Current Dermatology Reports

, Volume 7, Issue 3, pp 190–197 | Cite as

Urticaria, Urticarial Vasculitis, Angioedema, and Related Diseases

  • Mary Anderson
  • Tina Chu
  • Melissa M. Mauskar
Hospital- based Dermatology (D Kroshinsky, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Hospital-based Dermatology


Purpose of Review

Dermatologists are frequently called to evaluate patients with urticaria in the emergency room, urgent care clinics, and hospitals. Many acute urticarial eruptions will resolve without long-term sequelae; however, there are red flags that clinicians must be aware of.

Recent Findings

First-line treatment for acute urticaria is regular dose H1 antagonists; however, the dose can be increased up to fourfold for refractory disease. Short courses of corticosteroids should be avoided as rebound urticaria is common upon discontinuation. Urticarial vasculitis presents with persistent, atypical urticaria, burning, and residual lesions. The most common extra-cutaneous manifestation of urticarial vasculitis is musculoskeletal involvement. Schnitzler syndrome is a rare, severe condition but new evidence provides promise for use of biologic therapies.


Acute spontaneous urticarial eruptions are commonly encountered in hospitalized patients. This review provides readers with the tools needed to delineate benign eruptions from more concerning conditions.


Urticaria Urticarial vasculitis Angioedema Hypocomplementemic urticarial vasculitis Hypocomplementemic urticarial vasculitis syndrome 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    •• Zuberbier T, Aberer W, Asero R. The EAACI/GA2LEN/EDF/WAO guideline for the definition, classification, diagnosis and management of urticaria. The 2017 revision and Update Allergy 2018 Jan 15. Epub 2018. This guideline provides an internationally updated definition and classification of urticaria. It also outlines evidence and consensus-based diagnostic and therapeutic approaches for the different subtypes of urticaria. 48 delegates of 42 national and international societies participated in the conference to develop the new guideline. CrossRefPubMedCentralPubMedGoogle Scholar
  2. 2.
    Maurer M, Ortonne JP, Zuberbier T. Chronic urticaria: a patient survey on quality-of-life, treatment usage and doctor-patient relation. Allergy. 2009;64(4):581–8.CrossRefPubMedCentralPubMedGoogle Scholar
  3. 3.
    Kanani A, Schellenberg R, Warrington R. Urticaria and angioedema. Allergy Asthma Clin Immunol. 2011;7(Suppl 1):S9.CrossRefPubMedCentralPubMedGoogle Scholar
  4. 4.
    • Kaplan AP. Chronic spontaneous urticaria: pathogenesis and treatment considerations. Allergy Asthma Immunol Res. 2017;9(6):477–82. This study summarizes current concepts in the pathogenesis of chronic spontaneous urticaria. The authors also detail treatments for chronic spontaneous urticaria, including considerations for antihistamine-refractory patients and those unresponsive to omalizumab and cyclosporine. CrossRefPubMedCentralPubMedGoogle Scholar
  5. 5.
    Magerl M, Altrichter S, Borzova E, Giménez-Arnau A, Grattan CEH, Lawlor F, et al. The definition, diagnostic testing, and management of chronic inducible urticarias—the EAACI/GA(2) LEN/Edf/UNEV consensus recommendations 2016 update and revision. Allergy. 2016;71(6):780–802.CrossRefPubMedGoogle Scholar
  6. 6.
    Vonakis BM, Saini SS. New concepts in chronic urticaria. Curr Opin Immunol. 2008;20(6):709–16.CrossRefPubMedCentralPubMedGoogle Scholar
  7. 7.
    Cribier B. Urticaria and hepatitis. Clin Rev Allergy Immunol. 2006;30(1):25–9.CrossRefPubMedCentralPubMedGoogle Scholar
  8. 8.
    Mareri A, Adler SP, Nigro G. Herpesvirus-associated acute urticaria: an age matched case-control study. PLoS One. 2013;8(12):e85378.CrossRefPubMedCentralPubMedGoogle Scholar
  9. 9.
    Dreyfus DH. Serological evidence that activation of ubiquitous human herpesvirus-6 (HHV-6) plays a role in chronic idiopathic/spontaneous urticaria (CIU). Clin Exp Immunol. 2016;183(2):230–8.CrossRefPubMedCentralPubMedGoogle Scholar
  10. 10.
    Calado G, Loureiro G, Machado D, Tavares B, Ribeiro C, Pereira C, et al. Streptococcal tonsillitis as a cause of urticaria: tonsillitis and urticaria. Allergol Immunopathol (Madr). 2012;40(6):341–5.CrossRefGoogle Scholar
  11. 11.
    Minciullo PL, Cascio A, Barberi G, Gangemi S. Urticaria and bacterial infections. Allergy Asthma Proc. 2014;35(4):295–302.CrossRefPubMedCentralPubMedGoogle Scholar
  12. 12.
    Shipley D, Ormerod AD. Drug-induced urticaria. Recog Treat Am J Clin Dermatol. 2001;2(3):151–8.CrossRefGoogle Scholar
  13. 13.
    Pillans PI, Coulter DM, Black P. Angioedema and urticaria with angiotensin converting enzyme inhibitors. Eur J Clin Pharmacol. 1996;51(2):123–6.CrossRefPubMedCentralPubMedGoogle Scholar
  14. 14.
    Imoto S, Kawamura K, Tokumine Y, Araki N, Akita S, Nishimura C, et al. Acute non-hemolytic transfusion reactions and HLA class I antibody: advantages of solid phase assay compared with conventional complement-dependent assay. Transfus Med. 2010;20(2):95–103.CrossRefPubMedCentralPubMedGoogle Scholar
  15. 15.
    Stitt JM, Dreskin SC. Urticaria and autoimmunity: where are we now? Curr Allergy Asthma Rep. 2013;13(5):555–62.CrossRefPubMedCentralPubMedGoogle Scholar
  16. 16.
    Kim DH, Sung NH, Lee AY. Effect of levothyroxine treatment on clinical symptoms in hypothyroid patients with chronic urticaria and thyroid autoimmunity. Ann Dermatol. 2016;28(2):199–204.CrossRefPubMedCentralPubMedGoogle Scholar
  17. 17.
    • Kasumagic-Halilovic E, Beslic N, Ovcina-Kurtovic N. Thyroid autoimmunity in patients with chronic urticaria. Med Arch. 2017;71(1):29–31. The etiopathogenesis of chronic urticaria is unclear. This study shows strong evidence for an association between chronic urticaria and thyroid autoimmunity. The authors found that the frequency of thyroid auto-antibodies (anti-Tg and anti-TPO) was significantly higher in those with chronic urticaria. CrossRefPubMedCentralPubMedGoogle Scholar
  18. 18.
    Wagner N, Dirk D, Peveling-Oberhag A, Reese I, Rady-Pizarro U, Mitzel H, et al. A popular myth—low-histamine diet improves spontaneous urticaria—fact or fiction? J Eur Acad Dermatol Venereol. 2017;31(4):650–5.CrossRefPubMedCentralPubMedGoogle Scholar
  19. 19.
    Staevska M, Gugutkova M, Lazarova C, Kralimarkova T, Dimitrov V, Zuberbier T, et al. Night-time sedating H1-antihistamine increases daytime somnolence but not treatment efficacy in chronic spontaneous urticaria: a randomized controlled trial. Br J Dermatol. 2014;171(1):148–54.CrossRefPubMedCentralPubMedGoogle Scholar
  20. 20.
    Guevara-Gutierrez E, Bonilla-Lopez S, Hernandez-Arana S, Tiacuilo-Parra A. Safety and efficacy of cetirizine versus cetirizine plus ranitidine in chronic urticaria: double-blind randomized placebo-controlled study. J Dermatolog Treat. 2015;26(6):548–50.CrossRefPubMedCentralPubMedGoogle Scholar
  21. 21.
    Martinez C, Albet C, Agundez JA, et al. Comparative in vitro and in vivo inhibition of cytochrome P450 CYP1A2, CYP2D6, and CYP3A by H2-receptor antagonists. Clin Pharmacol Ther. 1999;65(4):369–76.CrossRefPubMedCentralPubMedGoogle Scholar
  22. 22.
    Kaplan AP. Treatment of chronic spontaneous urticaria. Allergy Asthma Immunol Res. 2012;4(6):326–31.CrossRefPubMedCentralPubMedGoogle Scholar
  23. 23.
    Maurer M, Rosen K, Hsieh HJ, et al. Omalizumab for the treatment of chronic idiopathic or spontaneous urticaria. N Engl J Med. 2013;368(10):924–35.CrossRefPubMedGoogle Scholar
  24. 24.
    Kaplan A, Ferrer M, Bernstein JA, Antonova E, Trzaskoma B, Raimundo K, et al. Timing and duration of omalizumab response in patients with chronic idiopathic/spontaneous urticaria. J Allergy Clin Immunol. 2016;137(2):474–81.CrossRefPubMedGoogle Scholar
  25. 25.
    Saini SS, Bindslev-Jensen C, Maurer M, Grob JJ, Bülbül Baskan E, Bradley MS, et al. Efficacy and safety of omalizumab in patients with chronic idiopathic/spontaneous urticaria who remain symptomatic on H1 antihistamines: a randomized, placebo-controlled study. J Invest Dermatol. 2015;135(3):925.CrossRefPubMedCentralPubMedGoogle Scholar
  26. 26.
    Asero R. ACE inhibitors may interfere with omalizumab in chronic spontaneous urticaria. J Eur Acad Dermatol Venereol. 2017;31(8):e358–9.CrossRefPubMedCentralPubMedGoogle Scholar
  27. 27.
    Hong S, Armogida S. Urticaria and angioedema. Cleveland Clinic Center for Continuing Education: Disease Management. Accessed May 13, 2018.
  28. 28.
    Stanaland BE. Treatment of patients with chronic idiopathic urticaria. Clin Rev Allergy Immunol. 2002;23(2):233–41.CrossRefPubMedCentralPubMedGoogle Scholar
  29. 29.
    Engler RJ, Squire E, Benson P. Chronic sulfasalazine therapy in the treatment of delayed pressure urticaria and angioedema. Ann Allergy Asthma Immunol. 1995;74(2):155–9.PubMedPubMedCentralGoogle Scholar
  30. 30.
    McGirt LY, Vasagar K, Gober LM, Saini SS, Beck LA. Successful treatment of recalcitrant chronic idiopathic urticaria with sulfasalazine. Arch Dermatol. 2006;142(10):1337–42.CrossRefPubMedCentralPubMedGoogle Scholar
  31. 31.
    Orden RA, Timble H, Saini SS. Efficacy and safety of sulfasalazine in patients with chronic idiopathic urticaria. Ann Allergy Asthma Immunol. 2014;112(1):64–70.CrossRefPubMedCentralPubMedGoogle Scholar
  32. 32.
    Pho LN, Eliason MJ, Regruto M, Hull CM, Powell DL. Treatment of chronic urticaria with colchicine. J Drugs Dermatol. 2011;10(12):1423–8.PubMedGoogle Scholar
  33. 33.
    Lawlor F, Black AK, Ward AM, et al. Delayed pressure urticaria, objective evaluation of a variable disease using a dermographometer and assessment of treatment using colchicine. 1989 Br J Dermatol 1989; 120(3): 403–408.CrossRefPubMedCentralPubMedGoogle Scholar
  34. 34.
    Criado RF, Criado PR, Martins JE, et al. Urticaria unresponsive to antihistaminic treatment: an open study of therapeutic options based on histopathologic features. J Dermatol Treat. 2009;19(2):92–6.CrossRefGoogle Scholar
  35. 35.
    De Silva NL, Damayanthi H, Rajapaske AC, et al. Leukotriene receptor antagonists for chronic urticaria: a systematic review. Allergy Asthma Clin Immunol. 2014;10(1):24.CrossRefPubMedCentralPubMedGoogle Scholar
  36. 36.
    Dincy CVP, George R, Jacob M, Mathai E, Pulimood S, Eapen EP. Clinicopathologic profile of normocomplementemic and hypocomplementemic urticarial vasculitis: a study from South India. J Eur Acad Dermatol Venerol. 2008;22(7):789–94.CrossRefGoogle Scholar
  37. 37.
    Suh KS, Kang DY, Lee KH, Han SH, Park JB, Kim ST, et al. Evolution of urticarial vasculitis: a clinical, dermoscopic and histopathological study. J Eur Acad Dermatol Venereol. 2014;28(5):674–5.CrossRefPubMedCentralPubMedGoogle Scholar
  38. 38.
    Hamad A, Jithpratuck W, Krishnaswamy G. Urticarial vasculitis and associated disorders. Ann Allergy Asthma Immunol. 2017;118(4):394–8.CrossRefPubMedCentralPubMedGoogle Scholar
  39. 39.
    Loricera J, Calvo-Rio V, Mata C, et al. Urticarial vasculitis in northern Spain: clinical study of 21 cases. Medicine (Baltimore). 2014;93(1):53–60.CrossRefGoogle Scholar
  40. 40.
    Schwartz HR, McDuffle FC, Black LF, et al. Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease. Mayo Clin Proc. 1982;57(4):231–8.PubMedPubMedCentralGoogle Scholar
  41. 41.
    Jachiet M, Flageul B, Deroux A, le Quellec A, Maurier F, Cordoliani F, et al. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients. Arthritis Rheumatol. 2015;67(2):527–34.CrossRefPubMedGoogle Scholar
  42. 42.
    Tanaka M, Moniwa N, Mita T, Tobisawa T, Matsumoto T, Mochizuki A, et al. A case of cresentic glomerulonephritis complicated with hypocomplementemic urticarial vasculitis syndrome and ANCA-associated vasculitis. Case Rep Nephrol Dial. 2017;7(3):144–53.CrossRefPubMedCentralPubMedGoogle Scholar
  43. 43.
    Park C, Choi SW, Kim M, et al. Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report. J Med Case Rep. 2014;8:352.CrossRefPubMedCentralPubMedGoogle Scholar
  44. 44.
    Salim SA, Yousuf T, Patel A, et al. Hypocomplementemic urticarial vasculitis syndrome with crescentic glomerulonephritis. Am J Med Sci. 2018;55(2):195–200.CrossRefGoogle Scholar
  45. 45.
    Wisnieski JJ, Baer AN, Christensen J, Cupps TR, Flagg DN, Verrier Jones J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore). 1995;74(1):24–41.CrossRefGoogle Scholar
  46. 46.
    Grotz W, Baba HA, Becker JU, Baumgartel MW. Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge. Dtsch Arztebl Int. 2009;106(46):756–63.PubMedPubMedCentralGoogle Scholar
  47. 47.
    Filosto M, Cavallaro T, Pasolini G, Broglio L, Tentorio M, Cotelli M, et al. Idiopathic hypocomplementemic urticarial vasculitis-linked nephropathy. J Neurol Sci. 2009;284(1–2):179–81.CrossRefPubMedCentralPubMedGoogle Scholar
  48. 48.
    Jara LJ, Navarro C, Medina G, Vera-Lastra O, Saavedra MA. Hypocomplementemic urticarial vasculitis syndrome. Curr Rheumatol Rep. 2009;11(6):410–5.CrossRefPubMedCentralPubMedGoogle Scholar
  49. 49.
    Raoufi M, Laine M, Amrani HN, et al. Severe pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV). Pan Afr Med J. 2016;24:285.CrossRefPubMedCentralPubMedGoogle Scholar
  50. 50.
    Sunderkotter CH, Zelger B, Chen KR, et al. Nomenclature of cutaneous vasculitis: dermatologic addendum to the 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheumatol. 2018;70(2):171–84.CrossRefPubMedCentralPubMedGoogle Scholar
  51. 51.
    Babajanians A, Chung-Park M, Wisnieski JJ. Recurrent pericarditis and cardiac tamponade in a patient with hypocomplementemic urticarial vasculitis syndrome. J Rheumatol. 1991;18(5):72–5.Google Scholar
  52. 52.
    Jones RR, Eady RA. Endothelial cell pathology as a marker for urticarial vasculitis: a light microscopic study. Br J Dermatol. 1984;110(2):139–49.CrossRefPubMedCentralPubMedGoogle Scholar
  53. 53.
    Mehregan DR, Gibson LE. Pathophysiology of urticarial vasculitis. Arch Dermatol. 1998;134(1):88–9.CrossRefPubMedCentralPubMedGoogle Scholar
  54. 54.
    Hamid S, Cruz PD Jr, Lee WM. Urticarial vasculitis caused by hepatitis C virus infection: response to interferon alfa therapy. J Am Acad Dermatol. 1998;39(2 Pt 1):278–80.CrossRefPubMedCentralPubMedGoogle Scholar
  55. 55.
    Mahajan VK, Singh R, Gupta M, Raina R. Telmisartan induced urticarial vasculitis. Indian J Pharmacol. 2015;47(5):560–2.CrossRefPubMedCentralPubMedGoogle Scholar
  56. 56.
    Fadahunsi AW, Garcia-Rosell M, Pattanaik D. Hypocomplementemic urticarial vasculitis syndrome possibly secondary to etanercept use. J Clin Rheumatol. 2015;21(5):274–5.CrossRefPubMedCentralPubMedGoogle Scholar
  57. 57.
    Koregol S, Naidu V, Rao S, Ankad BS. Enalapril induced normocomplementemic urticarial vasculitis. Indian J Dermatol Venereol Leprol. 2015;81(1):73–4.CrossRefPubMedCentralPubMedGoogle Scholar
  58. 58.
    Bock V, Friedlander M, Waring D, et al. Cutaneous adverse effects of hormonal adjuvant therapy for breast cancer: a case of localized urticarial vasculitis following anastrozole therapy and review of the literature. Australas J Dermatol. 2014;55(4):282–5.CrossRefPubMedCentralPubMedGoogle Scholar
  59. 59.
    Ozcakar ZB, Foster J 2nd, Diaz-Horta O, et al. DNASE1L3 mutations in hypocomplementemic urticarial vasculitis syndrome. Arthritis Rheum. 2013;65(8):2183–9.CrossRefPubMedCentralPubMedGoogle Scholar
  60. 60.
    Carbonella A, Mancano G, Gremese E, Alkuraya FS, Patel N, Gurrieri F, et al. An autosomal recessive DNASE1L3-related autoimmune disease with unusual clinical presentation mimicking systemic lupus erythematous. Lupus. 2017;26(7):768–72.CrossRefPubMedCentralPubMedGoogle Scholar
  61. 61.
    Alberti-Violetti S, Berti E, Marzano AV. Cutaneous and systemic vasculitides in dermatology: a histologic perspective. G Ital Dermatol Venereol. 2018;153(2):185–93.PubMedPubMedCentralGoogle Scholar
  62. 62.
    Aboobaker J, Greaves MW. Urticarial vasculiltis. Clin Exp Dermatol. 1986;11(5):436–44.CrossRefPubMedCentralPubMedGoogle Scholar
  63. 63.
    Alexander JL, Kalaaji AN, Shehan JM, Yokel BK, Pittelkow MR. Plasmapheresis for refractory urticarial vasculitis in a patient with B-cell chronic lymphocytic leukemia. J Drugs Dermatol. 2006;5(6):534–7.PubMedPubMedCentralGoogle Scholar
  64. 64.
    Kartai O, Gulec M, Caliskaner Z, Al e. Plasmapheresis in a patient with “refractory urticarial vasculitis”. Allergy Asthma Immunol Res. 2012;4(4):245–7.CrossRefGoogle Scholar
  65. 65.
    Staubach-Renz P, von Stebut E, Brauninger W, et al. Hypocomplementemic urticarial vasculitis syndrome. Successful therapy with intravenous immunoglobulins. Hautarzt. 2007;58(8):693–7.CrossRefPubMedCentralPubMedGoogle Scholar
  66. 66.
    Aurich S, Simon JC, Treudler R. Omalizumab does not improve skin lesions in a patient with hypocomplementemic urticarial vasculitis syndrome. J Eur Acad Dermatol Venereol. 2017;31(9):e395–7.CrossRefPubMedCentralPubMedGoogle Scholar
  67. 67.
    Fueyo-Casado A, Campos-Munoz L, Gonzalez-Guerra E, et al. Effectiveness of omalizumab in a case of urticarial vasculitis. Clin Exp Dermatol. 2017;42:403–5. Scholar
  68. 68.
    Ghazanfar MN, Thomsen SF. Omalizumab for urticarial vasculitis: case report and review of the literature. Case Rep Dermatol Med. 2015;2015:576893.PubMedPubMedCentralGoogle Scholar
  69. 69.
    Soubrier M. Schnitzler syndrome. Joint Bone Spine. 2008;75(3):263–6.CrossRefPubMedCentralPubMedGoogle Scholar
  70. 70.
    Gameiro A, Gouveia M, Pereira M, Tellechea O, Gonçalo M. Clinical characterization and long-term follow-up of Schnitzler syndrome. Clin Exp Dermatol. 2016;41(5):461–7.CrossRefPubMedCentralPubMedGoogle Scholar
  71. 71.
    Neel A, Henry B, Barbarot S, 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):1035–41.CrossRefPubMedCentralPubMedGoogle Scholar
  72. 72.
    Rowczenio DM, Pathak S, Arostegui JI, Mensa-Vilaro A, Omoyinmi E, Brogan P, et al. Molecular genetic investigation, clinical features, and response to treatment in 21 patients with Schnitzler syndrome. Blood. 2018;131(9):974–81.CrossRefPubMedCentralPubMedGoogle Scholar
  73. 73.
    Bonnekoh H, Scheffel J, Maurer M, Krause K. Use of skin biomarker profiles to distinguish Schnitzler syndrome from chronic spontaneous urticaria: results of a pilot study. Br J Dermatol. 2018;178(2):561–2.CrossRefPubMedCentralPubMedGoogle Scholar
  74. 74.
    Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome: the Mayo Clinic experience (1972-2011). J Am Acad Dermatol. 2012;67(6):1289–95.CrossRefPubMedCentralPubMedGoogle Scholar
  75. 75.
    Gusdorf L, Asli B, Barbarot S, Néel A, Masseau A, Puéchal X, et al. Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients. Allergy. 2017;72(2):177–82.CrossRefPubMedCentralPubMedGoogle Scholar
  76. 76.
    Urbanski M, Holfield K, Milne A, Abbas M. Schnitzler syndrome without a monocolonal gammopathy: a case report. J Cutan Med Surg. 2016;20(6):575–8.CrossRefPubMedCentralPubMedGoogle Scholar
  77. 77.
    Ahn MJ, Yu JE, Jeong J, Sim DW, Koh YI. A case of Schnitzler’s syndrome without monoclonal gammopathy-associated chronic urticaria treated with anakinra. Yonsei Med J. 2018;59(1):154–7.CrossRefPubMedCentralPubMedGoogle Scholar
  78. 78.
    Simon A, Asli B, Braun-Falco M, de Koning H, Fermand JP, Grattan C, et al. Schnitzler’s syndrome: diagnosis, treatment and follow-up. Allergy. 2013;68(5):562–8.CrossRefPubMedCentralPubMedGoogle Scholar
  79. 79.
    Crouch R, Akhras V, Sarkany R. Schnitzler’s syndrome: successful treatment with anakinra. Australas J Dermatol. 2007;48(3):178–81.CrossRefPubMedCentralPubMedGoogle Scholar
  80. 80.
    Vandenhende MA, Bentaberry F, Moriat P, Bonnet F. Anakinra: an effective treatment in Schnitzler syndrome. Joint Bone Spine. 2011;78(6):636–7. Scholar
  81. 81.
    Krause K, Weller K, Stefaniak R, Wittkowski H, Altrichter S, Siebenhaar F, et al. Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: an open-label study. Allergy. 2012;67(7):943–50.CrossRefPubMedCentralPubMedGoogle Scholar
  82. 82.
    Krause K, Tsianakas A, Wagner N, Fischer J, Weller K, Metz M, et al. Efficacy and safety of canakinumab in Schnitzler syndrome: a multicenter randomized placebo-controlled study. J Allergy Clin Immunol. 2017;139(4):1311–20.CrossRefPubMedCentralPubMedGoogle Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of DermatologyUT Southwestern Medical CenterDallasUSA

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