated periodic syndromes. Ann Rheum Dis. 2011;70(12):2075–2081.
   PubMed Web of Science ®Google Scholar
• Fingerhutova S, et al. Muckle-wells syndrome across four generations in one czech family: natural course of the disease. Front Immunol. 2019;10:802.
   PubMed Web of Science ®Google Scholar
• Kuemmerle-Deschner JB, Lohse P, Koetter I, et al. NLRP3 E311K mutation in a large family with Muckle-Wells syndrome–description of a heterogeneous phenotype and response to treatment. Arthritis Res Ther. 2011;13(6):R196.
   PubMed Web of Science ®Google Scholar
• Lepore L, Paloni G, Caorsi R, et al. Follow-up and quality of life of patients with cryopyrin-associated periodic syndromes treated with Anakinra. J Pediatr. 2010;157(2):310–315.e1.
   PubMed Web of Science ®Google Scholar
• Eskola V, Pohjankoski H, Kröger L, et al. Cryopyrin-associated periodic syndrome in early childhood can be successfully treated with interleukin-1 blockades. Acta Paediatr. 2018;107(4):577–580.
   PubMed Web of Science ®Google Scholar
• Kim KY, Kim SY, Park SE, et al. The first report on clinical manifestation of cryopyrin-associated periodic syndrome in korean children. Pediatr Infect Vaccine. 2018;25(3):113.
   Google Scholar
• Mirault T, Launay D, Cuisset L, et al. Recovery from deafness in a patient with Muckle-Wells syndrome treated with anakinra. Arthritis Rheum. 2006;54(5):1697–1700.
   PubMedGoogle Scholar
• Sabroe RA, Stokes CA, Parker LC, et al. Muckle-Wells syndrome without mutation in exon 3 of the NLRP3 gene, identified by evidence of excessive monocyte production of functional interleukin 1beta and rapid response to anakinra. Clin Exp Dermatol. 2013;38(8):874–877.
   PubMed Web of Science ®Google Scholar
• Naz Villalba E, Gomez de la Fuente E, Caro Gutierrez D, et al. Muckle-Wells syndrome: a case report with an NLRP3 T348M mutation. Pediatr Dermatol. 2016;33(5):e311–4.
   PubMed Web of Science ®Google Scholar
• Alexander T. Successful treatment of acute visual loss in Muckle-Wells syndrome with interleukin 1 receptor antagonist. Ann Rheum Dis. 2005;64(8):1245–1246.
   PubMed Web of Science ®Google Scholar
• Eungdamrong J, et al. Muckle-Wells treatment with anakinra. Dermatol Online J. 2013;19(12):20720.
   PubMedGoogle Scholar
• Dalgic B, Egritas O, Sari S, et al. A variant Muckle-Wells syndrome with a novel mutation in CIAS1 gene responding to anakinra. Pediatr Nephrol. 2007;22(9):1391–1394.
   PubMed Web of Science ®Google Scholar
• Mensa-Vilaro A, Teresa Bosque M, Magri G, et al. Brief report: late-onset cryopyrin-associated periodic syndrome due to myeloid-restricted somatic NLRP3 mosaicism. Arthritis Rheumatol. 2016;68(12):3035–3041.
   PubMed Web of Science ®Google Scholar
• Gerard S, Le Goff B, Maugars Y, et al. Lasting remission of a Muckle-Wells syndrome with CIAS-1 mutation using half-dose anakinra. Joint Bone Spine. 2007;74(6):659.
   PubMed Web of Science ®Google Scholar
• Stew BT, Fishpool SJC, Owens D, et al. Muckle-Wells syndrome: a treatable cause of congenital sensorineural hearing loss. B-ent. 2013;9(2):161–163.
   PubMed Web of Science ®Google Scholar
• Ross JB, Finlayson LA, Klotz PJ, et al. Use of anakinra (Kineret) in the treatment of familial cold autoinflammatory syndrome with a 16-month follow-up. J Cutan Med Surg. 2008;12(1):8–16.
   PubMed Web of Science ®Google Scholar
• Thornton BD, Hoffman HM, Bhat A, et al. Successful treatment of renal amyloidosis due to familial cold autoinflammatory syndrome using an interleukin 1 receptor antagonist. Am J Kidney Dis. 2007;49(3):477–481.
   PubMed Web of Science ®Google Scholar
• O’Connell SM, et al. Response to IL-1-receptor antagonist in a child with familial cold autoinflammatory syndrome. Pediatr Dermatol. 2007;24(1):85–89.
   PubMed Web of Science ®Google Scholar
• Metyas SK, Hoffman HM. Anakinra prevents symptoms of familial cold autoinflammatory syndrome and Raynaud’s disease. J. Rheumatol. 2006;33(10):2085–2087.
   PubMed Web of Science ®Google Scholar
• Hedrich CM, Bruck N, Paul D, et al. “Mutation negative” familial cold autoinflammatory syndrome (FCAS) in an 8-year-old boy: clinical course and functional studies. Rheumatol Int. 2012;32(9):2629–2636.
   PubMed Web of Science ®Google Scholar
• Gusdorf L, Lipsker D. Schnitzler syndrome: a review. Curr Rheumatol Rep. 2017;19(8):46.
   PubMed Web of Science ®Google Scholar
• Neel A, et al. Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler’s syndrome: a French multicenter study. Autoimmun Rev. 2014;13(10):1035–1041.
   PubMed Web of Science ®Google Scholar
• Rowczenio DM, Pathak S, Arostegui JI, et al. Molecular genetic investigation, clinical features, and response to treatment in 21 patients with Schnitzler syndrome. Blood. 2018;131(9):974–981.
   PubMed Web of Science ®Google Scholar
• de Koning HD, Schalkwijk J, van der Ven-Jongekrijg J, et al. Sustained efficacy of the monoclonal anti-interleukin-1 beta antibody canakinumab in a 9-month trial in Schnitzler’s syndrome. Ann Rheum Dis. 2013;72(10):1634–1638.
   PubMed Web of Science ®Google Scholar
• Gran JT, Midtvedt Ø, Haug S, et al. Treatment of Schnitzler’s syndrome with anakinra: report of three cases and review of the literature. Scand J Rheumatol. 2011;40(1):74–79.
   PubMed Web of Science ®Google Scholar
• de Koning HD. Beneficial response to anakinra and thalidomide in Schnitzler’s syndrome. Ann Rheum Dis. 2006;65(4):542–544.
   PubMed Web of Science ®Google Scholar
• Wastiaux H, Barbarot S, Gagey-Caron V, et al. Schnitzler syndrome: a dramatic improvement with anakinra. J Eur Acad Dermatol Venereol. 2009;23(1):85–87.
   PubMed Web of Science ®Google Scholar
• Launay D, Dutoit-Lefevre V, Faure E, et al. Effect of in vitro and in vivo anakinra on cytokines production in Schnitzler syndrome. PLoS One. 2013;8(3):e59327.
   PubMed Web of Science ®Google Scholar
• Szturz P, Adam Z, Hájek R, et al. Successful anakinra therapy in 2 patients with Schnitzler syndrome. Onkologie. 2011;34(5):265–268.
   PubMedGoogle Scholar
• Schuster C, Kränke B, Aberer E, et al. Schnitzler syndrome: response to anakinra in two cases and a review of the literature. Int J Dermatol. 2009;48(11):1190–1194.
   PubMed Web of Science ®Google Scholar
• Bashir M, Bettendorf B, Hariman R. A rare but fascinating disorder: case collection of patients with schnitzler syndrome. Case Rep Rheumatol. 2018;2018:1–4.
   Google Scholar
• Margerin F, Gottenberg JE, Lipsker D. Occurrence of rheumatoid arthritis in a patient treated with anakinra for schnitzler syndrome: a case report. J Rheumatol. 2016;43(7):1447–1447.
   PubMed Web of Science ®Google Scholar
• Koch A, et al. Schnitzler syndrome responding to interleukin-1 antagonist anakinra. J Biol Regul Homeost Agents. 2015;29(1 Suppl):19–21.
   PubMedGoogle Scholar
• Larocca CA, McEvoy JW, Ellis CL, et al. Schnitzler’s syndrome associated with pancreatitis: a disease of IL-1 dysregulation. Clin Rheumatol. 2012;31(1):169–174.
   PubMed Web of Science ®Google Scholar
• Besada E, Nossent H. Dramatic response to IL1-RA treatment in longstanding multidrug resistant Schnitzler’s syndrome: a case report and literature review. Clin Rheumatol. 2010;29(5):567–571.
   PubMed Web of Science ®Google Scholar
• Devlin LA, Wright G, Edgar JD. A rare cause of a common symptom, Anakinra is effective in the urticaria of Schnitzler Syndrome: a case report. Cases J. 2008;1(1):348.
   PubMedGoogle Scholar
• Faggioli P, Tamburello A, Roncoroni L, et al. Schnitzler syndrome, a rare autoinflammatory disease. Complete response to IL-1 blockade. Clin Pract. 2017;7(4):1018.
   PubMedGoogle Scholar
• Hidalgo-Garcia Y, et al. Schnitzler syndrome with response to anakinra monotherapy: 7 years of follow-up. Actas Dermosifiliogr. 2017;108(10):956–958.
   PubMed Web of Science ®Google Scholar
• Gameiro A, Gouveia M, Pereira M, et al. Clinical characterization and long-term follow-up of Schnitzler syndrome. Clin Exp Dermatol. 2016;41(5):461–467.
   PubMed Web of Science ®Google Scholar
• Fairris C, Darvay A. Schnitzler’s syndrome: a diagnostic conundrum. BMJ Case Rep. 2014;2014:pii:bcr2013202210.
   Google Scholar
• Lin RY. Disappearance of monoclonal protein in schnitzler’s syndrome treated with anakinra. Internet J Asthma Allergy Immunol. 2012;8(1):1.
   Google Scholar
• Ryan JG, de Koning HD, Beck LA, et al. IL-1 blockade in Schnitzler syndrome: ex vivo findings correlate with clinical remission. J Allergy Clin Immunol. 2008;121(1):260–262.
   PubMed Web of Science ®Google Scholar
• Klemmer N, Lenain P, Balguerie X, et al. Effectiveness of anti-IL1 in Schnitzler’s syndrome. Joint Bone Spine. 2007;74(5):509–510.
   PubMed Web of Science ®Google Scholar
• Rowczenio DM, Trojer H, Russell T, et al. Clinical characteristics in subjects with NLRP3 V198M diagnosed at a single UK center and a review of the literature. Arthritis Res Ther. 2013;15(1):R30.
   PubMed Web of Science ®Google Scholar
• Thonhofer R, Uitz E, Graninger W. Schnitzler’s syndrome–exacerbation after anti-TNF treatment. Rheumatology (Oxford). 2007;46(6):1041–1042.
   PubMed Web of Science ®Google Scholar
• Nham T, Saleh C, Chu D, et al. Refractory urticaria and the importance of diagnosing Schnitzler’s syndrome. BMJ Case Rep. 2019;12(4):e228546.
   PubMedGoogle Scholar
• Goodman AM, Cohen PR, Li A, et al. Schnitzler syndrome associated with MYD88 L265P mutation. JAAD Case Rep. 2019;5(4):312–316.
   PubMedGoogle Scholar
• Fank H, Caers J, Lambert M, et al. Schnitzler syndrome associated with hairy cell leukemia presenting with chronic urticaria and arthralgias. JAAD Case Rep. 2018;4(4):386–389.
   PubMedGoogle Scholar
• Wilmas K, et al. Schnitzler syndrome in a patient with a family history of monoclonal gammopathy. Dermatol Online J. 2018;24(1):pii:13030/qt41x7q1z5.
   Google Scholar
• Urbanski M, Holfeld K, Milne A, et al. Schnitzler syndrome without a monoclonal gammopathy: a case report. J Cutan Med Surg. 2016;20(6):575–578.
   PubMed Web of Science ®Google Scholar
• Chiu H, Tsai T-F. Long-term control of refractory Schnitzler syndrome with anakinra: a case report. Dermatologica Sinica – DERMATOL SIN. 2010;28(2):82–86.
   Web of Science ®Google Scholar
• Soudet S, Fajgenbaum D, Delattre C, et al. Schnitzler syndrome co-occurring with idiopathic multicentric Castleman disease that responds to anti-IL-1 therapy: a case report and clue to pathophysiology. Curr Res Transl Med. 2018;66(3):83–86.
   PubMed Web of Science ®Google Scholar
• Sharma S, Joske D, Watson D, et al. Schnitzler syndrome: a rare cause of chronic recalcitrant urticaria successfully treated with Anakinra. Australas J Dermatol. 2018;59(3):e222–e224.
   PubMed Web of Science ®Google Scholar
• Ahn MJ, Yu JE, Jeong J, et al. A case of schnitzler’s syndrome without monoclonal gammopathy-associated chronic urticaria treated with anakinra. Yonsei Med J. 2018;59(1):154–157.
   PubMed Web of Science ®Google Scholar
• Gouveia AI, Micaelo M, Pierdomenico F, et al. Schnitzler syndrome: a dramatic response to anakinra. Dermatol Ther (Heidelb). 2016;6(2):299–302.
   PubMed Web of Science ®Google Scholar
• Baresic M. Different therapeutic paths (colchicine vs. anakinra) in two patients with schnitzler’s syndrome. Arch Rheumatol. 2016;31(4):377–380.
   PubMed Web of Science ®Google Scholar
• Cascavilla N, Bisceglia M, D’Arena G. Successful treatment of Schnitzler’s syndrome with anakinra after failure of rituximab trial. Int J Immunopathol Pharmacol. 2010;23(2):633–636.
   PubMed Web of Science ®Google Scholar
• Frischmeyer-Guerrerio PA, Rachamalla R, Saini SS. Remission of Schnitzler syndrome after treatment with anakinra. Ann Allergy Asthma Immunol. 2008;100(6):617–619.
   PubMed Web of Science ®Google Scholar
• Dybowski F. Successful use of anakinra to treat refractory Schnitzler’s syndrome. Clin Exp Rheumatol. 2008;26(2):354–357.
   PubMed Web of Science ®Google Scholar
• Kluger N, et al. Efficacy of interleukin 1 receptor antagonist (anakinra) on a refractory case of Schnitzler’s syndrome. Acta Derm Venereol. 2008;88(3):287–288.
   PubMed Web of Science ®Google Scholar
• Gilson M, et al. Treatment of Schnitzler’s syndrome with anakinra. Clin Exp Rheumatol. 2007;25(6):931.
   PubMed Web of Science ®Google Scholar
• Crouch R, Akhras V, Sarkany R. Schnitzler’s syndrome: successful treatment with anakinra. Australas J Dermatol. 2007;48(3):178–181.
   PubMed Web of Science ®Google Scholar
• Eiling E, Möller M, Kreiselmaier I, et al. Schnitzler syndrome: treatment failure to rituximab but response to anakinra. J Am Acad Dermatol. 2007;57(2):361–364.
   PubMed Web of Science ®Google Scholar
• Schneider SW, Gaubitz M, Luger TA, et al. Prompt response of refractory Schnitzler syndrome to treatment with anakinra. J Am Acad Dermatol. 2007;56(5):S120–S2.
   PubMed Web of Science ®Google Scholar
• Treudler R, Kauer F, Simon JC. Striking effect of the IL-1 receptor antagonist anakinra in chronic urticarial rash with polyclonal increase in IgA and IgG. Acta Derm Venereol. 2007;87(3):280–281.
   PubMed Web of Science ®Google Scholar
• Aouba A, Pressiat C, Pricopi M, et al. Complete remission of Schnitzler syndrome and Waldenstrom macroglobulinemia under rituximab-cyclophosphamide-dexamethasone. Dermatology. 2015;230(1):18–22.
   PubMed Web of Science ®Google Scholar
• Volz T, Wölbing F, Fischer J, et al. Dermal interleukin-1 expression and effective and long-lasting therapy with interleukin-1 receptor antagonist anakinra in Schnitzler syndrome. Acta Derm Venerol. 2012;92(4):393–394.
   PubMed Web of Science ®Google Scholar
• Vandenhende M-A, Bentaberry F, Morlat P, et al. Anakinra: an effective treatment in the Schnitzler syndrome. Joint Bone Spine. 2011;78(6):636–637.
   PubMed Web of Science ®Google Scholar
• Oranges T, Insalaco A, Diociaiuti A, et al. Severe osteoarticular involvement in isotretinoin-triggered acne fulminans: two cases successfully treated with anakinra. J Eur Acad Dermatol Venereol. 2017;31(6):e277–e279.
   PubMed Web of Science ®Google Scholar
• 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(3):297–299.
   PubMedGoogle Scholar
• Amazan E, Ezzedine K, Mossalayi MD, et al. Expression of interleukin-1 alpha in amicrobial pustulosis of the skin folds with complete response to anakinra. J Am Acad Dermatol. 2014;71(2):e53–6.
   PubMed Web of Science ®Google Scholar
• Emmi G, Talarico R, Lopalco G, et al. Efficacy and safety profile of anti-interleukin-1 treatment in Behcet’s disease: a multicenter retrospective study. Clin Rheumatol. 2016;35(5):1281–1286.
   PubMed Web of Science ®Google Scholar
• Cantarini L, Vitale A, Scalini P, et al. Anakinra treatment in drug-resistant Behcet’s disease: a case series. Clin Rheumatol. 2015;34(7):1293–1301.
   PubMed Web of Science ®Google Scholar
• Bilginer Y, Ayaz NA, Ozen S. Anti-IL-1 treatment for secondary amyloidosis in an adolescent with FMF and Behcet’s disease. Clin Rheumatol. 2010;29(2):209–210.
   PubMed Web of Science ®Google Scholar
• Botsios C, Sfriso P, Furlan A, et al. Resistant Behcet disease responsive to anakinra. Ann Intern Med. 2008;149(4):284–286.
   PubMed Web of Science ®Google Scholar
• de Risi-Pugliese T, Cohen Aubart F, Haroche J, et al. Clinical, histological, immunological presentations and outcomes of bullous systemic lupus erythematosus: 10 New cases and a literature review of 118 cases. Semin Arthritis Rheum. 2018;48(1):83–89.
   PubMed Web of Science ®Google Scholar
• Cardis MA, Montealegre Sanchez GA, Goldbach-Mansky R, et al. Recurrent fevers, progressive lipodystrophy, and annular plaques in a child. J Am Acad Dermatol. 2019;80(1):291–295.
   PubMed Web of Science ®Google Scholar
• Wang H, Das L, Tan Hung Tiong J, et al. Candle syndrome: an extended clinical spectrum. Rheumatology (Oxford). 2014;53(11):2119–2120.
   PubMed Web of Science ®Google Scholar
• Groh M, Rogowska K, Monsarrat O, et al. Interleukin-1 receptor antagonist for refractory anti-MDA5 clinically amyopathic dermatomyopathy. Clin Exp Rheumatol. 2015;33(6):904–905.
   PubMed Web of Science ®Google Scholar
• Lenormand C, Lipsker D. Efficiency of interleukin-1 blockade in refractory delayed-pressure urticaria. Ann Intern Med. 2012;157(8):599–600.
   PubMed Web of Science ®Google Scholar
• Aksentijevich I, Masters SL, Ferguson PJ, et al. An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist. N Engl J Med. 2009;360(23):2426–2437.
   PubMed Web of Science ®Google Scholar
• Minkis K, Aksentijevich I, Goldbach-Mansky R, et al. Interleukin 1 receptor antagonist deficiency presenting as infantile pustulosis mimicking infantile pustular psoriasis. Arch Dermatol. 2012;148(6):747–752.
   PubMedGoogle Scholar
• Sozeri B, et al. A novel mutation of interleukin-1 receptor antagonist (IL1RN) in a DIRA patient from Turkey: diagnosis and treatment. Turk J Pediatr. 2018;60(5):588–592.
   PubMed Web of Science ®Google Scholar
• Mendonca LO, Malle L, Donovan FX, et al. Deficiency of Interleukin-1 Receptor Antagonist (DIRA): report of the first indian patient and a novel deletion affecting IL1RN. J Clin Immunol. 2017;37(5):445–451.
   PubMed Web of Science ®Google Scholar
• Schnellbacher C, Ciocca G, Menendez R, et al. Deficiency of interleukin-1 receptor antagonist responsive to anakinra. Pediatr Dermatol. 2013;30(6):758–760.
   PubMed Web of Science ®Google Scholar
• Brau-Javier CN, Gonzales-Chavez J, Toro JR. Chronic cutaneous pustulosis due to a 175-kb deletion on chromosome 2q13: excellent response to anakinra. Arch Dermatol. 2012;148(3):301–304.
   PubMedGoogle Scholar
• Podlipnik S, de la Mora L, Alsina M, et al. Pneumocystis jirovecii pneumonia in a patient with pustular psoriasis with an IL-36RN deficiency treated with infliximab: case report and review of the literature. Australas J Dermatol. 2017;58(2):e44–e47.
   PubMed Web of Science ®Google Scholar
• Rossi-Semerano L, Piram M, Chiaverini C, et al. First clinical description of an infant with interleukin-36-receptor antagonist deficiency successfully treated with anakinra. Pediatrics. 2013;132(4):e1043–e1047.
   PubMed Web of Science ®Google Scholar
• Viguier M. Successful treatment of generalized pustular psoriasis with the interleukin-1-receptor antagonist Anakinra: lack of correlation with IL1RN mutations. Ann Intern Med. 2010;153(1):66–67.
   PubMed Web of Science ®Google Scholar
• 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(1):212–215.
   PubMed Web of Science ®Google Scholar
• Huffmeier U, et al. Successful therapy with anakinra in a patient with generalized pustular psoriasis carrying IL36RN mutations. Br J Dermatol. 2014;170(1):202–204.
   PubMed Web of Science ®Google Scholar
• Andre R, et al. Long-term therapy with anakinra in hidradenitis suppurativa in three patients. Int J Dermatol. 2019;58(11):e208–e209.
   PubMed Web of Science ®Google Scholar
• Menis D, Maroñas-Jiménez L, Delgado-Marquez AM, et al. Two cases of severe hidradenitis suppurativa with failure of anakinra therapy. Br J Dermatol. 2015;172(3):810–811.
   PubMed Web of Science ®Google Scholar
• Abbara S, Georgin-Lavialle S, Stankovic Stojanovic K, et al. Association of hidradenitis suppurativa and familial Mediterranean fever: a case series of 6 patients. Joint Bone Spine. 2017;84(2):159–162.
   PubMed Web of Science ®Google Scholar
• van der Zee HH, Prens EP. Failure of anti-interleukin-1 therapy in severe hidradenitis suppurativa: a case report. Dermatology. 2013;226(2):97–100.
   PubMed Web of Science ®Google Scholar
• Zarchi K, Dufour DN, Jemec GB. Successful treatment of severe hidradenitis suppurativa with anakinra. JAMA Dermatol. 2013;149(10):1192–1194.
   PubMed Web of Science ®Google Scholar
• Russo V, Alikhan A. Failure of anakinra in a case of severe hidradenitis suppurativa. J Drugs Dermatol. 2016;15(6):772–774.
   PubMed Web of Science ®Google Scholar
• Bodar EJ, Simon A, de Visser M, et al. Complete remission of severe idiopathic cold urticaria on interleukin-1 receptor antagonist (anakinra). Neth J Med. 2009;67(9):302–305.
   PubMed Web of Science ®Google Scholar
• Sparsa L, Afif N, Goetz J, et al. Jessner-Kanof disease induced by leflunomide: a dermal variant of cutaneous lupus? Rheumatol Int. 2011;31(2):255–258.
   PubMed Web of Science ®Google Scholar
• Cohen PR, Kurzrock R. Anakinra-responsive lichen planus in a woman with Erdheim-Chester disease: a therapeutic enigma. Dermatol Online J. 2014;20(1):21241.
   PubMedGoogle Scholar
• Longhurst HJ, Yong PFK, Manson AL, et al. Mullins’ syndrome: a new gammopathy-related autoinflammatory syndrome resistant to anakinra. QJM. 2015;108(6):497–501.
   PubMed Web of Science ®Google Scholar
• Aouba A, Leclerc-Mercier S, Fraitag S, et al. Assessment and effective targeting of Interleukin-1 in multicentric reticulohistyocytosis. Joint Bone Spine. 2015;82(4):280–283.
   PubMed Web of Science ®Google Scholar
• Lipsker D, Perrigouard C, Foubert A, et al. Anakinra for difficult-to-treat neutrophilic panniculitis: IL-1 blockade as a promising treatment option for neutrophil-mediated inflammatory skin disease. Dermatology. 2010;220(3):264–267.
   PubMed Web of Science ®Google Scholar
• Belani H, Gensler L, Bajpai U, et al. Neutrophilic urticaria with systemic inflammation: a case series. JAMA Dermatol. 2013;149(4):453–458.
   PubMed Web of Science ®Google Scholar
• Tauber M, Viguier M, Alimova E, et al. Partial clinical response to anakinra in severe palmoplantar pustular psoriasis. Br J Dermatol. 2014;171(3):646–649.
   PubMed Web of Science ®Google Scholar
• Demidowich AP, Freeman AF, Kuhns DB, et al. Brief report: genotype, phenotype, and clinical course in five patients with PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne). Arthritis Rheum. 2012;64(6):2022–2027.
   PubMed Web of Science ®Google Scholar
• Stichweh DS, Punaro M, Pascual V. Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome. Pediatr Dermatol. 2005;22(3):262–265.
   PubMed Web of Science ®Google Scholar
• Brenner M, Ruzicka T, Plewig G, et al. Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra. Br J Dermatol. 2009;161(5):1199–1201.
   PubMed Web of Science ®Google Scholar
• Marzano AV, Trevisan V, Gattorno M, et al. Pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH): a new autoinflammatory syndrome associated with a novel mutation of the PSTPIP1 gene. JAMA Dermatol. 2013;149(6):762–764.
   PubMed Web of Science ®Google Scholar
• Braun-Falco M, Kovnerystyy O, Lohse P, et al. Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH)–a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol. 2012;66(3):409–415.
   PubMed Web of Science ®Google Scholar
• Jennings L, Molloy O, Quinlan C, et al. Treatment of pyoderma gangrenosum, acne, suppurative hidradenitis (PASH) with weight-based anakinra dosing in a Hepatitis B carrier. Int J Dermatol. 2017;56(6):e128–e129.
   PubMed Web of Science ®Google Scholar
• Leuenberger M, Berner J, Di Lucca J, et al. PASS Syndrome: an IL-1-driven autoinflammatory disease. Dermatology. 2016;232(2):254–258.
   PubMed Web of Science ®Google Scholar
• Herberger K, Dissemond J, Brüggestrat S, et al. Biologics and immunoglobulins in the treatment of pyoderma gangrenosum – analysis of 52 patients. J Dtsch Dermatol Ges. 2019;17(1):32–41.
   Web of Science ®Google Scholar
• Beynon C, Chin MF, Hunasehally P, et al. Successful treatment of autoimmune disease-associated pyoderma gangrenosum with the IL-1 receptor antagonist anakinra: a case series of 3 patients. J Clin Rheumatol. 2017;23(3):181–183.
   PubMed Web of Science ®Google Scholar
• Hsiao JL, Antaya RJ, Berger T, et al. Hidradenitis suppurativa and concomitant pyoderma gangrenosum: a case series and literature review. Arch Dermatol. 2010;146(11):1265–1270.
   PubMedGoogle Scholar
• McAllister BP, Williams ED, Yoo LJ, et al. Refractory peristomal pyoderma gangrenosum successfully treated with intravenous immunoglobulin: a case report. Am J Gastroenterol. 2015;110(12):1739–1740.
   PubMed Web of Science ®Google Scholar
• Etzler C, Cannon S, Hyde P. Refractory pyoderma gangrenosum in an infant. Pediatr Dermatol. 2015;32(1):156–157.
   PubMed Web of Science ®Google Scholar
• Lin Z, et al. Failure of anakinra treatment of pyoderma gangrenosum in an IBD patient and relevance to the PSTPIP1 gene. Inflamm Bowel Dis. 2011;17(6):E41–2.
   PubMed Web of Science ®Google Scholar
• Acquitter M, Plantin P, Kupfer I, et al. Anakinra improves pyoderma gangrenosum in psoriatic arthritis: a case report. Ann Intern Med. 2015;163(1):70–71.
   PubMed Web of Science ®Google Scholar
• Mercuri SR, Paolino G, De Flammineis E, et al. Successful treatment of pyoderma gangrenosum with anakinra in a patient with Wiskott-Aldrich syndrome. Dermatol Ther. 2018;31(2):e12582.
   PubMed Web of Science ®Google Scholar
• Zeeli T, Padalon-Brauch G, Ellenbogen E, et al. Pyoderma gangrenosum, acne and ulcerative colitis in a patient with a novel mutation in the PSTPIP1 gene. Clin Exp Dermatol. 2015;40(4):367–372.
   PubMed Web of Science ®Google Scholar
• Colina M, Pizzirani C, Khodeir M, et al. Dysregulation of P2X7 receptor-inflammasome axis in SAPHO syndrome: successful treatment with anakinra. Rheumatology (Oxford). 2010;49(7):1416–1418.
   PubMed Web of Science ®Google Scholar
• Shahid Z, Kalayanamitra R, Patel R, et al. Refractory sweet syndrome treated with anakinra. Cureus. 2019;11(4):e4536.
   PubMed Web of Science ®Google Scholar
• Kluger N, Gil-Bistes D, Guillot B, et al. Efficacy of anti-interleukin-1 receptor antagonist anakinra (Kineret(R)) in a case of refractory Sweet’s syndrome. Dermatology. 2011;222(2):123–127.
   PubMed Web of Science ®Google Scholar
• Lipof JS, Beck LA, Reddy SC, et al. Necrotizing sweet syndrome of the upper extremity after elective hand surgery. J Hand Surg Am. 2018;43(4):389.e1–389.e6.
   Web of Science ®Google Scholar
• Botsios C, Sfriso P, Punzi L, et al. Non-complementaemic urticarial vasculitis: successful treatment with the IL-1 receptor antagonist, anakinra. Scand J Rheumatol. 2007;36(3):236–237.
   PubMed Web of Science ®Google Scholar
• Szturz P, Adam Z, Řehák Z, et al. Xanthelasma palpebrarum responding to interleukin-1 blockade. Intern Med J. 2014;44(6):617–618.
   PubMed Web of Science ®Google Scholar
• ClinicalTrials.gov. Kineret CAPS post authorisation study. Bethesda (MD): National Library of Medicine; 2014.
   Google Scholar
• ClinicalTrials.gov. The use of kineret (Anakinra) in the treatment of familial cold urticaria. Bethesda (MD): National Library of Medicine; 2005.
   Google Scholar
• ClinicalTrials.gov. Anakinra for inflammatory pustular skin diseases. Bethesda (MD): National Library of Medicine; 2013.
   Google Scholar
• ClinicalTrials.gov. Schnitzler syndrome: clinical study, physiopathological and search for genetic factors. Bethesda (MD): National Library of Medicine; 2009.
   Google Scholar
• Dhimolea E. Canakinumab. mAbs. 2010;2(1):3–13.
   PubMed Web of Science ®Google Scholar