Urticarial Vasculitis

No Results

No Results

processing….

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], relapsing polychondritis). [3, 4, 5, 6, 7]

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. [8, 9] The presence of antineutrophilic cytoplasmic antibodies is rare. Many patients ultimately prove to have SLE. Other etiologies include drug reactions and parasitic infections. [10]

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

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. [11]

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.

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

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.

Urticarial vasculitis tends to become a chronic condition and patients should be educated about its course. For most patients, this is a disease that affects the skin, with a minority of patients developing systemic complications.

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). [12, 13] Mortality is rare. Some cohorts have demonstrated systemic involvement in roughly half of the patients, including musculoskeletal and ocular complications. [14]

Since urticarial vasculitis may be chronic, educate patients about its course. For patient education resources, see the Allergy Center and Skin, Hair, and Nails Center, as well as Hives and Angioedema.

Oi M, Satoh T, Yokozeki H, Nishioka K. Infectious urticaria with purpura: a mild subtype of urticarial vasculitis?. Acta Derm Venereol. 2005. 85(2):167-70. [Medline].

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].

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].

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

Hamad A, Jithpratuck W, Krishnaswamy G. Urticarial vasculitis and associated disorders. Ann Allergy Asthma Immunol. 2017 Apr. 118 (4):394-398. [Medline].

Raboudi A, Litaiem N, Jones M, Zeglaoui F. Hypocomplementemic urticarial vasculitis occurring in a patient with relapsing polychondritis. Int J Dermatol. 2018 May 20. [Medline].

Sjöwall C, Mandl T, Skattum L, Olsson M, Mohammad AJ. Epidemiology of hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis). Rheumatology (Oxford). 2018 Apr 30. [Medline].

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].

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

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].

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].

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].

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].

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].

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].

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

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].

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].

Salim SA, Yousuf T, Patel A, Fülöp T, Agarwal M. Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis. Am J Med Sci. 2018 Feb. 355 (2):195-200. [Medline].

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].

Aurich S, Simon JC, Treudler R. Omalizumab does not improve skin lesions in a patient with hypocomplementemic urticarial vasculitis syndrome. J Eur Acad Dermatol Venereol. 2017 Mar 8. [Medline].

Fueyo-Casado A, Campos-Muñoz L, González-Guerra E, Pedraz-Muñoz J, Cortés-Toro JA, López-Bran E. Effectiveness of omalizumab in a case of urticarial vasculitis. Clin Exp Dermatol. 2017 Mar 1. [Medline].

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].

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

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

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.

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

Disclosure: Nothing to disclose.

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.

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.

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: Lilly; Amgen <br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor; 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: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

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.

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.

Urticarial Vasculitis

Research & References of Urticarial Vasculitis|A&C Accounting And Tax Services
Source