Hypersensitivity Vasculitis 

  • Author: Jeffrey P Callen, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 14, 2010
 

Background

Hypersensitivity vasculitis (leukocytoclastic vasculitis) is a histopathologic term commonly used to denote a small vessel vasculitis. Many possible causes exist for hypersensitivity vasculitis, but a cause is not found in as many as 50% of patients.

Hypersensitivity vasculitis (a form of small vessel vasculitis) may manifest clinically as cutaneous disease only or it may manifest as skin disease with involvement of other organs. The internal organs most commonly affected in hypersensitivity vasculitis are the joints, gastrointestinal tract, and the kidneys. The prognosis for hypersensitivity vasculitis is good when no internal involvement is clinically present. Hypersensitivity vasculitis may be acute and self-limited or chronic. Note the image below.

Hypersensitivity vasculitis. Hypersensitivity vasculitis.
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Pathophysiology

Circulating immune complexes play a role in the pathogenesis of hypersensitivity vasculitis.[1] Although immune complexes are involved in the pathogenesis of hypersensitivity vasculitis, other autoantibodies, such as antineutrophil cytoplasmic antibody (ANCA), may be associated with disease manifestations, and other inflammatory mediators, adhesion molecules, and local factors may affect the endothelial cells and play a role in the manifestations of this disease.[2] The exact mechanisms causing hypersensitivity vasculitis remain to be elucidated.

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Epidemiology

Frequency

United States

The incidence of hypersensitivity vasculitis is unknown, but the condition is presumed to be relatively rare.

International

Several studies from Spain have been conducted on hypersensitivity vasculitis.[3, 4, 5, 6, 7] Hypersensitivity vasculitis reportedly has an incidence of 10-30 cases per million people per year. Henoch-Schönlein purpura reportedly has an incidence of 14 cases per million people per year.

Mortality/Morbidity

The prognosis for hypersensitivity vasculitis is generally good, but mortality is possible if the kidneys, the gastrointestinal tract, the lungs, the heart, or the central nervous system is involved. Patients with chronic cutaneous disease may develop ulceration or recurrent painful bouts of purpura. Some hypersensitivity vasculitis patients alter their lives because of recurrent purpuric eruptions or accompanying symptoms.

Race

Hypersensitivity vasculitis is reported most often in the white population, but epidemiologic studies are not available to assess whether hypersensitivity vasculitis is associated with any specific ethnic group or skin type.

Sex

Although hypersensitivity vasculitis appears to affect men and women in approximately equal numbers, some of the studies from Spain suggest that hypersensitivity vasculitis is slightly more common in men than in women.[7]

Age

Hypersensitivity vasculitis may occur at any age. In both adults and children, hypersensitivity vasculitis may appear identical to Henoch-Schönlein purpura. A precise differentiation of hypersensitivity vasculitis from Henoch-Schönlein purpura is not possible in this author's view.

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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; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Medicis Honoraria Consulting; Celgene Honoraria Consulting

Specialty Editor Board

Michelle Pelle, MD  Clinical Assistant Professor, Division of Dermatology, Department of Medicine, University of California at San Diego

Michelle Pelle, MD is a member of the following medical societies: American Academy of Dermatology, California Medical Association, Medical Dermatology Society, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Hypersensitivity vasculitis.
Henoch-Schönlein purpura.
Histopathologic features of leukocytoclastic vasculitis.
Urticarial vasculitis. These lesions differ from routine hives by lasting longer (often >24 h), being less pruritic, and often resolving with a bruise or residual pigmentation.
Erythema elevatum diutinum, a rare cutaneous vasculitis.
 
 
 
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