eMedicine Specialties > Dermatology > Diseases of the Vessels

Urticarial Vasculitis

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, J Dingell Veterans Affairs Medical Center
Coauthor(s): Jennifer Michelle Heyl, MD, Staff Physician, Department of Dermatology, Wayne State University School of Medicine; Iltefat Hamzavi, MD, Staff Physician, Department of Dermatology, Wayne State University School of Medicine, Michigan
Contributor Information and Disclosures

Updated: Aug 6, 2009

Introduction

Background

Urticarial vasculitis is an eruption of erythematous wheals that clinically resemble urticaria but histologically show changes of leukocytoclastic vasculitis.1,2 Urticarial vasculitis may be divided into normocomplementemic and hypocomplementemic variants. Both subsets can be associated with systemic symptoms (eg, angioedema, arthralgias, abdominal or chest pain, fever, pulmonary disease, renal disease, episcleritis, uveitis, and Raynaud phenomenon). The hypocomplementemic form more often is associated with systemic symptoms and has been linked to connective-tissue disease (ie, systemic lupus erythematosus [SLE]).3,4

Pathophysiology

The pathophysiology of urticarial vasculitis is similar to other forms of cutaneous small vessel leukocytoclastic vasculitis. Urticarial vasculitis is a type III hypersensitivity reaction in which antigen-antibody complexes are deposited in the vascular lumina. This reaction results in complement activation and chemotaxis of neutrophils. These cells release various proteolytic enzymes, such as collagenase and elastase, resulting in damage to the vascular lumina. Some authors have speculated that eosinophils may be involved in the early stages of the vasculitic lesions. Patients with hypocomplementemic urticarial vasculitis are more likely to show autoantibodies to C1q and vascular endothelial cells.5,6 The presence of antineutrophilic cytoplasmic antibodies is rare.

Frequency

United States

The exact frequency of urticarial vasculitis is not known in the United States or worldwide.

International

Previous studies varied in their definitions of the condition. However, when a study in the United Kingdom used consistent criteria restricted to patients diagnosed with vasculitis by biopsy and with urticarial lesions of more than 3 months duration, 2.1% of 1310 patients with urticaria were found to have urticarial vasculitis.

Mortality/Morbidity

Urticarial vasculitis carries a good prognosis, with most occurrences resolving in months to years. Urticarial vasculitis associated with hypocomplementemia is associated with a greater incidence of coexisting disease (ie, angioedema, connective-tissue disease [primarily SLE], chronic obstructive pulmonary disease). Mortality is rare.

Sex

The male-to-female ratio for urticarial vasculitis is 1:2.

Age

The median age of urticarial vasculitis involvement is 43 years, with a range of 15-90 years. While urticarial vasculitis is primarily a disease of middle-aged adults, it can be seen in persons of any age.

Clinical

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
  • 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.7  
    • 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.8
    • Viral disease, such as hepatitis B, hepatitis C,9 and infectious mononucleosis
  • Most cases of urticarial vasculitis are idiopathic.
  • Urticarial vasculitis is divided into hypocomplementemic and normocomplementemic categories.10
    • 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.

Physical

Lesions of urticarial vasculitis initially appear as erythematous wheals (see Media File 1). 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 ...

Raised erythematous wheals with postinflammatory hyperpigmentations suggest urticarial vasculitis.

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Raised erythematous wheals with postinflammatory ...

Raised erythematous wheals with postinflammatory hyperpigmentations suggest urticarial vasculitis.


Causes

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

More on Urticarial Vasculitis

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

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  2. Weldon D. When your patients are itching to see you: not all hives are urticaria. Allergy Asthma Proc. Jan-Feb 2005;26(1):1-7. [Medline].

  3. Saigal K, Valencia IC, Cohen J, Kerdel FA. Hypocomplementemic urticarial vasculitis with angioedema, a rare presentation of systemic lupus erythematosus: rapid response to rituximab. J Am Acad Dermatol. Nov 2003;49(5 Suppl):S283-5. [Medline].

  4. Venzor J, Lee WL, Huston DP. Urticarial vasculitis. Clin Rev Allergy Immunol. Oct 2002;23(2):201-16. [Medline].

  5. D'Cruz DP, Wisnieski JJ, Asherson RA, Khamashta MA, Hughes GR. Autoantibodies in systemic lupus erythematosus and urticarial vasculitis. J Rheumatol. Sep 1995;22(9):1669-73. [Medline].

  6. Kallenberg CG. Anti-C1q autoantibodies. Autoimmun Rev. Sep 2008;7(8):612-5. [Medline].

  7. 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].

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

  9. 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].

  10. 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].

  11. 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].

  12. [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].

  13. 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].

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

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

  16. 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].

  17. 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].

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  19. 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].

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

  21. 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].

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

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Further Reading

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Keywords

urticarial vasculitis, normocomplementemic urticarial vasculitis, hypocomplementemic urticarial vasculitis, wheals, hives, allergic reaction, hypersensitivity reaction, systemic lupus erythematosus, SLE, connective tissue disease, connective-tissue disease, vasculitis

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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, 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, Staff 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.

Medical Editor

Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; 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.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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.

Managing Editor

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; Medicis Honoraria Consulting; Celgene Honoraria Consulting

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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