Vasculitis
Cutaneous Manifestations of Vasculitis

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Objectives

To discuss the clinical features, diagnostic evaluation, and treatment options for cutaneous vasculitis.

Methods

The literature in the PubMed database was reviewed regarding the presentation, pathophysiology, clinical workup, and treatment of cutaneous vasculitis.

Results

Available classification criteria of vasculitis are based on histopathologic criteria or clinicohistologic features. These have been designed more for research purposes than for clinical application. Skin findings such as palpable purpura, nodules, urticaria, ulcers, and infarction are clues to the presence of vasculitis. Pathologic findings of fibrinoid necrosis, infiltration by neutrophils or lymphocytes, and deposition of complement and immunoglobulin may be helpful in reaching a specific diagnosis. However, there is considerable overlap across different conditions.

Conclusions

The correct diagnosis of cutaneous manifestations of vasculitis requires an understanding of vasculitis classification, recognition of specific clinical patterns, and the ability to interpret histopathologic data.

Section snippets

Methods

We reviewed the English literature in the PubMed database using the search terms: “cutaneous vasculitis” and “cutaneous” in combination with “urticarial vasculitis,” “Henoch–Schönlein purpura,” “cryoglobulinemic vasculitis,” “polyarteritis nodosa,” “Kawasaki disease,” “microscopic polyangiitis,” “Wegener granulomatosis,” or “Churg–Strauss syndrome.” Auxiliary search terms used in conjunction with the above keywords included “rituximab,” “etanercept,” “infliximab,” “dapsone,” “treatment,” and

Classification of Cutaneous Vasculitis

Vasculitides are challenging to diagnose and classify, because their protean manifestations overlap with a broad spectrum of disorders. Moreover, individual vasculitic disorders do not share a single etiology or pathologic mechanism. Recent classification schemes are based on histopathologic criteria in the case of the Chapel Hill Consensus Conference and clinicohistologic features as developed by the American College of Rheumatology (4, 5). These classifications stratify patients who are

Discussion

Cutaneous vasculitis presents in a wide variety of conditions with different degrees of systemic involvement. It is crucial to obtain a careful history, physical examination, and necessary diagnostic tests to distinguish self-limited conditions confined to the skin from those with systemic involvement and serious sequelae. It is challenging to organize one's approach to cutaneous vasculitis. Excessive focus on vessel size, serological findings, or the types of cellular infiltrates may lead to

References (112)

  • R.I. Fox et al.

    Sjogren's syndrome in dermatology

    Clin Dermatol

    (2006)
  • M.D. Davis et al.

    Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation

    J Am Acad Dermatol

    (1997)
  • A. Churg

    Recent advances in the diagnosis of Churg-Strauss syndrome

    Mod Pathol

    (2001)
  • I.M. Braverman et al.

    Demonstration of immune complexes in spontaneous and histamine-induced lesions and in normal skin of patients with leukocytoclastic angitis

    J Invest Dermatol

    (1975)
  • M.D. Davis et al.

    Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis

    J Am Acad Dermatol

    (1998)
  • J.A. Carlson et al.

    Cutaneous vasculitis: diagnosis and management

    Clin Dermatol

    (2006)
  • N.P. Sanchez et al.

    The clinical and histopathologic spectrums of urticarial vasculitis: study of forty cases

    J Am Acad Dermatol

    (1982)
  • J.L. Millns et al.

    The therapeutic response of urticarial vasculitis to indomethacin

    J Am Acad Dermatol

    (1980)
  • L.R. Lopez et al.

    The hypocomplementemic urticarial-vasculitis syndrome: therapeutic response to hydroxychloroquine

    J Allergy Clin Immunol

    (1984)
  • Y. Ilan et al.

    Schonlein-Henoch syndrome in adults and children

    Semin Arthritis Rheum

    (1991)
  • R. Coppo et al.

    Predictors of outcome in Henoch-Schonlein nephritis in children and adults

    Am J Kidney Dis

    (2006)
  • J. Ronkainen et al.

    Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial

    J Pediatr

    (2006)
  • J.C. Brouet et al.

    Biologic and clinical significance of cryoglobulinsA report of 86 cases

    Am J Med

    (1974)
  • F. Dammacco et al.

    Natural interferon-alpha versus its combination with 6-methyl-prednisolone in the therapy of type II mixed cryoglobulinemia: a long-term, randomized, controlled study

    Blood

    (1994)
  • C. Mat et al.

    Small vessel vasculitis and vasculitis confined to skin

    Baillieres Clin Rheumatol

    (1997)
  • A.P. Sheth et al.

    Cutaneous polyarteritis nodosa of childhood

    J Am Acad Dermatol

    (1994)
  • J.C. Burns et al.

    Kawasaki syndrome

    Lancet

    (2004)
  • C.G. Kallenberg et al.

    Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential

    Kidney Int

    (1994)
  • A.S. Wiik

    Clinical use of serological tests for antineutrophil cytoplasmic antibodiesWhat do the studies say?

    Rheum Dis Clin North Am

    (2001)
  • T. Lotti et al.

    Cutaneous small-vessel vasculitis

    J Am Acad Dermatol

    (1998)
  • D.F. Fiorentino

    Cutaneous vasculitis

    J Am Acad Dermatol

    (2003)
  • W.M. Sams et al.

    Leukocytoclastic vasculitis

    Arch Dermatol

    (1976)
  • G.G. Hunder et al.

    The American College of Rheumatology 1990 criteria for the classification of vasculitisIntroduction

    Arthritis Rheum

    (1990)
  • J.C. Jennette et al.

    Nomenclature of systemic vasculitidesProposal of an international consensus conference

    Arthritis Rheum

    (1994)
  • J.K. Rao et al.

    Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis

    Ann Intern Med

    (1998)
  • R.A. Watts et al.

    Effect of classification on the incidence of polyarteritis nodosa and microscopic polyangiitis

    Arthritis Rheum

    (1996)
  • J.H. Stone et al.

    “Essential” cutaneous vasculitis: what every rheumatologist should know about vasculitis of the skin

    Curr Opin Rheumatol

    (2001)
  • P. Seo et al.

    Large-vessel vasculitis

    Arthritis Rheum

    (2004)
  • J.C. Jennette et al.

    Small-vessel vasculitis

    N Engl J Med

    (1997)
  • J.A. Carlson et al.

    Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis

    Am J Dermatopathol

    (2005)
  • D.R. Mehregan et al.

    Pathophysiology of urticarial vasculitis

    Arch Dermatol

    (1998)
  • R.A. Watts et al.

    Epidemiology of the vasculitides

    Curr Opin Rheumatol

    (2003)
  • N.A. Galaria et al.

    Leukocytoclastic vasculitis due to etanercept

    J Rheumatol

    (2000)
  • M.E. McCain et al.

    Etanercept and infliximab associated with cutaneous vasculitis

    Rheumatology (Oxford)

    (2002)
  • M.D. Srivastava et al.

    Immunology of cutaneous vasculitis associated with both etanercept and infliximab

    Scand J Immunol

    (2005)
  • L. Chung et al.

    Successful use of rituximab for cutaneous vasculitis

    Arch Dermatol

    (2006)
  • P. Kandula et al.

    Rituximab-induced leukocytoclastic vasculitis: a case report

    Arch Dermatol

    (2006)
  • B.F. Mandell et al.

    Infections and systemic vasculitis

    Curr Opin Rheumatol

    (1998)
  • T. Somer et al.

    Vasculitides associated with infections, immunization, and antimicrobial drugs

    Clin Infect Dis

    (1995)
  • O. Trejo et al.

    Cryoglobulinemia: study of etiologic factors and clinical and immunologic features in 443 patients from a single center

    Medicine (Baltimore)

    (2001)

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    Source of Support: None.

    Lisa Y. Xu, MD, and Edward M. Esparza, MD, contributed equally to this article.

    Conflict of Interest: None.

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