Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Urticarial vasculitis

INTRODUCTION

Urticarial vasculitis (UV) is considered a clinicopathologic entity consisting of two elements:

Clinical manifestations of urticaria

Histopathological evidence of cutaneous leukocytoclastic vasculitis (LCV) of the small vessels, largely involving the postcapillary venules [1-4]

UV may predominantly involve the skin or affect other organ systems [5]. Hypocomplementemia, when present, may be associated with extensive vasculitis and systemic features that most commonly involve the musculoskeletal, pulmonary, renal, and/or gastrointestinal systems. Although UV is most commonly idiopathic, it can occur in association with autoimmune diseases, drug reactions, infections, or malignancy. Discussion of UV is confounded by the lack of accepted criteria for distinguishing UV from other cutaneous vasculitides and associated conditions.

The epidemiology, clinical features, laboratory and biopsy findings, differential diagnosis, treatment, and prognosis of UV are reviewed here. A more general discussion of urticaria and related conditions is presented elsewhere. (See "Chronic urticaria: Clinical manifestations, diagnosis, pathogenesis, and natural history".)

                                                

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2014. | This topic last updated: Jun 27, 2014.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  1. McDuffie FC, Sams WM Jr, Maldonado JE, et al. Hypocomplementemia with cutaneous vasculitis and arthritis. Possible immune complex syndrome. Mayo Clin Proc 1973; 48:340.
  2. Soter NA. Chronic urticaria as a manifestation of necrotizing venulitis. N Engl J Med 1977; 296:1440.
  3. Warin RP. Urticarial vasculitis. Br Med J (Clin Res Ed) 1983; 286:1919.
  4. Huston DP, Bressler RB. Urticaria and angioedema. Med Clin North Am 1992; 76:805.
  5. Wisnieski JJ. Urticarial vasculitis. Curr Opin Rheumatol 2000; 12:24.
  6. Davis MD, Brewer JD. Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am 2004; 24:183.
  7. Zeiss CR, Burch FX, Marder RJ, et al. A hypocomplementemic vasculitic urticarial syndrome. Report of four new cases and definition of the disease. Am J Med 1980; 68:867.
  8. Chen HJ, Bloch KJ. Hypocomplementemic urticarial vasculitis, jaccoud's arthropathy, valvular heart disease, and reversible tracheal stenosis: a surfeit of syndromes. J Rheumatol 2001; 28:383.
  9. Agnello V, Ruddy S, Winchester RJ, et al. Hereditary C2 deficiency in systemic lupus erythematosus and acquired complement abnormalities in an unusual SLE-related syndrome. Birth Defects Orig Artic Ser 1975; 11:312.
  10. Oishi M, Takano M, Miyachi K, et al. A case of unusual SLE related syndrome characterized by erythema multiforme, angioneurotic edema, marked hypocomplementemia, and Clq precipitins of the low molecular weight type. Int Arch Allergy Appl Immunol 1976; 50:463.
  11. Agnello V, Koffler D, Eisenberg JW, et al. C1g precipitins in the sera of patients with systemic lupus erythematosus and other hypocomplementemic states: characterization of high and low molecular weight types. J Exp Med 1971; 134:228s.
  12. Schwartz HR, McDuffie FC, Black LF, et al. Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease. Mayo Clin Proc 1982; 57:231.
  13. Sissons JG, Peters DK, Williams DG, et al. Skin lesions, angio-oedema, and hypocomplementaemia. Lancet 1974; 2:1350.
  14. Feig PU, Soter NA, Yager HM, et al. Vasculitis with urticaria, hypocomplementemia, and multiple system involvement. JAMA 1976; 236:2065.
  15. Schultz DR, Perez GO, Volanakis JE, et al. Glomerular disease in two patients with urticaria-cutaneous vasculitis and hypocomplementemia. Am J Kidney Dis 1981; 1:157.
  16. Wisnieski JJ, Jones SM. IgG autoantibody to the collagen-like region of Clq in hypocomplementemic urticarial vasculitis syndrome, systemic lupus erythematosus, and 6 other musculoskeletal or rheumatic diseases. J Rheumatol 1992; 19:884.
  17. Kohro-Kawata J, Wener MH, Mannik M. The effect of high salt concentration on detection of serum immune complexes and autoantibodies to C1q in patients with systemic lupus erythematosus. J Rheumatol 2002; 29:84.
  18. Black AK. Urticarial vasculitis. Clin Dermatol 1999; 17:565.
  19. Berg RE, Kantor GR, Bergfeld WF. Urticarial vasculitis. Int J Dermatol 1988; 27:468.
  20. Jones RR, Bhogal B, Dash A, Schifferli J. Urticaria and vasculitis: a continuum of histological and immunopathological changes. Br J Dermatol 1983; 108:695.
  21. Venzor J, Lee WL, Huston DP. Urticarial vasculitis. Clin Rev Allergy Immunol 2002; 23:201.
  22. Dienstag JL, Rhodes AR, Bhan AK, et al. Urticaria associated with acute viral hepatitis type B: studies of pathogenesis. Ann Intern Med 1978; 89:34.
  23. Cicek D, Kandi B, Oguz S, et al. An urticarial vasculitis case induced by glatiramer acetate. J Dermatolog Treat 2008; 19:305.
  24. Goulão J, Cunha H, Anes I, et al. Urticarial vasculitis due do infliximab. J Eur Acad Dermatol Venereol 2008; 22:882.
  25. Alijotas-Reig J. Recurrent urticarial vasculitis related to nonanimal hyaluronic acid skin filler injection. Dermatol Surg 2009; 35 Suppl 1:395.
  26. Davies KA, Norsworthy PJ, Athanassiou P, Walport MJ. Anti-C1q antibodies activate complement and may be pathogenic in SLE. Arthritis Rheum 1997; 40(suppl):S308.
  27. Wisnieski JJ, Naff GB. Serum IgG antibodies to C1q in hypocomplementemic urticarial vasculitis syndrome. Arthritis Rheum 1989; 32:1119.
  28. Wisnieski JJ, Jones SM. Comparison of autoantibodies to the collagen-like region of C1q in hypocomplementemic urticarial vasculitis syndrome and systemic lupus erythematosus. J Immunol 1992; 148:1396.
  29. Botsios C, Sfriso P, Punzi L, Todesco S. Non-complementaemic urticarial vasculitis: successful treatment with the IL-1 receptor antagonist, anakinra. Scand J Rheumatol 2007; 36:236.
  30. Krause K, Mahamed A, Weller K, et al. Efficacy and safety of canakinumab in urticarial vasculitis: an open-label study. J Allergy Clin Immunol 2013; 132:751.
  31. Ozçakar ZB, Foster J 2nd, Diaz-Horta O, et al. DNASE1L3 mutations in hypocomplementemic urticarial vasculitis syndrome. Arthritis Rheum 2013; 65:2183.
  32. Monroe EW. Urticarial vasculitis: an updated review. J Am Acad Dermatol 1981; 5:88.
  33. WINKELMANN RK, DITTO WB. CUTANEOUS AND VISCERAL SYNDROMES OF NECROTIZING OR "ALLERGIC" ANGIITIS: A STUDY OF 38 CASES. Medicine (Baltimore) 1964; 43:59.
  34. Cox AJ. Pathologic changes in hypersensitivity angiitis. In: The Skin, Helwig EB, Mostofi FK (Eds), The Williams & Wilkins Co, Baltimore 1971. p.279.
  35. Davis MD, Daoud MS, Kirby B, et al. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol 1998; 38:899.
  36. Kao NL, Zeitz HJ. Urticarial skin lesions and polymyositis due to lymphocytic vasculitis. West J Med 1995; 162:156.
  37. Sanchez NP, Winkelmann RK, Schroeter AL, Dicken CH. The clinical and histopathologic spectrums of urticarial vasculitis: study of forty cases. J Am Acad Dermatol 1982; 7:599.
  38. Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol 1992; 26:441.
  39. Davis AE 3rd. C1 inhibitor and hereditary angioneurotic edema. Annu Rev Immunol 1988; 6:595.
  40. Mathison DA, Arroyave CM, Bhat KN, et al. Hypocomplementemia in chronic idiopathic urticaria. Ann Intern Med 1977; 86:534.
  41. Natbony SF, Phillips ME, Elias JM, et al. Histologic studies of chronic idiopathic urticaria. J Allergy Clin Immunol 1983; 71:177.
  42. Zuberbier T, Henz BM, Fiebiger E, et al. Anti-FcepsilonRIalpha serum autoantibodies in different subtypes of urticaria. Allergy 2000; 55:951.
  43. Champion RH. Urticaria: then and now. Br J Dermatol 1988; 119:427.
  44. Aboobaker J, Greaves MW. Urticarial vasculitis. Clin Exp Dermatol 1986; 11:436.
  45. Soylu A, Kavukçu S, Uzuner N, et al. Systemic lupus erythematosus presenting with normocomplementemic urticarial vasculitis in a 4-year-old girl. Pediatr Int 2001; 43:420.
  46. Dahl MV. Clinical pearl: diascopy helps diagnose urticarial vasculitis. J Am Acad Dermatol 1994; 30:481.
  47. Soter NA. Urticarial vasculitis. In: The Urticarias, Champion RH, Greaves MW, Kobza Black A, Pye RJ (Eds), Churchhill Livingstone, Edinburgh 1985. p.141.
  48. Gammon WR, Wheeler CE Jr. Urticarial vasculitis: report of a case and review of the literature. Arch Dermatol 1979; 115:76.
  49. O'Donnell B, Black AK. Urticarial vasculitis. Int Angiol 1995; 14:166.
  50. Sturgess AS, Littlejohn GO. Jaccoud's arthritis and panvasculitis in the hypocomplementemic urticarial vasculitis syndrome. J Rheumatol 1988; 15:858.
  51. Bywaters EGL. Jaccoud's syndrome: a sequel to the joint involvement of systemic lupus erythematosus. Clin Rheum Dis 1975; 1:125.
  52. Mizutani W, Quismorio FP Jr. Lupus foot: deforming arthropathy of the feet in systemic lupus erythematosus. J Rheumatol 1984; 11:80.
  53. Martini A, Ravelli A, Viola S, Burgio RG. Systemic lupus erythematosus with Jaccoud's arthropathy mimicking juvenile rheumatoid arthritis. Arthritis Rheum 1987; 30:1062.
  54. Manthorpe R, Bendixen G, Schiøler H, Viderbaek A. Jaccoud's syndrome. A nosographic entity associated with systemic lupus erythematosus. J Rheumatol 1980; 7:169.
  55. Ishikawa O, Miyachi Y, Watanabe H. Hypocomplementaemic urticarial vasculitis associated with Jaccoud's syndrome. Br J Dermatol 1997; 137:804.
  56. Houser SL, Askenase PW, Palazzo E, Bloch KJ. Valvular heart disease in patients with hypocomplementemic urticarial vasculitis syndrome associated with Jaccoud's arthropathy. Cardiovasc Pathol 2002; 11:210.
  57. Palazzo E, Bourgeois P, Meyer O, et al. Hypocomplementemic urticarial vasculitis syndrome, Jaccoud's syndrome, valvulopathy: a new syndromic combination. J Rheumatol 1993; 20:1236.
  58. Cadnapaphornchai MA, Saulsbury FT, Norwood VF. Hypocomplementemic urticarial vasculitis: report of a pediatric case. Pediatr Nephrol 2000; 14:328.
  59. Callen JP, Kalbfleisch S. Urticarial vasculitis: a report of nine cases and review of the literature. Br J Dermatol 1982; 107:87.
  60. Messiaen T, Van Damme B, Kuypers D, et al. Crescentic glomerulonephritis complicating the course of a hypocomplementemic urticarial vasculitis. Clin Nephrol 2000; 54:409.
  61. Kobayashi S, Nagase M, Hidaka S, et al. Membranous nephropathy associated with hypocomplementemic urticarial vasculitis: report of two cases and a review of the literature. Nephron 1994; 66:1.
  62. Renard M, Wouters C, Proesmans W. Rapidly progressive glomerulonephritis in a boy with hypocomplementaemic urticarial vasculitis. Eur J Pediatr 1998; 157:243.
  63. Falk DK. Pulmonary disease in idiopathic urticarial vasculitis. J Am Acad Dermatol 1984; 11:346.
  64. Jones MD, Tsou E, Lack E, Cupps TR. Pulmonary disease in systemic urticarial vasculitis: the role of bronchoalveolar lavage. Am J Med 1990; 88:431.
  65. Paira SO. Bilateral pleural effusion in a patient with urticarial vasculitis. Clin Rheumatol 1994; 13:504.
  66. Knobler H, Admon D, Leibovici V, Okon E. Urticarial vasculitis and recurrent pleural effusion: a systemic manifestation of urticarial vasculitis. Dermatologica 1986; 172:120.
  67. Gibson LE, Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am 1990; 16:309.
  68. Eiser AR, Singh P, Shanies HM. Sustained dapsone-induced remission of hypocomplementemic urticarial vasculitis--a case report. Angiology 1997; 48:1019.
  69. Babajanians A, Chung-Park M, Wisnieski JJ. Recurrent pericarditis and cardiac tamponade in a patient with hypocomplementemic urticarial vasculitis syndrome. J Rheumatol 1991; 18:752.
  70. Corwin JM, Baum J. Iridocyclitis in two patients with hypocomplementemic cutaneous vasculitis. Am J Ophthalmol 1982; 94:111.
  71. Bielory L, Noble KG, Frohman LP. Urticarial vasculitis and visual loss. J Allergy Clin Immunol 1991; 88:819.
  72. Hong L, Wackers F, Dewar M, et al. Atypical fatal hypocomplementemic urticarial vasculitis with involvement of native and homograft aortic valves in an African American man. J Allergy Clin Immunol 2000; 106:1196.
  73. Ludivico CL, Myers AR, Maurer K. Hypocomplementemic urticarial vasculitis with glomerulonephritis and pseudotumor cerebri. Arthritis Rheum 1979; 22:1024.
  74. Bonelli U, Guerra L, Bettoli V, Passarini B. Urticarial vasculitis with generalized pustular psoriasis and pseudotumor cerebri: a case report. Ann It Derm Clin Sper 1988; 42:171.
  75. Tanaka H, Waga S, Kakizaki Y, et al. Chronic urticaria associated with aseptic meningitis: an atypical urticarial vasculitis? Acta Paediatr Jpn 1997; 39:64.
  76. Koul PA, Wahid A, Shah SU, et al. Hypocomplementemic urticarial vasculitis and lower cranial nerve palsies. J Assoc Physicians India 2000; 48:536.
  77. Bolla G, Disdier P, Verrot D, et al. Acute transverse myelitis and primary urticarial vasculitis. Clin Rheumatol 1998; 17:250.
  78. GOLTZ RW. Cutaneous manifestations of allergic angiitis. J Lancet 1962; 82:218.
  79. Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore) 1995; 74:24.
  80. RUITER M, HADDERS HN. Predominantly cutaneous forms of necrotising angiitis. J Pathol Bacteriol 1959; 77:71.
  81. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25:1271.
  82. Jara LJ, Navarro C, Medina G, et al. Hypocomplementemic urticarial vasculitis syndrome. Curr Rheumatol Rep 2009; 11:410.
  83. Bisaccia E, Adamo V, Rozan SW. Urticarial vasculitis progressing to systemic lupus erythematosus. Arch Dermatol 1988; 124:1088.
  84. Kulthanan K, Cheepsomsong M, Jiamton S. Urticarial vasculitis: etiologies and clinical course. Asian Pac J Allergy Immunol 2009; 27:95.
  85. Her MY, Song JY, Kim DY. Hypocomplementemic urticarial vasculitis in systemic lupus erythematosus. J Korean Med Sci 2009; 24:184.
  86. Asherson RA, D'Cruz D, Stephens CJ, et al. Urticarial vasculitis in a connective tissue disease clinic: patterns, presentations, and treatment. Semin Arthritis Rheum 1991; 20:285.
  87. Alexander EL, Arnett FC, Provost TT, Stevens MB. Sjögren's syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity. Ann Intern Med 1983; 98:155.
  88. Doyle MK, Cuellar ML. Drug-induced vasculitis. Expert Opin Drug Saf 2003; 2:401.
  89. Lin RY, Caren CB, Menikoff H. Hypocomplementaemic urticarial vasculitis, interstitial lung disease and hepatitis C. Br J Dermatol 1995; 132:821.
  90. Olson JC, Esterly NB. Urticarial vasculitis and Lyme disease. J Am Acad Dermatol 1990; 22:1114.
  91. Wands JR, Perrotto JL, Isselbacher KJ. Circulating immune complexes and complement sequence activation in infectious mononucleosis. Am J Med 1976; 60:269.
  92. Gammon WR. Manifestations of drug reactions. Urticaria and cutaneous necrotizing venulitis. Clin Dermatol 1986; 4:50.
  93. McLean RH, Weinstein A, Chapitis J, et al. Familial partial deficiency of the third component of complement (C3) and the hypocomplementemic cutaneous vasculitis syndrome. Am J Med 1980; 68:549.
  94. Glovsky MM, Braunwald J, Opelz G, Alenty A. Hypersensitivity to procarbazine associated with angioedema, urticaria, and low serum complement activity. J Allergy Clin Immunol 1976; 57:134.
  95. Borradori L, Rybojad M, Morel P, et al. Chronic urticaria and moderate leukocytoclastic vasculitis associated with C3 nephritic factor activity. Arch Dermatol 1989; 125:1589.
  96. Carmichael AJ, Marsden JR. Urticarial vasculitis: a presentation of C3 nephritic factor. Br J Dermatol 1993; 128:589.
  97. Strickland DK, Ware RE. Urticarial vasculitis: an autoimmune disorder following therapy for Hodgkin's disease. Med Pediatr Oncol 1995; 25:208.
  98. Highet AS. Urticarial vasculitis and IgA myeloma. Br J Dermatol 1980; 102:355.
  99. Lipsker D, Cribier B, Maloisel F, et al. Chronic urticaria and IgA myeloma. Acta Derm Venereol 1998; 78:395.
  100. Lewis JE. Urticarial vasculitis occurring in association with visceral malignancy. Acta Derm Venereol 1990; 70:345.
  101. Sprossmann A, Müller RP. [Urticaria-vasculitis syndrome in metastatic malignant testicular teratoma]. Hautarzt 1994; 45:871.
  102. Wilson D, McCluggage WG, Wright GD. Urticarial vasculitis: a paraneoplastic presentation of B-cell non-Hodgkin's lymphoma. Rheumatology (Oxford) 2002; 41:476.
  103. García-Porrúa C, González-Gay MA. Cutaneous vasculitis as a paraneoplastic syndrome in adults. Arthritis Rheum 1998; 41:1133.
  104. Asherson RA, Buchanan NM, d'Cruz D, Hughes GR. Urticarial vasculitis, IgA deficiency and C1 esterase inhibitor deficiency in the presence of an IgG monoclonal gammopathy--a case report. Clin Exp Dermatol 1992; 17:137.
  105. Farell AM, Sabroe RA, Bunker CB. Urticarial vasculitis associated with polycythaemia rubra vera. Clin Exp Dermatol 1996; 21:302.
  106. Matthews KP. Chronic urticaria. In: Clinical management of urticaria and anaphylaxis, Schocket AL (Ed), Marcel Dekker, Inc, New York 1993. p.21.
  107. Kwon CW, Lee CW, Kim YT, Kim JH. Urticarial vasculitis developed on the striae distensae during pregnancy. Int J Dermatol 1993; 32:751.
  108. Wanderer AA, Nuss DD, Tormey AD, Giclas PC. Urticarial leukocytoclastic vasculitis with cold urticaria. Report of a case and review of the literature. Arch Dermatol 1983; 119:145.
  109. Eady RA, Keahey TM, Sibbald RG, Kobza Black A. Cold urticaria with vasculitis: report of a case with light and electron microscopic, immunofluorescence and pharmacological studies. Clin Exp Dermatol 1981; 6:355.
  110. Demierre MF, Winkelman WJ. Idiopathic cold-induced urticarial vasculitis and monoclonal IgG gammopathy. Int J Dermatol 1996; 35:151.
  111. Armstrong RB, Horan DB, Silvers DN. Leukocytoclastic vasculitis in urticaria induced by ultraviolet irradiation. Arch Dermatol 1985; 121:1145.
  112. Massa MC, Su WP. Lymphocytic vasculitis: is it a specific clinicopathologic entity? J Cutan Pathol 1984; 11:132.
  113. Boonk WJ, Nieboer C, Huijgens PC. Pathogenetic studies in chronic urticaria. Failure to demonstrate vasculitis, complement activation and fibrinolysis. Dermatologica 1986; 173:264.
  114. Cicardi M, Zingale LC, Pappalardo E, et al. Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies. Medicine (Baltimore) 2003; 82:274.
  115. Lahiri M, Lim AY. Angioedema and systemic lupus erythematosus--a complementary association? Ann Acad Med Singapore 2007; 36:142.
  116. Nettis E, Colanardi MC, Loria MP, Vacca A. Acquired C1-inhibitor deficiency in a patient with systemic lupus erythematosus: a case report and review of the literature. Eur J Clin Invest 2005; 35:781.
  117. Aslam A, Misbah SA. Is acquired C1 inhibitor deficiency associated with lupus a distinct disease entity? Comment on the article by Cacoub et al. Arthritis Rheum 2002; 46:2827.
  118. Jazwinska EC, Gatenby PA, Dunckley H, Serjeantson SW. C1 inhibitor functional deficiency in systemic lupus erythematosus (SLE). Clin Exp Immunol 1993; 92:268.
  119. Ochonisky S, Intrator L, Wechsler J, et al. Acquired C1 inhibitor deficiency revealing systemic lupus erythematosus. Dermatology 1993; 186:261.
  120. Wozel G, Rietzschel I. [Acquired deficiency of C1 esterase inhibitor, persistent eyelid edema and subcutaneous lupus erythematosus]. Dermatol Monatsschr 1988; 174:741.
  121. Agnello V. Lupus diseases associated with hereditary and acquired deficiencies of complement. Springer Semin Immunopathol 1986; 9:161.
  122. Kieffer C, Cribier B, Lipsker D. Neutrophilic urticarial dermatosis: a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature. Medicine (Baltimore) 2009; 88:23.
  123. McNeil DJ, Kinsella TD, Crawford AM, Fritzler MJ. The AHA syndrome: arthritis, hives and angioedema. Rheumatol Int 1987; 7:277.
  124. de Koning HD, Bodar EJ, van der Meer JW, et al. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum 2007; 37:137.
  125. Janier M, Bonvalet D, Blanc MF, et al. Chronic urticaria and macroglobulinemia (Schnitzler's syndrome): report of two cases. J Am Acad Dermatol 1989; 20:206.
  126. Schnitzler L, Hurez D, Verret JL. [Chronic urticaria--osteo-condensation--macroglobulinemia. Principal case. Study of 20 cases]. Ann Dermatol Venereol 1989; 116:547.
  127. Morita A, Sakakibara S, Yokota M, Tsuji T. A case of urticarial vasculitis associated with macroglobulinemia (Schnitzler's syndrome). J Dermatol 1995; 22:32.
  128. Borradori L, Rybojad M, Puissant A, et al. Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler's syndrome. Br J Dermatol 1990; 123:113.
  129. de Koning HD, Bodar EJ, Simon A, et al. Beneficial response to anakinra and thalidomide in Schnitzler's syndrome. Ann Rheum Dis 2006; 65:542.
  130. Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome: the Mayo Clinic experience (1972-2011). J Am Acad Dermatol 2012; 67:1289.
  131. Jain T, Offord CP, Kyle RA, Dingli D. Schnitzler syndrome: an under-diagnosed clinical entity. Haematologica 2013; 98:1581.
  132. Dybowski F, Sepp N, Bergerhausen HJ, Braun J. Successful use of anakinra to treat refractory Schnitzler's syndrome. Clin Exp Rheumatol 2008; 26:354.
  133. 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; 23:633.
  134. 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; 29:567.
  135. de Koning HD, Schalkwijk J, van der Meer JW, Simon A. Successful canakinumab treatment identifies IL-1β as a pivotal mediator in Schnitzler syndrome. J Allergy Clin Immunol 2011; 128:1352.
  136. 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; 72:1634.
  137. Krause K, Feist E, Fiene M, et al. Complete remission in 3 of 3 anti-IL-6-treated patients with Schnitzler syndrome. J Allergy Clin Immunol 2012; 129:848.
  138. Asli B, Bienvenu B, Cordoliani F, et al. Chronic urticaria and monoclonal IgM gammopathy (Schnitzler syndrome): report of 11 cases treated with pefloxacin. Arch Dermatol 2007; 143:1046.
  139. Ozen S, Bilginer Y. A clinical guide to autoinflammatory diseases: familial Mediterranean fever and next-of-kin. Nat Rev Rheumatol 2014; 10:135.
  140. Hoffman HM, Mueller JL, Broide DH, et al. Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome. Nat Genet 2001; 29:301.
  141. Muramatsu C, Tanabe E. Urticarial vasculitis: response to dapsone and colchicine. J Am Acad Dermatol 1985; 13:1055.
  142. Matthews CN, Saihan EM, Warin RP. Urticaria-like lesions associated with systemic lupus erythematosus: response to dapsone. Br J Dermatol 1978; 99:455.
  143. Fortson JS, Zone JJ, Hammond ME, Groggel GC. Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone. J Am Acad Dermatol 1986; 15:1137.
  144. Highet AS. Urticarial vasculitis resembling systemic lupus erythematosus: efficacy of prednisone and dapsone combined. Br J Dermatol 1980; 102:358.
  145. Holtman JH, Neustadt DH, Klein J, Callen JP. Dapsone is an effective therapy for the skin lesions of subacute cutaneous lupus erythematosus and urticarial vasculitis in a patient with C2 deficiency. J Rheumatol 1990; 17:1222.
  146. Nürnberg W, Grabbe J, Czarnetzki BM. Urticarial vasculitis syndrome effectively treated with dapsone and pentoxifylline. Acta Derm Venereol 1995; 75:54.
  147. Leslie KS, Gaffney K, Ross CN, et al. A near fatal case of the dapsone hypersensitivity syndrome in a patient with urticarial vasculitis. Clin Exp Dermatol 2003; 28:496.
  148. Ash-Bernal R, Wise R, Wright SM. Acquired methemoglobinemia: a retrospective series of 138 cases at 2 teaching hospitals. Medicine (Baltimore) 2004; 83:265.
  149. Worm M, Sterry W, Kolde G. Mycophenolate mofetil is effective for maintenance therapy of hypocomplementaemic urticarial vasculitis. Br J Dermatol 2000; 143:1324.
  150. Stack PS. Methotrexate for urticarial vasculitis. Ann Allergy 1994; 72:36.
  151. Borcea A, Greaves MW. Methotrexate-induced exacerbation of urticarial vasculitis: an unusual adverse reaction. Br J Dermatol 2000; 143:203.
  152. Ormerod AD. Urticaria. Recognition, causes and treatment. Drugs 1994; 48:717.
  153. Moorthy AV, Pringle D. Urticaria, vasculitis, hypocomplementemia, and immune-complex glomerulonephritis. Arch Pathol Lab Med 1982; 106:68.
  154. Ramirez G, Saba SR, Espinoza L. Hypocomplementemic vasculitis and renal involvement. Nephron 1987; 45:147.
  155. Soma J, Sato H, Ito S, Saito T. Nephrotic syndrome associated with hypocomplementaemic urticarial vasculitis syndrome: successful treatment with cyclosporin A. Nephrol Dial Transplant 1999; 14:1753.
  156. Tejani AT, Butt K, Trachtman H, et al. Cyclosporine A induced remission of relapsing nephrotic syndrome in children. Kidney Int 1988; 33:729.
  157. Mukhtyar C, Misbah S, Wilkinson J, Wordsworth P. Refractory urticarial vasculitis responsive to anti-B-cell therapy. Br J Dermatol 2009; 160:470.
  158. Mallipeddi R, Grattan CE. Lack of response of severe steroid-dependent chronic urticaria to rituximab. Clin Exp Dermatol 2007; 32:333.
  159. 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; 39:278.
  160. Misiani R, Bellavita P, Fenili D, et al. Interferon alfa-2a therapy in cryoglobulinemia associated with hepatitis C virus. N Engl J Med 1994; 330:751.
  161. Matteson EL. Interferon alpha 2a therapy for urticarial vasculitis with angioedema apparently following hepatitis A infection. J Rheumatol 1996; 23:382.
  162. Worm M, Muche M, Schulze P, et al. Hypocomplementaemic urticarial vasculitis: successful treatment with cyclophosphamide-dexamethasone pulse therapy. Br J Dermatol 1998; 139:704.
  163. Asherson RA, Buchanan N, Kenwright S, et al. The normocomplementemic urticarial vasculitis syndrome--report of a case and response to colchicine. Clin Exp Dermatol 1991; 16:424.
  164. Berkman SA, Lee ML, Gale RP. Clinical uses of intravenous immunoglobulins. Ann Intern Med 1990; 112:278.
  165. Yamazaki-Nakashimada MA, Duran-McKinster C, Ramírez-Vargas N, Hernandez-Bautista V. Intravenous immunoglobulin therapy for hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus in a child. Pediatr Dermatol 2009; 26:445.
  166. Demitsu T, Yoneda K, Iida E, et al. Urticarial vasculitis with haemorrhagic vesicles successfully treated with reserpine. J Eur Acad Dermatol Venereol 2008; 22:1006.
  167. Epstein MM, Watsky KL, Lanzi RA. The role of diet in the treatment of a patient with urticaria and urticarial vasculitis. J Allergy Clin Immunol 1992; 90:414.