Dermatologic Manifestations of Urticarial Vasculitis Clinical Presentation

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

History

Patients with urticarial vasculitis present with an urticarial eruption, often accompanied by a painful or burning sensation. Lesions are generalized wheals or erythematous plaques, occasionally with central clearing, lasting for more than 24 hours in a fixed location (in contrast to urticaria, which resolves in minutes to hours or migrates continually). Petechiae may be noted within the lesions, and they may resolve with ecchymoses or postinflammatory hyperpigmentation. Patients may have photosensitivity, lymphadenopathy, arthralgia, angioedema (40%), fever, abdominal pain, dyspnea, and pleural and pericardial effusions.[4] Most cases of urticarial vasculitis are idiopathic.

The primary causes of urticarial vasculitis are as follows:

  • Drug induced, such as ACE inhibitors, penicillin, sulfonamides, fluoxetine, cimetidine, diltiazem, thiazides, potassium iodide, non-steroid inflammatory drugs, and glatiramer acetate.[9]
  • Rheumatic disease, such as SLE and Sjögren syndrome: Urticarial vasculitis has also been reported with immunoglobulin A and immunoglobulin M monoclonal gammopathies, mixed cryoglobulins, and hematologic and solid malignancies.[10]
  • Viral disease, such as hepatitis B, hepatitis C,[11] and infectious mononucleosis

Urticarial vasculitis is divided into hypocomplementemic and normocomplementemic categories, as follows[12] :

  • Hypocomplementemia often is associated with a systemic condition, such as SLE (in which >50% of patients have hypocomplementemia).[3] In addition, as many as 71% of patients with hypocomplementemic urticarial vasculitis have a positive antinuclear antibody titer but do not fulfill the American Rheumatism Association criteria for SLE.[5] Some authors have suggested evaluation of hypocomplementemic urticarial vasculitis for immunoglobulin G antibodies to C1q. Individuals with these antibodies have a higher incidence of angioedema, ocular inflammation, glomerulonephritis, and obstructive pulmonary disease.
  • Normocomplementemic vasculitis can be associated with connective-tissue disease but at a much lower rate.
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Physical

Lesions of urticarial vasculitis initially appear as erythematous wheals (see image below). As the lesions progress, purpura may develop. Often, the urticarial vasculitis lesions resolve with postinflammatory pigmentation. Annular or targetoid lesions may be observed.

Raised erythematous wheals with postinflammatory hRaised erythematous wheals with postinflammatory hyperpigmentations suggest urticarial vasculitis.
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Causes

The etiology of urticarial vasculitis has not been elucidated. Associated conditions are listed in History.

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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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  8. Buck A, Christensen J, McCarty M. Hypocomplementemic urticarial vasculitis syndrome: a case report and literature review. J Clin Aesthet Dermatol. Jan 2012;5(1):36-46. [Medline].

  9. Cicek D, Kandi B, Oguz S, Cobanoglu B, Bulut S, Saral Y. An urticarial vasculitis case induced by glatiramer acetate. J Dermatolog Treat. 2008;19(5):305-7. [Medline].

  10. de Castro FR, Masouye I, Winkelmann RK, Saurat JH. Urticarial pathology in Schnitzler's (hyper-IgM) syndrome. Dermatology. 1996;193(2):94-9. [Medline].

  11. Hamid S, Cruz PD Jr, Lee WM. Urticarial vasculitis caused by hepatitis C virus infection: response to interferon alfa therapy. J Am Acad Dermatol. Aug 1998;39(2 Pt 1):278-80. [Medline].

  12. Davis MD, Daoud MS, Kirby B, Gibson LE, Rogers RS 3rd. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol. Jun 1998;38(6 Pt 1):899-905. [Medline].

  13. Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. Mar 1992;26(3 Pt 2):441-8. [Medline].

  14. [Guideline] American Academy of Allergy, Asthma and Immunology. Consultation and referral guidelines citing the evidence: how the allergist-immunologist can help. J Allergy Clin Immunol. Feb 2006;117(2 Suppl Consultation):S495-523. [Medline].

  15. Worm M, Sterry W, Kolde G. Mycophenolate mofetil is effective for maintenance therapy of hypocomplementaemic urticarial vasculitis. Br J Dermatol. Dec 2000;143(6):1324. [Medline].

  16. Ghadban R, Zenone T, Leveque-Michaud C, Louerat C, Rousset H. [Hypocomplementemic urticarial vasculitis]. Rev Med Interne. Nov 2008;29(11):929-31. [Medline].

  17. Berg RE, Kantor GR, Bergfeld WF. Urticarial vasculitis. Int J Dermatol. Sep 1988;27(7):468-72. [Medline].

  18. Black AK, Lawlor F, Greaves MW. Consensus meeting on the definition of physical urticarias and urticarial vasculitis. Clin Exp Dermatol. Nov 1996;21(6):424-6. [Medline].

  19. Kano Y, Orihara M, Shiohara T. Cellular and molecular dynamics in exercise-induced urticarial vasculitis lesions. Arch Dermatol. Jan 1998;134(1):62-7. [Medline].

  20. Lacy CF, Armstrong LL, Goldman MP. Drug Information Handbook 2000-2001. 8th ed. Hudson, Ohio: Lexi-Comp; 2000.

  21. Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. Nov 1998;39(5 Pt 1):667-87; quiz 688-90. [Medline].

  22. Mehregan DR, Gibson LE. Pathophysiology of urticarial vasculitis. Arch Dermatol. Jan 1998;134(1):88-9. [Medline].

  23. Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore). Jan 1995;74(1):24-41. [Medline].

  24. Wolverton SE, Wilkin JK. Systemic Drugs for Skin Diseases. Philadelphia, Pa: WB Saunders; 1995.

 
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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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