Leukocytoclastic Vasculitis Clinical Presentation

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

History

  • Patients with cutaneous vasculitis may experience itching, burning, or pain, or they may have asymptomatic lesions. See the image below. Erythema elevatum diutinum, a rare cutaneous vascuErythema elevatum diutinum, a rare cutaneous vasculitis.
  • 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.
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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, as depicted below. Patients experience more burning than itching. Urticarial vasculitis. Lesions differ from routineUrticarial 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.
    • 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]
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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.
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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.
Erythema elevatum diutinum, a rare cutaneous vasculitis.
 
 
 
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