eMedicine Specialties > Rheumatology > Vasculitis

Leukocytoclastic Vasculitis: Differential Diagnoses & Workup

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

Differential Diagnoses

Amyloidosis, AA (Inflammatory)
Immune Thrombocytopenic Purpura
Antiphospholipid Syndrome
Meningococcemia
Atrial Myxoma
Scurvy
Behcet Disease
Wegener Granulomatosis

Other Problems to Be Considered

Hypersensitivity vasculitis
Idiopathic thrombocytopenia purpura
Polyangiitis overlap syndrome
Thrombocytopenia

Workup

Laboratory Studies

  • The evaluation of the patient with vasculitis serves two purposes. The first is determining the presence of systemic disease. The second is identifying an associated disorder, which aids in the prediction of the patient's prognosis.
  • Although no routine has been established, testing for leukocytoclastic vasculitis (LCV) in all adult patients includes complete blood cell count, erythrocyte sedimentation rate, urinalysis, and blood chemistry panel.
  • Although stool guaiac testing and Hematest are unreliable, such tests should be obtained in patients with cutaneous vasculitis.
  • Serologic studies, such as antinuclear antibody, ANCA (cytoplasmic ANCA [cANCA], perinuclear ANCA [pANCA], atypical ANCA), and rheumatoid factor, should be obtained in patients without an obvious cause of their disease. A recent study has linked IgA-type antiphospholipid antibodies with Henoch Schönlein purpura in adult patients.2
  • In patients with suspected lupus erythematosus and patients with urticarial vasculitis, complement levels may be obtained, including total hemolytic complement (CH100 or CH50), C3 levels, and C4 levels.
  • In patients without otherwise identified disease, tests for paraproteins should include serum protein electrophoresis, cryoglobulins, and hepatitis C antibody.
    • Hepatitis B was associated with vasculitis in the past; however, it appears that the association may have occurred by virtue of coinfection with hepatitis C (previously termed non-A/non-B hepatitis).
    • Cryoglobulins may be present in patients with leukocytoclastic vasculitis, especially in association with infections (hepatitis C, bacterial endocarditis). Results for rheumatoid factor are often positive in patients with cryoglobulinemia.
  • Patients with fever and/or a heart murmur should undergo cardiac ultrasonography and blood cultures.
  • HIV testing should be performed in patients at high risk and in patients with an otherwise unidentifiable cause of leukocytoclastic vasculitis.
  • If the peripheral smear result is abnormal, obtaining bone marrow may be useful.
  • Whether patients with cutaneous vasculitis need to be tested for malignancy is controversial. In general, the author believes that such testing should be performed only in the presence of suggestive symptoms or findings.3

Imaging Studies

  • Chest radiography is part of the routine evaluation for leukocytoclastic vasculitis.
  • Visceral angiography may be obtained in patients with a severe vasculitic syndrome.

Other Tests

  • Pulmonary function tests should be obtained in patients with hypocomplementemic urticarial vasculitis.

Procedures

  • A skin biopsy of a relatively fresh lesion should be performed in most, if not all, adult patients with suspected leukocytoclastic vasculitis. Children with suspected vasculitis are often not forced to endure biopsy for humanitarian reasons.
  • Muscle or nerve biopsy or biopsy of visceral organs may be performed in patients with severe vasculitic syndromes; however, most patients with leukocytoclastic vasculitis of the skin do not require such tests.
  • Direct immunofluorescence microscopy may be obtained in selected patients. IgA results are positive in patients with Henoch-Schönlein purpura.

Histologic Findings

Skin biopsy reveals the presence of vascular and perivascular infiltration of polymorphonuclear leukocytes with formation of nuclear dust (leukocytoclasis), extravasation of erythrocytes, and fibrinoid necrosis of the vessel walls. This process is dynamic, and biopsy of a lesion performed too early or too late in its evolution may not reveal these findings. The presence of eosinophils has been correlated with drug-associated disease.

The picture of leukocytoclastic vasculitis is a pattern that can occur in any vasculitic syndrome but that may also occur in nonvasculitic diseases such as neutrophilic dermatoses, at the base of leg ulceration, or in some insect bite-reactions. Careful clinical-pathologic correlation is necessary.

Immunofluorescent staining may reveal immunoglobulins (eg, immunoglobulin G, immunoglobulin M) and complement components (eg, C3, C4) deposited on the skin basement membrane, suggesting immune complex deposition. In Henoch-Schönlein purpura, IgA deposits may be found.

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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