Dermatologic Manifestations of Urticarial Vasculitis Workup

  • Author: Darius Mehregan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 28, 2012
 

Laboratory Studies

Check CH50, C3, C4, Clq, and antibodies to Clq in urticarial vasculitis patients. If these test results are positive, evaluate renal function and urinalysis to check for the effects of vasculitis on the kidneys.

If the history suggests viral infections, obtain hepatitis B, hepatitis C, and heterophile antibody serologies.

Direct immunofluorescence may show deposition of vascular C3, fibrin, and immunoglobulins. A lupus band may be detected in patients with underlying lupus erythematosus.

If warranted, obtain antinuclear antibody and lupus serologies. Anti-SSA and anti-SSB may be seen in patients with Sjögren syndrome. Test results for antineutrophilic cytoplasmic antibodies are generally negative, and, if they are positive, the possibility of Wegener granulomatosis or microscopic polyangiitis should be considered.

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Imaging Studies

Obtain chest x-ray films for urticarial vasculitis patients with hypocomplementemia and pulmonary symptoms.

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Other Tests

If the patient is hypocomplementemic and has pulmonary symptoms, consider ordering pulmonary function tests.

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Procedures

Perform skin biopsy to confirm the diagnosis of urticarial vasculitis. Recent lesions, less than 48 hours in onset, are the best for biopsy. Biopsy of a lesion of less than 24 hours' duration is best for direct immunofluorescence.

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Histologic Findings

On biopsy, histologic findings are those of a leukocytoclastic vasculitis, defined as damage to the small vessels in the papillary and reticular dermis (see images below).[13]

A low-power histologic image of urticarial vasculiA low-power histologic image of urticarial vasculitis shows leukocytoclastic vasculitis with damage to the vessel wall and a neutrophilic infiltrate. A high-power view of the histology of urticarial vA high-power view of the histology of urticarial vasculitis shows extensive fibrin deposition in the vessel walls. Surrounding the vessels is a mixed infiltrate predominately composed of neutrophils with leukocytoclasis.

Early lesions show a perivascular neutrophilic infiltrate involving postcapillary venules. Leukocytoclasis is present, expansion of the vessel wall occurs, and the endothelium is intact. Eosinophils may be noted early. Fibrin deposition and extravasation of red blood cells ensue.

Later in the lesion's course, infiltrate may become a mixture of lymphocytes and neutrophils. Consider performing direct immunofluorescence on the skin biopsy, which may show deposition of complement and fibrin in the blood vessels and, occasionally, immunoglobulin M, immunoglobulin G, and immunoglobulin A along the basement membrane zone of the skin.

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Contributor Information and Disclosures
Author

Darius Mehregan, MD  Associate Professor, Hermann Pinkus Chairman of Dermatology, Department of Dermatology, Wayne State University; Clinical Associate Professor of Pathology, University of Toledo; Dermatopathologist, Pinkus Laboratory; Consulting Staff, Department of Dermatology, J Dingell Veterans Affairs Medical Center

Darius Mehregan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, International Society of Dermatology, International Society of Dermatopathology, Phi Beta Kappa, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Jennifer Michelle Heyl, MD  Resident Physician, Department of Dermatology, Wayne State University School of Medicine

Disclosure: Nothing to disclose.

Iltefat Hamzavi, MD  Staff Physician, Department of Dermatology, Wayne State University School of Medicine, Michigan

Iltefat Hamzavi, MD is a member of the following medical societies: American Academy of Dermatology and American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Gregory J Raugi, MD, PhD  Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

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

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, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

References

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Raised erythematous wheals with postinflammatory hyperpigmentations suggest urticarial vasculitis.
A low-power histologic image of urticarial vasculitis shows leukocytoclastic vasculitis with damage to the vessel wall and a neutrophilic infiltrate.
A high-power view of the histology of urticarial vasculitis shows extensive fibrin deposition in the vessel walls. Surrounding the vessels is a mixed infiltrate predominately composed of neutrophils with leukocytoclasis.
 
 
 
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