Leukocytoclastic Vasculitis 

  • Author: Jeffrey P Callen, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: May 2, 2012
 

Background

Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis and hypersensitivity angiitis, is a histopathologic term commonly used to denote a small-vessel vasculitis (see image shown below). Leukocytoclastic vasculitis has many causes, but no cause is identified in up to 50% of patients with this condition.

Histopathology of leukocytoclastic vasculitis. Histopathology of leukocytoclastic vasculitis.

Leukocytoclastic vasculitis may be localized to the skin or may manifest in other organs. The internal organs affected most commonly include the joints, the gastrointestinal tract, and the kidneys. The prognosis is good in the absence of internal involvement.

Leukocytoclastic vasculitis may be acute or chronic. Chronic disease that primarily involves the skin should be treated with nontoxic modalities whenever possible, avoiding the use of systemic corticosteroids and immunosuppressive agents.

For additional information on cutaneous manifestations of leukocytoclastic vasculitis, see the eMedicine article Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis) in the Dermatology volume.

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Pathophysiology

Circulating immune complexes were once believed to be the cause of leukocytoclastic vasculitis. Although immune complexes are involved in the pathogenesis of leukocytoclastic vasculitis, other autoantibodies such as antineutrophil cytoplasmic antibody (ANCA), other inflammatory mediators, and local factors that involve the endothelial cells and adhesion molecules play an important role. However, the exact mechanisms remain unknown.

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Epidemiology

Frequency

United States

The incidence of leukocytoclastic vasculitis is unknown, but the disorder is presumed to be uncommon.

International

Several studies on leukocytoclastic vasculitis have been conducted in Spain. Hypersensitivity vasculitis (see first image below) occurs in 10-30 persons per million persons per year. Fourteen cases of Henoch-Schönlein purpura (see second image below) per million persons per year have been reported.

Hypersensitivity vasculitis. Hypersensitivity vasculitis. Henoch-Schönlein purpura. Henoch-Schönlein purpura.

Mortality/Morbidity

  • The prognosis of leukocytoclastic vasculitis is generally good, but if the kidneys, gastrointestinal tract, lungs, heart, or central nervous system is involved, mortality is possible.
  • Chronic cutaneous disease may involve ulceration or painful bouts of purpura. Some patients alter their lives because of recurrent purpuric eruptions.

Race

  • Leukocytoclastic vasculitis appears to be reported more often in whites than in other races.

Sex

  • Leukocytoclastic vasculitis affects men and women in approximately equal proportions. Some of the studies from Spain suggest that leukocytoclastic vasculitis may be slightly more common in men than in women.

Age

  • Leukocytoclastic vasculitis may occur at any age.
  • In children, it may be called Henoch-Schönlein purpura.
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Contributor Information and Disclosures
Author

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Celgene Honoraria Safety Monitoring Committee

Specialty Editor Board

Bryan L Martin, DO  Associate Dean for Graduate Medical Education, Designated Institutional Official, Associate Medical Director, Director, Allergy Immunology Program, Professor of Medicine and Pediatrics, Ohio State University College of Medicine

Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, and American Osteopathic Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

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Hypersensitivity vasculitis.
Henoch-Schönlein purpura.
Histopathology of leukocytoclastic vasculitis.
Urticarial vasculitis. Lesions differ from routine hives in that they last longer (often >24 h), are less pruritic, and often resolve with a bruise or residual pigmentation.
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