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Cutaneous Vasculitis: A Rheumatologist Perspective

  • ALLERGIC SKIN DISEASES (L FONACIER, SECTION EDITOR)
  • Published:
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Abstract

Cutaneous vasculitis (CV) comprises a wide spectrum of diseases and displays a variety of clinical lesions, the most common being a palpable purpura. CV may be a process confined exclusively to the skin or be a manifestation of a more widespread entity associated to a variable grade of visceral involvement. In this regard, CV may occur as part of the clinical spectrum of primary systemic vasculitis, autoimmune diseases or less commonly as presenting manifestation of cancer or severe infections. An adequate clinical approach is required to establish optimal management of this condition.

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Abbreviations

AAV:

ANCA-associated vasculitis

ACR:

American College of Rheumatology

ANCA:

Anti-neutrophil cytoplasmic antibody

CHCC:

Chapel Hill Consensus Conference

CTD:

Connective tissue diseases

CV:

Cutaneous vasculitis

EGPA:

Eosinophilic granulomatosis with polyangiitis

EULAR:

European League Against Rheumatism

GPA:

Granulomatosis with polyangiitis

HBV:

Hepatitis B virus

HCV:

Hepatitis C virus

HV:

Hypersensitivity vasculitis

IgAV:

IgA vasculitis

MPA:

Microscopic polyangiitis

PAN:

Polyarteritis nodosa

PSS:

Primary Sjögren syndrome

PSV:

Primary systemic vasculitis

RA:

Rheumatoid arthritis

SLE:

Systemic lupus erythematosus

References

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

  1. Gonzalez-Gay MA, Garcia-Porrua C. Epidemiology of the vasculitides. Rheum Dis Clin North Am. 2001;27:729–49.

    Article  PubMed  CAS  Google Scholar 

  2. Hunder GG. Vasculitis: diagnosis and therapy. Am J Med. 1996;100(Suppl 2A):37S–45.

    Article  PubMed  CAS  Google Scholar 

  3. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides: the proposal of an international consensus conference. Arthritis Rheum. 1994;37:187–92.

    Article  PubMed  CAS  Google Scholar 

  4. •• Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11. This has the most recent nomenclature and definitions for the vasculitides. It emphasizes the importance of histological features for the classification of vasculitides.

    Article  PubMed  CAS  Google Scholar 

  5. Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990;33:1122–8.

    Article  PubMed  CAS  Google Scholar 

  6. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33:1129–34.

    Article  PubMed  CAS  Google Scholar 

  7. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33:1101–7.

    Article  PubMed  CAS  Google Scholar 

  8. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic Granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094–100.

    Article  PubMed  CAS  Google Scholar 

  9. Lightfodd Jr RW, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33:1088–93.

    Article  Google Scholar 

  10. Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum. 1990;33:1114–21.

    Article  PubMed  CAS  Google Scholar 

  11. Calabrese LH, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis. Arthritis Rheum. 1990;33:1108–13.

    Article  PubMed  CAS  Google Scholar 

  12. Luqmani RA, Suppiah R, Grayson PC, et al. Nomenclature and classification of vasculitis-update on the ACR/EULAR Diagnosis and Classification of Vasculitis Study (DCVAS). Clin Exp Immunol. 2011;164:11–3.

    Article  PubMed  Google Scholar 

  13. Guillevin L, Dorner T. Vasculitis: mechanisms involved and clinical manifestations. Arthritis Res Ther. 2007;9 Suppl 2:s9.

    Article  PubMed  Google Scholar 

  14. Watts RA, Jolliffe VA, Grattan CE, et al. Cutaneous vasculitis in a defined population. Clinical and epidemiological associations. J Rheumatol. 1998;25:920–4.

    PubMed  CAS  Google Scholar 

  15. García-Porrúa C, Gonzalez-Gay MA. Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schönlein purpura in adults. Semin Arthritis Rheum. 1999;28:404–12.

    Article  PubMed  Google Scholar 

  16. Leelavathi M, Aziz SA, Gangaram HB, Hussein SH. Cutaneous vasculitis: A review of aetiology and clinical manifestations in 85 patients in Malaysia. Med J Malaysia. 2009;64:210–2.

    PubMed  CAS  Google Scholar 

  17. Al-Mutairi N. Spectrum of cutaneous vasculitis in adult patients from the Farwaniya region of Kuwait. Med Princ Pract. 2008;17:43–8.

    Article  PubMed  Google Scholar 

  18. Chua SH, Lim JT, Ang CB. Cutaneous vasculitis seen at a skin referral centre in Singapore. Singap Med J. 1999;40:147–50.

    CAS  Google Scholar 

  19. Tai YJ, Chong AH, Williams RA, et al. Retrospective analysis of adult patients with cutaneous leucocytoclastic vasculitis. Australas J Dermatol. 2006;47:92–6.

    Article  PubMed  Google Scholar 

  20. Gyselbrecht L, Keyser F, Ongenae K, et al. Etiological factors and underlying conditions in patients with leucocytoclastic vasculitis. Clin Exp Rheumatol. 1996;14:665–8.

    PubMed  CAS  Google Scholar 

  21. García-Porrúa C, Gonzalez-Gay MA. Bacterial infection presenting as cutaneous vasculitis in adults. Clin Exp Rheumatol. 1999;17:471–3.

    PubMed  Google Scholar 

  22. Jessop SJ. Cutaneous leukocytoclastic vasculitis: a clinical and aetiological study. Br J Rheumatol. 1995;34:942–5.

    Article  PubMed  CAS  Google Scholar 

  23. Hodge SJ, Callen JP, Ekenstam E. Cutaneous leukocytoclastic vasculitis: correlation of histopathological changes with clinical severity and course. J Cutan Pathol. 1987;14:279–84.

    Article  PubMed  CAS  Google Scholar 

  24. Ekenstam E, Callen JP. Cutaneous leukocytoclastic vasculitis: clinical and laboratory features of 82 patients seen in private practice. Arch Dermatol. 1984;120:484–9.

    Article  Google Scholar 

  25. García-Porrua C, Gonzalez-Gay MA, Lopez-Lazaro L. Drug associated cutaneous vasculitis in adults in northwestern Spain. J Rheumatol. 1999;26:1942–4.

    PubMed  Google Scholar 

  26. Gibson LE, Su D. Cutaneous vasculitis. Rheum Dis Clin North Am. 1995;21:1097–113.

    PubMed  CAS  Google Scholar 

  27. Garcia-Porrua C, Gonzalez-Gay MA. Cutaneous vasculitis as a paraneoplastic syndrome in adults. Arthritis Rheum. 1998;41:1133–5.

    Article  PubMed  CAS  Google Scholar 

  28. Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, García-Fuentes M. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine. 1998;77:403–18.

    Article  PubMed  CAS  Google Scholar 

  29. Stone JH, Nousari HC. “Essential” cutaneous vasculitis: what every rheumatologist should know about vasculitis of the skin. Curr Opin Rheumatol. 2001;13:23–34.

    Article  PubMed  CAS  Google Scholar 

  30. Gonzalez-Gay MA, García-Porrua C, Salvarani C, et al. Cutaneous vasculitis: a diagnostic approach. Clin Exp Rheumatol. 2003;21 Suppl 32:S85–8.

    PubMed  CAS  Google Scholar 

  31. Gonzalez-Gay MA, García-Porrua C, Pujol RM. Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol. 2005;17:56–61.

    PubMed  Google Scholar 

  32. Gonzalez-Gay MA, García-Porrua C, Lueiro M, et al. Scurvy can mimic cutaneous vasculitis: three case reports. Rev Rhum Engl Ed. 1999;66:360–1.

    PubMed  CAS  Google Scholar 

  33. Khetan P, Sethuraman G, Khaitan BK, et al. An aetiological and clinicopathological study on cutaneous vasculitis. Indian J Med Res. 2012;135:107–13.

    Article  PubMed  Google Scholar 

  34. Garcia-Porrua C, Llorca J, Gonzalez-Louzao C, González-Gay MA. Hypersensitivity vasculitis in adults: a benign disease usually limited to skin. Clin Exp Rheumatol. 2001;19:85–8.

    PubMed  CAS  Google Scholar 

  35. Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensitivity vasculitis and Henoch-Schönlein purpura: a comparison between the 2 disorders. J Rheumatol. 1992;19:721–8.

    PubMed  CAS  Google Scholar 

  36. Martinez-Taboada VM, Blanco R, Garcia-Fuentes M, et al. Clinical outcome of 95 patients wih hypersensitivity vasculitis. Am J Med. 1997;102:186–91.

    Article  PubMed  CAS  Google Scholar 

  37. Pagnoux C, Seror R, Henegar C, et al. Clinical features and outcomes in 348 patients with Polyarteritis Nodosa. A systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62:616–26.

    Article  PubMed  Google Scholar 

  38. Fiorentino D. Cutaneous vasculitis. J Am Acad Dermatol. 2003;48:311–40.

    Article  PubMed  Google Scholar 

  39. • Chen KR. Skin involvement in ANCA-associated vasculitis. Clin Exp Nephrol 2012 Nov 28 [Epub ahead of print]. Elegant and concise review of skin involvement in ANCA-associated vasculitis.

  40. Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis. Clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42:421–30.

    Article  PubMed  CAS  Google Scholar 

  41. Frances C, Du LT, Piette JC, et al. Wegener’s granulomatosis. Dermatological manifestations in 75 cases with clinicopathologic correlation. Arch Dermatol. 1994;130:861–7.

    Article  PubMed  CAS  Google Scholar 

  42. Lie JT. Wegener’s granulomatosis: histological documentation of common and uncommon manifestations in 216 patients. Vasa. 1997;26:261–70.

    PubMed  CAS  Google Scholar 

  43. Patten SE, Tomecki KJ. Wegener´s granulomatosis: cutaneous and oral mucosal disease. J Am Acad Dermatol. 1993;28:710–8.

    Article  PubMed  CAS  Google Scholar 

  44. Zycinska K, Wardyn K, Zielonka TM, et al. Cutaneous changes: an initial manifestation of pulmonary Wegener´s granulomatosis. Adv Exp Med Biol. 2013;755:307–10.

    Article  PubMed  Google Scholar 

  45. Barksdale SK, Hallahan CW, Kerr GS, et al. Cutaneous pathology in Wegener’s granulomatosis. A clinicopathologic study of 75 biosies in 46 patients. Am J Surg Pathol. 1995;19:161–72.

    Article  PubMed  CAS  Google Scholar 

  46. Bosco L, Peroni A, Schena D, et al. Cutaneous manifestations of Churg-Strauss syndrome: report of two cases and review of the literature. Clin Rheumatol. 2011;30:573–80.

    Article  PubMed  Google Scholar 

  47. Davis MD, Daoud MS, Mcevoy MT, Su WP. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. 1997;37:199–203.

    Article  PubMed  CAS  Google Scholar 

  48. Garcia-Porrua C, Calviño MC, Llorca J, et al. Henoch-Schönlein purpura in children and adults: clinical differences in a defined population. Semin Arthritis Rheum. 2002;32:149–56.

    Article  PubMed  Google Scholar 

  49. Calviño MC, Llorca J, Garcia-Porrua C, et al. Henoch-Schönlein purpura in children from northwestern Spain: a 20 year epidemiologic and clinical study. Medicine (Baltimore). 2001;80:279–90.

    Article  Google Scholar 

  50. Saulsbury FT. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine. 1999;88:395–409.

    Article  Google Scholar 

  51. Shin JI, Park JM, Shin YH, et al. Predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch-Schönlein purpura. Scand J Rheumatol. 2006;35:56–60.

    Article  PubMed  CAS  Google Scholar 

  52. Rigante D, Candelli M, Federico G, et al. Predictive factors of renal involvement or relapsing disease in children with Henoch-Schönlein purpura. Rheumatol Int. 2005;25:45–8.

    Article  PubMed  Google Scholar 

  53. Byun JW, Song HJ, Kim L, et al. Predictive factors of relapse in adult with Henoch-Schönlein purpura. Am J Dermatopathol. 2012;34:139–44.

    Article  PubMed  Google Scholar 

  54. • Poterucha TJ, Wetter DA, Gibson LE, et al. Histopathology and correlates of systemic disease in adult Henoch-Schönlein purpura: a retrospective study of microscopic and clinical findings in 68 patients at Mayo Clinic. J Am Acad Dermatol. 2013;68:420–4. The authors disclosed a correlation between histopathological findings in the skin biopsy and the risk of kidney involvement in patients with Henoch–Schönlein purpura.

    Article  PubMed  Google Scholar 

  55. Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet. 2012;379:348–60.

    Article  PubMed  CAS  Google Scholar 

  56. Ferri C, Sebastiani M, Giuggioli D, et al. Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients. Semin Arthritis Rheum. 2004;33:355–74.

    Article  PubMed  Google Scholar 

  57. Trejo O, Ramos-Casals M, Garcia-Carrasco M, et al. Cryoglobulinemia. Study of etiologic factors and clinical and immunologic features in 443 patients from a single center. Medicine (Baltimore). 2001;80:252–62.

    Article  CAS  Google Scholar 

  58. Terrier B, Krastinova E, Marie I, et al. Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the cryoVas survey. Blood. 2012;119:5996–6004.

    Article  PubMed  CAS  Google Scholar 

  59. Terrier B, Karras A, Kahn JE, et al. The spectrum of type I cryoglobulinemia vasculitis. New insights based on 64 cases. Medicine (Baltimore). 2013;92:61–8.

    Article  CAS  Google Scholar 

  60. Turesson C, O’Fallon WM, Crowson CS, et al. Extra-articular disease manifestations in rheumatoid arthritis: incidence trends and risk factors over 46 years. Ann Rheum Dis. 2003;62:722–7.

    Article  PubMed  CAS  Google Scholar 

  61. Genta MS, Genta RM, Gabay C. Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum. 2006;36:88–98.

    Article  PubMed  Google Scholar 

  62. Chen KR, Toyohara A, Suzuki A, Miyakawa S. Clinical and histopathological spectrum of cutaneous vasculitis in rheumatoid arthritis. Br J Dermatol. 2002;147:905–13.

    Article  PubMed  Google Scholar 

  63. Cimmino MA, Salvarani C, Macchioni P, et al. Extra-articular manifestations in 587 Italian patients with rheumatoid arthritis. Rheumatol Int. 2000;19:213–7.

    Article  PubMed  CAS  Google Scholar 

  64. Kaye O, Beckers CC, Paquet P, et al. The frequency of cutaneous vasculitis is not increased in patients with rheumatoid arthritis treated with methotrexate. J Rheumatol. 1996;23:253–7.

    PubMed  CAS  Google Scholar 

  65. Turesson C, O’Fallon WM, Crowson CS, et al. Occurrence of extraarticular disease manifestations is associated with excess mortality in a community based cohort of patients with rheumatoid arthritis. J Rheumatol. 2002;29:62–7.

    PubMed  Google Scholar 

  66. Gonzalez-Gay MA, Garcia-Porrua C. Systemic vasculitis in adults in northwestern Spain, 1988–1997. Clinical and epidemiological aspects. Medicine (Baltimore). 1999;78:292–308.

    Article  CAS  Google Scholar 

  67. Ergun T, Inanc N, Tuney D, et al. Skin manifestations of rheumatoid arthritis: a study of 215 Turkish patients. Int J Dermatol. 2008;47:894–902.

    Article  PubMed  Google Scholar 

  68. Turesson C, Jacobsson L, Bergström U. Extra-articular rheumatoid arthritis: prevalence and mortality. Rheumatology. 1999;38:668–74.

    Article  PubMed  CAS  Google Scholar 

  69. Lopez-Longo FJ, Gonzalez-Fernandez CM, Rodriguez-Mahou M, et al. Vasculitis in systemic lupus erithematosus. Prevalence and clinical characteristic in 670 patients. Medicine (Baltimore). 2006;85:95–104.

    Article  Google Scholar 

  70. Lopez-Longo FJ, Gonzalez-Fernandez CM, Rodriguez-Mahou M, et al. Clinical expression of systemic lupus erythematosus with anti-U1-RNP and anti-Sm antibodies. Rev Clin Esp. 1997;197:329–35.

    PubMed  CAS  Google Scholar 

  71. Fukuda MV, Lo SC, Almeida CS, Shinjo SK. Anti-Ro antibody and cutaneous vasculitis in systemic lupus erythematosus. Clin Rheumatol. 2009;28:301–4.

    Article  PubMed  Google Scholar 

  72. Shinjo SK, Bonfa E. Cutaneous vasculitis in systemic lupus erythematosus: association with ant-ribosomal P protein antibody and Raynaud phenomenon. Clin Rheumatol. 2011;30:173–7.

    Article  PubMed  Google Scholar 

  73. Wu CS, Hu CY, Chan CJ, Chang SK, Hsu PN. Genetic polymorphism of the growth arrest-specific 6 gene is associated with cutaneous vasculitis in Taiwanese patients with systemic lupus erythematosus. Clin Rheumatol. 2012;31:1443–8.

    Article  PubMed  Google Scholar 

  74. Malladi AS, Sack KE, Shiboski SC, et al. Primary Sjögren syndrome as a systemic disease: a study of participants enrolled in an international Sjögren’s syndrome registry. Arthritis Care Res. 2012;64:911–8.

    Article  Google Scholar 

  75. Ramos-Casals M, Anaya JM, Garcia-Carrasco M, et al. Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients. Medicine (Baltimore). 2004;83:96–106.

    Article  Google Scholar 

  76. Carlson JA. The histological assessment of cutaneous vasculitis. Histopathology. 2010;56:3–23.

    Article  PubMed  Google Scholar 

  77. Kluger N, Frances C. Cutaneous vasculitis and their differential diagnosis. Clin Exp Rheumatol. 2009;27 suppl 52:S124–38.

    PubMed  CAS  Google Scholar 

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Conflict of Interest

Trinitario Pina declares that he has no conflict of interest.

Ricardo Blanco declares that he has no conflict of interest.

Miguel A. González-Gay declares that he has no conflict of interest.

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

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Authors and Affiliations

  1. Rheumatology Division, Hospital de Torrevieja, Alicante, Spain

    Trinitario Pina

  2. Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IFIMAV, Avenida de Valdecilla, s/n, 39008, Santander, Spain

    Ricardo Blanco & Miguel A. González-Gay

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  1. Trinitario Pina
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  2. Ricardo Blanco
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Correspondence to Miguel A. González-Gay.

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Pina, T., Blanco, R. & González-Gay, M.A. Cutaneous Vasculitis: A Rheumatologist Perspective. Curr Allergy Asthma Rep 13, 545–554 (2013). https://doi.org/10.1007/s11882-013-0367-7

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