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Cutaneous Vasculitis: A Clinical Approach

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Clinical and Basic Immunodermatology

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Abstract

Vasculitis, an inflammatory condition of blood vessel walls, varies in severity from a self-limited disorder to a life-threatening disease, and can be caused by many different mechanisms. This condition can be an idiopathic primary process or a secondary manifestation of certain triggers such as infection, malignancy or systemic inflammatory conditions. Vasculitis can present many challenges to the clinician, including diagnosis and classification, necessary diagnostic studies and appropriate treatment. Depending on the organs and caliber of blood vessels involved, vasculitis can manifest with a wide spectrum of clinical and histopathologic findings. In cases of suspected vasculitis, the laboratory and histolopathologic studies yield invaluable information beyond the history and physical examination. Confirmation of vasculitis should prompt a search for potential underlying systemic disorders. Treatment for vasculitis is driven by the severity of symptoms and extent of organ involvement. Most cases of vaculitis are isolated occurrences and can be managed conservatively with rest and elevation of affected limb, and nonsteroidal anti-inflammatory drugs for symptom control. For disease that is recalcitrant or with evidence of systemic involvement, more aggressive therapy, including immunosuppressive agents is necessary. Traditional agents for treating vascultis include corticosteroids, cyclophosphamide, azathioprine, methotrexate, colchicine, dapsone and mycophenolate mofetil. Newer modalities include rituximab and TNF-α inhibitors. This chapter reviews the classification and diagnosis of cutaneous vasculitic conditions and current treatment options.

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Author information

Authors and Affiliations

  1. Department of Dermatology, Broward Health Medical Center, 540 Lido Drive, Fort Lauderdale, FL, 33301, USA

    Carlos H. Nousari MD

  2. Department of Dermatology, Nova Southwestern University, Broward Health Medical Center, Fort Lauderdale, FL, USA

    Michael R. Baze DO, PhD

Authors
  1. Carlos H. Nousari MD
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  2. Michael R. Baze DO, PhD
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Corresponding author

Correspondence to Carlos H. Nousari MD .

Editor information

Editors and Affiliations

  1. School of Medicine, University of Maryland Baltimore, Baltimore, Maryland, USA

    Anthony A. Gaspari

  2. Department of Dermatology, University of Texas Health Science Center, Houston, Texas, USA

    Stephen K. Tyring

  3. Department of Dermatology and Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA

    Daniel H. Kaplan

Appendices

Questions

  1. 1.

    Which of the following statements is true regarding Henoch-Schonlein purpura (HSP) and renal disease?

    1. A.

      End-stage renal disease (ESRD) occurs in 50 % of patients

    2. B.

      Pupura above the waist is associated with the development of renal disease

    3. C.

      Azathioprine is not an effective treatment of nephritis

    4. D.

      Renal insufficiency is more likely to occur in children than in adults

  2. 2.

    Which of the following regarding Polyarteritis nodosa is correct?

    1. A.

      Hepatitis B virus has been implicated as an etiologic agent

    2. B.

      Shallow biopsies that sample superficial dermis are sufficient for diagnosis

    3. C.

      Is the prototype small-vessel vasculitis

    4. D.

      Plasmapheresis is the mainstay of therapy

  3. 3.

    Each of the following distinguishes Churg-Strauss syndrome from Wegener’s granulomatosis except

    1. A.

      Eosinophilia

    2. B.

      Associated atopy and asthma

    3. C.

      Higher levels of p-ANCA

    4. D.

      Granulomatous inflammation

  4. 4.

    The following therapeutic agents have been shown effective for induction of remission in ANCA-associated vasculitis except

    1. A.

      Corticosteroids

    2. B.

      Rituximab

    3. C.

      Cyclophosphamide

    4. D.

      Dapsone

  5. 5.

    All of the following characterize Cryoglobulinemic vasculitis except

    1. A.

      Cryoglobulin type I represents a true vasculitis

    2. B.

      Palpable purpura of the legs is the most common lesion seen

    3. C.

      Cryoglobulin type II patients demonstrate monoclonal IgM rheumatoid factor and polyclonal IgG

    4. D.

      Cold enhances most of the lesions

Answers

  1. 1.

    B

  2. 2.

    A

  3. 3.

    D

  4. 4.

    D

  5. 5.

    A

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Nousari, C.H., Baze, M.R. (2017). Cutaneous Vasculitis: A Clinical Approach. In: Gaspari, A., Tyring, S., Kaplan, D. (eds) Clinical and Basic Immunodermatology. Springer, Cham. https://doi.org/10.1007/978-3-319-29785-9_26

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  • DOI: https://doi.org/10.1007/978-3-319-29785-9_26

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