eMedicine Specialties > Rheumatology > Vasculitis

Leukocytoclastic Vasculitis

Author: Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Contributor Information and Disclosures

Updated: Jan 15, 2009

Introduction

Background

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

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.

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.

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 occurs in 10-30 persons per million persons per year. Fourteen cases of Henoch-Schönlein purpura per million persons per year have been reported.

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.

Clinical

History

  • Patients with cutaneous vasculitis may experience itching, burning, or pain, or they may have asymptomatic lesions.
  • Cutaneous vasculitis may occur in the absence of any systemic disease.
  • Cutaneous vasculitis may manifest as an eruption only, or it may occur in conjunction with collagen-vascular disorders, paraproteinemia, certain foods, oral or parenteral medications, infections, or, rarely, malignancy.
  • The physician should elicit information from the patient about possible systemic manifestations. Questions should be directed at evaluating for fever, arthralgia, arthritis, myalgia, abdominal pain, diarrhea, hematochezia, cough, hemoptysis, sinusitis, paresthesia, weakness, and hematuria.
  • Information should be obtained about symptoms of an associated disorder. Questions should be aimed at determining the history of intravenous drug use, history of hepatitis, history of a transfusion, travel history, symptoms or history of inflammatory bowel disease, and history or symptoms of a collagen-vascular disorder, particularly rheumatoid arthritis, lupus erythematosus, or Sjögren syndrome.

Physical

The most common manifestation of cutaneous vasculitis is palpable purpura, but other manifestations may develop.

  • Palpable purpura is the most common presentation of small-vessel vasculitis.
    • Lesions are usually round and 1-3 mm.
    • They may coalesce to form plaques; in some instances, they may ulcerate.
    • Palpable purpura is most common on the legs, but any surface can be involved. In some cases, the purpuric lesions are barely palpable.
  • Urticarial lesions may develop in some patients with leukocytoclastic vasculitis (LCV); in rare cases, this type of lesion predates the purpuric lesions.
    • The urticarial lesions are of a different character than typical urticaria. They tend to be of longer duration (often >24 h) and tend to resolve with some residual pigmentation or ecchymosis. Patients experience more burning than itching.
    • To determine the duration of individual lesions, the examiner may encircle several lesions and ask the patient to periodically observe them and note when they resolve or move to another site.
  • Patients with hypocomplementemic urticarial vasculitis may develop chronic obstructive pulmonary disease, and a careful examination of the heart and lungs is warranted.
  • Livedo reticularis is a rare manifestation of small-vessel vasculitis. It is more common in patients with occlusive or inflammatory disease of medium-sized vessels.
  • Nodular lesions may develop in some patients with small-vessel vasculitis.
  • Ulceration is more common in vasculitis that affects larger vessels, but it may complicate intense purpura.
  • A careful physical examination is warranted in patients with vasculitis and should include specific observation of the cardiopulmonary, musculoskeletal, and gastrointestinal systems.
  • Retiform purpura has been described by Piette and Stone and has been linked to immunoglobulin A (IgA)–associated disease.1

Causes

  • Between one third and one half of the cases of cutaneous vasculitis are idiopathic, and the remainder have various identifiable causes.
  • The most common drugs that can cause cutaneous vasculitis are antibiotics, particularly beta-lactam drugs, nonsteroidal anti-inflammatory drugs, and diuretics. However, almost all drugs are potential causes. Foreign proteins such as streptokinase, those found in vaccines, and those used in monoclonal antibody therapy can be associated with a serum sickness syndrome with leukocytoclastic vasculitis.
  • Various infections may be associated with vasculitis.
    • Upper respiratory tract infections, particularly with beta-hemolytic streptococci, and viral hepatitis are implicated most often.
    • HIV infection is also associated with some cases of cutaneous vasculitis.
    • Leukocytoclastic vasculitis may also be seen with bacterial endocarditis.
    • Occasionally, ascertaining whether a drug (eg, antibiotic) or an infection (eg, upper respiratory tract infection) is responsible for leukocytoclastic vasculitis is impossible because the occurrence of the vasculitis postdates the infection and the drug used to treat the infection.
  • Foods or food additives may cause vasculitis.
  • Hepatitis C is a regularly recognized cause of vasculitis, probably through the presence of cryoglobulins; however, in the past, hepatitis B was implicated in some cases of vasculitis.
  • Collagen-vascular diseases account for 10-15% of vasculitis cases.
    • In particular, rheumatoid arthritis, Sjögren syndrome, and lupus erythematosus may have an associated vasculitis.
    • In many cases, the presence of vasculitis denotes active disease.
  • Inflammatory bowel disease, ulcerative colitis, and Crohn disease may be associated with cutaneous vasculitis.
  • Malignancy accounts for less than 1% of cases of cutaneous vasculitis.
    • Perhaps lymphoproliferative diseases are more common, particularly hairy cell leukemia; however, any type of tumor at any site may be related to cutaneous vasculitis.
    • Effective tumor therapy has led to an apparent cure of the vasculitis in some patients.
  • Cutaneous vasculitis may be part of a larger-vessel vasculitis such as Wegener granulomatosis, polyarteritis nodosa, microscopic polyarteritis, or Churg-Strauss syndrome.

More on Leukocytoclastic Vasculitis

Overview: Leukocytoclastic Vasculitis
Differential Diagnoses & Workup: Leukocytoclastic Vasculitis
Treatment & Medication: Leukocytoclastic Vasculitis
Follow-up: Leukocytoclastic Vasculitis
Multimedia: Leukocytoclastic Vasculitis
References
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References

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Further Reading

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Keywords

leukocytoclastic vasculitis, LCV, hypersensitivity vasculitis, allergic angiitis, small-vessel vasculitis, Henoch-Schonlein purpura, Henoch-Schönlein purpura, serum sickness, serum sickness syndrome, urticarial vasculitis, upper respiratory tract infections, beta-hemolytic streptococci, viral hepatitis, HIV, bacterial endocarditis, hepatitis C, hepatitis B, collagen vascular disease, rheumatoid arthritis, Sjögren syndrome, lupus erythematosus, inflammatory bowel disease, ulcerative colitis, Crohn disease, hairy cell leukemia, cutaneous vasculitis, Wegener granulomatosis, polyarteritis nodosa, microscopic polyarteritis, Churg-Strauss syndrome, erythema elevatum diutinum, hypocomplementemic urticarial vasculitis

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Contributor Information and Disclosures

Author

Jeffrey P Callen, MD, Professor of Medicine, 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; Genetech Honoraria Consulting; Celgene Honoraria Consulting

Medical Editor

Bryan L Martin, DO, Chief, Allergy Immunology Department, Walter Reed Army Medical Center; Associate Professor of Medicine and Pediatrics, Uniformed Services University of the Health Sciences; United States Army Consultant in Allergy Immunology and Immunizations
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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, 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 of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, 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, Professor of Medicine, Temple University 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: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; Merck, Amgen, Biogen, Zimmer, Wyeth, Johnson&Johnson, Stryker, Medtronic, Zimmer.Abbott,  Ownership interest Other; West Penn Allegheny Health System Consulting fee Consulting; Alpharma Honoraria Consulting; Proctor&Gamble Grant/research funds Independent contractor

 
 
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