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Leukocytoclastic Vasculitis

  • Author: A Brooke W Eastham, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Jul 05, 2016
 

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 the image below).[1] Histologically, LCV is characterized by leukocytoclasis, which refers to vascular damage caused by nuclear debris from infiltrating neutrophils. LCV classically presents as palpable purpura. Less common clinical findings include urticarial plaques, vesicles, bullae, and pustules.

Histopathology of leukocytoclastic vasculitis. Histopathology of leukocytoclastic vasculitis.

LCV may be secondary to medications, underlying infection, collagen-vascular disorders, or malignancy. However, approximately half of cases are idiopathic.[2, 3]

LCV may be localized to the skin or may be associated with systemic involvement.[4] Internal disease most often manifests in the joints, the gastrointestinal (GI) tract, and the kidneys.

In the absence of internal involvement, the prognosis is excellent, with the majority of cases resolving within weeks to months. Approximately 10% of patients will have chronic or recurrent disease.[5]

LCV may be acute or chronic. Patients with chronic disease may experience persistent lesions or intermittent recurrence. Cases that primarily involve the skin should be treated with nontoxic modalities whenever possible, avoiding the use of systemic corticosteroids and immunosuppressive agents.

Henoch-Schönlein purpura (HSP), a specific subtype of LCV warranting separate discussion, is characterized by predominant IgA-mediated vessel injury. The classic clinical findings of palpable purpura in HSP are often preceded by viral respiratory illness. HSP is more common in children, but can also occur in adults. Children may develop systemic disease with GI, joint, and/or kidney involvement. In adults, arthritis and kidney disease occur more frequently.[6] HSP in adults, especially older men, may be associated with malignancy.[7]

For additional information on HSP, see Henoch-Schönlein purpura.

For additional information on cutaneous manifestations of leukocytoclastic vasculitis, see Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis).

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Pathophysiology

Immune complex deposition, with resultant neutrophil chemotaxis and release of proteolytic enzymes and free oxygen radicals, is a key component in the pathophysiology of LCV.[8, 9] In addition, other autoantibodies, such as antineutrophil cytoplasmic antibody (ANCA); inflammatory mediators, including tumor necrosis factor alpha; and enhanced expression of vascular adhesion molecules may play a role.[10] 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.[11, 12, 13] 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

Patients with LCV generally have a good prognosis, but if the kidneys, GI tract, lungs, heart, or central nervous system are involved, morbidity may increase and mortality can occur.

Cutaneous lesions of LCV are often asymptomatic, but may be associated with pruritus or pain.

Bullous lesions, as well as chronic cutaneous disease, may involve ulceration or painful episodes of purpura, which may cause physical limitations.

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Race-, Sex-, and Age-related Demographics

Leukocytoclastic vasculitis is reported more often in whites than in other races.

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

Leukocytoclastic vasculitis may occur at any age. Henoch-Schönlein purpura is more common in children under 10 years of age.

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Contributor Information and Disclosures
Author

A Brooke W Eastham, MD Board Certified Dermatologist, Nashville Skin and Cancer

A Brooke W Eastham, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Medical Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

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, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Ruth Ann Vleugels, MD, MPH Assistant Professor of Dermatology, Harvard Medical School; Associate Physician, Department of Dermatology, Brigham and Women's Hospital; Associate Physician, Department of Immunology and Allergy, Children's Hospital Boston

Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

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, American Osteopathic Association

Disclosure: Nothing to disclose.

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