GUIDELINES IN DERMATOLOGY

gdkapak

VASCULITIS

Gizem Karataş1
Kenan Aydoğan2

1Erzurum Training and Research Hospital, Department of Dermatology and Venereology, Erzurum, Türkiye
2Uludağ University, Faculty of Medicine, Dermatology and Venereology, Bursa, Türkiye

Karataş G, Aydoğan K. Vasculitis. In: Kutlubay Z, editor. Guidelines in Dermatology. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.219-234.

ABSTRACT

Cutaneous vasculitis reflects inflammation of dermal blood vessels. Its symptoms vary depending on the location and size of the affected vessels and have a wide clinical spectrum. Cutaneous vasculitis can reflect a cutaneous component of a systemic vasculitis, a skin-limited or skin-dominant expression or variant of a systemic vasculitis, or be a single-organ vasculitis. Vasculitides are primarily divided according to the size of the affected vessel; small, small and medium (mixed), medium, large vessel vasculitis. The type of inflammatory infiltrate and accumulations of immunoglobulin, complement or fibrinogen in the vessel wall are a key finding in the diagnosis of cutaneous vasculitis. Lesions are multiple and polymorphic and they can provide some valuable information on the size of the affected vessels and the type of vasculitis. Involvement of small superficial vessels results mostly in urticarial, but relatively persistent plaques, papules, pustules and palpable purpura on the lower extremities, dependent areas, and under tight clothing. Involvement of vessels in the dermohypodermic junction or hypodermis results in ulcers, nodules, or livedo. Vasculitis is more of a reaction pattern rather than a specific disease entity. Therefore, the clinical presentation of vasculitis dictates a thorough history, review of systems, and a meticulous physical examination. The presence or absence of internal organ involvement and severity of disease guides therapy. Since most cases of isolated cutaneous vasculitis are self-limited and resolve spontaneously over 3 to 4 weeks, most patients require no systemic treatment. For those with severe, intractable, or chronic and recurring vasculitis, systemic therapy can be indicated and should be tailored to the severity of the disease. High-quality literature is lacking to guide management. Oral glucocorticoids may be required for a short period of time for painful, ulcerative, or otherwise severe disease in order to speed resolution. Among drugs which are reasonable longer-term options are colchicine, dapsone, azathioprine or hydroxychloroquine. The aim of our article is to provide an overview of elementary skin lesions and clinicopathologic in vasculitis.

Keywords: Vasculitis; Cutaneous vasculitis; Purpura; Livedo; Ulcer

Referanslar

  1. Sunderkötter CH, Zelger B, Chen KR, Requena L, Piette W, Carlson JA, et al. Nomenclature of Cutaneous Vasculitis: Dermatologic Addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheumatol. 2018 Feb;70(2):171-184. [Crossref]  [PubMed]
  2. Resnick AH, Esterly NB. Vasculitis in children. Int J Dermatol. 1985;24(3):139-146. [Crossref]  [PubMed]
  3. Frumholtz L, Laurent-Roussel S, Lipsker D, Terrier B. Cutaneous Vasculitis: Review on Diagnosis and Clinicopathologic Correlations. Clin Rev Allergy Immunol. 2021 Oct;61(2):181-193. [Crossref]  [PubMed]
  4. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. (2013) 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65(1):1-11 [PubMed]
  5. Fraticelli P, Benfaremo D, Gabrielli A. Diagnosis and management of leukocytoclastic vasculitis. Intern Emerg Med. 2021;16(4):831-841. [Crossref]  [PubMed]  [PMC]
  6. Micheletti RG. Cutaneous Small Vessel Vasculitis: A Practical Guide to Diagnosis and Management. Am J Clin Dermatol. 2023;24(1):89-95. [Crossref]  [PubMed]
  7. Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol. 1985;13(2 Pt 1):193-200. [Crossref]  [PubMed]
  8. Sais G, Vidaller A, Jucgla A, Gallardo F, Peyri J. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol 1995;131(12):1399-402. [Crossref]  [PubMed]
  9. Fredenberg MF, Malkinson FD. Sulfone therapy in the treatment of leukocytoclastic vasculitis. Report of three cases. J Am Acad Dermatol. 1987;16(4):772-8. [Crossref]  [PubMed]
  10. Callen JP, Spencer LV, Burruss JB, Holtman J. Azathioprine. An effective, corticosteroid-sparing therapy for patients with recalcitrant cutaneous lupus erythematosus or with recalcitrant cutaneous leukocytoclastic vasculitis. Arch Dermatol. 1991;127(4):515-22. [Crossref]  [PubMed]
  11. Haeberle MT, Adams WB, Callen JP. Treatment of severe cutaneous small-vessel vasculitis with mycophenolate mofetil. Arch Dermatol. 2012;148(8):887-8. [Crossref]  [PubMed]
  12. Micheletti RG, Pagnoux C. Management of cutaneous vasculitis. Presse Med. 2020;49(3): 104033. [Crossref]  [PubMed]
  13. Lopez LR, Davis KC, Kohler PF, Schocket AL. The hypocomplementemic urticarial-vasculitis syndrome: therapeutic response to hydroxychloroquine. J Allergy Clin Immunol. 1984;73:600-3. [Crossref]  [PubMed]
  14. Nurnberg W, Grabbe J, Czametzki BM. Synergistic effects of pentoxifylline and dapsone in leucocytoclastic vasculitis. Lancet. 1994;343(8895):491. [Crossref]  [PubMed]
  15. Chung L, Funke AA, Chakravarty EF, Callen JP, Fiorentino DF. Successful use of rituximab for cutaneous vasculitis. Arch Dermatol. 2006;142(11):1407-10. [Crossref]  [PubMed]
  16. Mang R, Ruzicka T, Stege H. Therapy for severe necrotizing vasculitis with infliximab. J Am Acad Dermatol. 2004;51(2):321-2. [Crossref]  [PubMed]
  17. Tosca A, Ioannidou DJ, Katsantonis JC, Kyriakis KP. Cyclosporin A in the treatment of cutaneous vasculitis. Clinical and cellular effects. J Eur Acad Dermatol Venereol. 1996;6:135-41. [Crossref]
  18. Ong CS, Benson EM. Successful treatment of chronic leucocytoclastic vasculitis and persistent ulceration with intravenous immunoglobulin. Br J Dermatol. 2000;143(2):447. [Crossref]  [PubMed]
  19. Thomas AE, Baird SF, Anderson J. Purpuric and petechial rashes in adults and children: initial assessment. BMJ. 2016; 352: i1285. [Crossref]  [PubMed]
  20. Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology (Oxford). 2019;58(9):1607-1616. [Crossref]  [PubMed]
  21. Reamy BV, Servey JT, Williams PM. Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review. Am Fam Physician. 2020;102(4):229-233. [PubMed]
  22. Kolkhir P, Bonnekoh H, Kocatürk E, Hide M, Metz M, Sánchez-Borges M, et al. Management of urticarial vasculitis: A worldwide physician perspective. World Allergy Organ J 2020;13(3). [Crossref]  [PubMed]  [PMC]
  23. Gu SL, Jorizzo JL. Urticarial vasculitis. Int J Womens Dermatol. 2021;7(3):290-297. [Crossref]  [PubMed]  [PMC]
  24. Kolkhir P, Grakhova M, Bonnekoh H, Krause K, Maurer M. Treatment of urticarial vasculitis: A systematic review. J Allergy Clin Immunol 2019;143(2):458-66. [Crossref]  [PubMed]
  25. E. Muchtar, H. Magen, M. Gertz, How I treat cryoglobulinemia, Blood 129 (3) (2017 Jan 19) 289-298. [Crossref]  [PubMed]
  26. Cacoub P, Si Ahmed SN, Ferfar Y, Pol S, Thabut D,Hezode C, et al. Long-term efficacy of interferon-free antiviral treatment regimens in patients with hepatitis C virus-associated cryoglobulinemia vasculitis. Clin Gastroenterol Hepatol. (2019) 17:518-26. [Crossref]  [PubMed]
  27. Monti G, Saccardo F, Rinaldi G, Petrozzino MR, Gomitoni A, Invernizzi F. Colchicine in the treatment of mixed cryoglobulinemia. Clin Exp Rheumatol. (1995) 13:S197-9. [PubMed]
  28. Invernizzi F, Monti G. Colchicine and mixed cryoglobulinemia. Arthritis Rheum. (1993) 36:722-3. [Crossref]  [PubMed]
  29. De Vita S, Quartuccio L, Isola M, Mazzaro C, Scaini P, Lenzi M, et al. A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis. Arthritis Rheum. (2012) 64:843 53. [Crossref]  [PubMed]
  30. Donghi D, Schanz U, Sahrbacher U, Recher M, Trüeb RM, Müllhaupt B, et al. Life-threatening or organ-impairing HenochSchönlein purpura: plasmapheresis may save lives and limit organ damage. Dermatology. (2009) 219:167-70. [Crossref]  [PubMed]
  31. Kuhl V, Vogt T, Anghelescu I. Intravenous immunoglobulin and prednisolone treatment of cryoglobulinemic polyneuropathy. Nervenarzt. (2001) 72:445-8. [Crossref]  [PubMed]
  32. Ferri C, Giuggioli D, Cazzato M, Sebastiani M, Mascia MT, Zignego AL, et al. HCV-related cryoglobulinemic vasculitis: an update on its etiopathogenesis and therapeutic strategies. Clin Exp Rheumatol. (2003) 21:S78-84. [PubMed]
  33. Momen SE, Jorizzo J, Al-Niaimi F. Erythema elevatum diutinum: a review of presentation and treatment. J Eur Acad Dermatol Venereol. 2014;28(12):1594-1602. [Crossref]  [PubMed]
  34. Frankel DH, Soltani K, Medenica MM, Rippon JW. Tinea of the face caused by Trichophyton rubrum with histologic changes of granuloma faciale. J Am Acad Dermatol 1988; 18: 403-406. [Crossref]  [PubMed]
  35. Ortonne N, Wechsler J, Bagot M, Grosshans E, Cribier B. Granuloma faciale: a clinicopathologic study of 66 patients. J Am Acad Dermatol 2005; 53: 1002-1009. [Crossref]  [PubMed]
  36. Lindhaus C, Elsner P. Granuloma Faciale Treatment: A Systematic Review. Acta Derm Venereol. 2018;98(1):14-18. [Crossref]  [PubMed]
  37. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. 1990;33(8):1101-1107. [Crossref]  [PubMed]
  38. Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, et al. 2021 American College of Rheumatology/ Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol. 2021;73(8):1366-1383. [Crossref]  [PubMed]
  39. Vaglio, A., Buzio, C. & Zwerina, J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy 68, 261-273 (2013). [Crossref]  [PubMed]
  40. Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, et al. Evidence-Based Guideline ort he diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2023;19(6):378-393. [Crossref]  [PubMed]
  41. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al.: EULAR/ERA-EDTA recommendations for the management of ANCAassociated vasculitis. Ann Rheum Dis. 2016, 75:1583-94. [Crossref]  [PubMed]
  42. Jain K, Jawa P, Derebail VK, Falk RJ: Treatment updates in antineutrophil cytoplasmic autoantibodies (ANCA) vasculitis. Kidney360. 2021, 2:763-70. [Crossref]  [PubMed]  [PMC]
  43. Neumann I: Immunosuppressive and glucocorticoid therapy for the treatment of ANCA-associated vasculitis. Rheumatology (Oxford). 2020, 59:iii60-7. 10.1093/rheumatology/keaa035 https://pubmed.ncbi.nlm.nih.gov/32348519/ [Crossref]  [PubMed]
  44. Samman KN, Ross C, Pagnoux C, Makhzoum JP: Update in the management of ANCA-associated vasculitis: recent developments and future perspectives. Int J Rheumatol. 2021, 2021:5534851. [Crossref]  [PubMed]  [PMC]
  45. Onuora S: Avacopan offers alternative to steroids for ANCA associated vasculitis . Nat Rev Rheumatol. 2021, 17:249. 10.1038/s41584-021-00615-0 [Crossref]  [PubMed]
  46. Sexton DJ: Plasma exchange and glucocorticoids in severe ANCA associated vasculitis . N Engl J Med. 2020, 382:2168. 10.1056/NEJMc2004843 [Crossref]  [PubMed]
  47. van der Geest KSM, Brouwer E, Sanders JS, Sandovici M, Bos NA, Boots AMH, et al.: Towards precision medicine in ANCA-associated vasculitis . Rheumatology (Oxford). 2018, 57:1332-9. [Crossref]  [PubMed]
  48. Chanouzas D, McGregor JAG, Nightingale P, Salama AD, Szpirt WM, Basu N, et al.: Intravenous pulse methylprednisolone for induction of remission in severe ANCA associated vasculitis: a multi-center retrospective cohort study. BMC Nephrol. 2019, 20:58. 10.1186/s12882-019-1226-0 [Crossref]  [PubMed]  [PMC]
  49. Hernandez-Rodriguez J, Alba MA, Prieto-Gonzalez S, Cid MC. Diagnosis and classification of polyarteritis nodosa [review]. J Autoimmun 2014;48-49: 84-9. [Crossref]  [PubMed]
  50. Chung SA, Gorelik M, Langford CA, Maz M, Abril A, Guyatt G, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa. Arthritis Care Res (Hoboken). 2021;73(8):10611070. [Crossref]  [PubMed]
  51. Segura S, Pujol RM, Trindade F, Requena L. Vasculitis in erythema induratum of Bazin: a histopathologic study of 101 biopsy specimens from 86 patients. J Am Acad Dermatol. 2008;59(5):839-51. [Crossref]  [PubMed]
  52. Schulz EJ, Whiting DA. Treatment of erythema nodosum and nodular vasculitis with potassium iodide. Br J Dermatol 1976: 94: 75-78. [Crossref]  [PubMed]
  53. Horio T, Imamura S, Danno K, Ofuji S. Potassium iodide in the treatment of erythema nodosum and nodular vasculitis. Arch Dermatol 1981: 117: 29-31. [Crossref]  [PubMed]
  54. Wee E, Kelly RI. Treatment of nodular vasculitis with colchicine. Australas J Dermatol. (2017) 58:e79-82. [PubMed]
  55. Taverna JA, Radfar A, Pentland A, Poggioli G, Demierre MF. Case reports: nodular vasculitis responsive to mycophenolate mofetil. J Drugs Dermatol 2006: 5: 992-993. [PubMed]
  56. Shaffer N, Kerdel FA. Nodular vasculitis (erythema induratum): treatment with auranofin. J Am Acad Dermatol 1991: 25: 426-429. [Crossref]  [PubMed]
  57. Capra JD, Winchester RJ, Kunkel HG. Hypergammaglobulinemic purpura. Studies on the unusual anti-globulins characteristic of the sera of these patients. Medicine. 1971;50(2):12538. [Crossref]  [PubMed]
  58. Malaviya AN, Kaushik P, Budhiraja S, al-Mutairi M, Nampoory MR, Hussein A, et al. Hypergammaglobulinemic purpura of Waldenstrom: report of 3 cases with a short review. Clinical and experimental rheumatology. 2000;18(4):518-22. [PubMed]
  59. Waldenstrom J. Hyperglobulinemic purpura, chronic sialoadenitis and Sjogren's syndrome. Scand J Rheumatol Suppl 1986;61:139-41. [Crossref]  [PubMed]
  60. Lopez LR,Schocket AL, Carr RI. et al. Lymphocytotoxic antibodies and intermediate immune complexes in hypergammaglobulinemic purpura of Waldenstrom. Ann Allergy 1988;61:93-6 [PubMed]
  61. Hudson CP, Callen JP. Cutaneous leukocytoclastic vasculitis with hyperglobulinemia and splenomegaly. Arch Dermatol 1984;120:1224-6 [Crossref]  [PubMed]
  62. Braverman IM, Yen A. Demonstration of immune complexes in spontaneous and histamine-induced lesions and in normal skin of patients with leukocytoclastic angitis. J Invest Dermatol. 1975;64(2):105-12. [Crossref]  [PubMed]
  63. Sanchez NP, Van Hale HM, Su WP. Clinical and histopathologic spectrum of necrotizing vasculitis. Report of findings in 101 cases. Arch Dermatol. 1985;121(2):220-4. [Crossref]  [PubMed]
  64. Callen JP. af Ekenstam E. Cutaneous leukocytoclastic vasculitis: clinical experience in 44 patients. South Med J. (1987) 80:848- 51. [Crossref]  [PubMed]
  65. Sais G, Vidaller A, Jucgla A, Gallardo F, Peyri J. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol. (1995) 131:1399-402. [Crossref]  [PubMed]
  66. Fredenberg MF, Malkinson FD. Sulfone therapy in the treatment of leukocytoclastic vasculitis. Report of three cases. J Am Acad Dermatol. (1987) 16:772-8. [Crossref]  [PubMed]
  67. Martinez-Taboada VM, Blanco R, Garcia-Fuentes M, Rodriguez-Valverde V. Clinical features and outcome of95 patients with hypersensitivity vasculitis. Am J Med. 1997 Feb;102(2):186-91. [Crossref]  [PubMed]
  68. Russel JP, Gibson LE. Primary cutaneous small vessel vasculitis: approach to diagnosis and treatment. Int J Dermatol. (2006) 45:3-13. [Crossref]  [PubMed]
  69. Espinoza LR, Espinoza CG, Vasey FB, Germain BF. Oral methotrexate therapy for chronic rheumatoid arthritis ulcerations. J Am Acad Dermatol. (1986) 15:508. [Crossref]  [PubMed]