Dermatologic Manifestations of Urticarial Vasculitis

  • Author: Darius Mehregan, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Mar 31, 2016
 

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]

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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. Many patients ultimately prove to have SLE. Other etiologies include drug reactions and parasitic infections.[7]

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Epidemiology

Frequency

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

Leukocytoclastic vasculitis displayed an incidence of 45 cases per million population from a study conducted in Olmsted County, Minnesota.[8]

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.

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.

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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 School of Medicine; Clinical Associate Professor of Pathology, University of Toledo College of Medicine; Dermatopathologist, Pinkus Dermatopathology 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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

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

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

Disclosure: Nothing to disclose.

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

Disclosure: Nothing to disclose.

Rahil M Dharia Wayne State University School of Medicine

Rahil M Dharia is a member of the following medical societies: American Association of Physicians of Indian Origin, American Medical Association, American Medical Student Association/Foundation, Association of Students for Hinduism Awareness, Michigan Association of Physicians of Indian Heritage

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

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, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

References
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  2. Weldon D. When your patients are itching to see you: not all hives are urticaria. Allergy Asthma Proc. 2005 Jan-Feb. 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. 2003 Nov. 49(5 Suppl):S283-5. [Medline].

  4. Venzor J, Lee WL, Huston DP. Urticarial vasculitis. Clin Rev Allergy Immunol. 2002 Oct. 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. 1995 Sep. 22(9):1669-73. [Medline].

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

  7. Shaigany S, Dabela E, Teich AF, Husain S, Grossman ME. Resolution of urticarial vasculitis after treatment of neurocysticercosis. J Am Acad Dermatol. 2015 Jan. 72(1):e32-3. [Medline].

  8. Arora A, Wetter DA, Gonzalez-Santiago TM, Davis MD, Lohse CM. Incidence of leukocytoclastic vasculitis, 1996 to 2010: a population-based study in Olmsted County, Minnesota. Mayo Clin Proc. 2014 Nov. 89 (11):1515-24. [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. 1998 Aug. 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. 1998 Jun. 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. 1992 Mar. 26(3 Pt 2):441-8. [Medline].

  14. Jachiet M, Flageul B, Deroux A, Le Quellec A, Maurier F, Cordoliani F, et al. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients. Arthritis Rheumatol. 2015 Feb. 67(2):527-34. [Medline].

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

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

  17. Jara LJ, Navarro C, Medina G, Vera-Lastra O, Saavedra MA. Hypocomplementemic urticarial vasculitis syndrome. Curr Rheumatol Rep. 2009 Dec. 11(6):410-5. [Medline].

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

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

 
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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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