Medical Care

Urticarial vasculitis tends to run a chronic course. Mortality is low, unless renal or pulmonary disease occurs. The goal of treatment is to achieve long-term control with the least amount of toxicity.^{[25, 26]}

A complete patient history is the basis for treatment. In the history, ask for time of onset of the lesions; duration of the lesions (eg, >24 h); whether lesions are painful or burning, rather than pruritic; and the history of resolution with purpura or hyperpigmentation. Inquire about the patient's medications, fever, arthralgia, dyspnea, abdominal pain, and symptoms of angioedema. Omalizumab has produced mixed results.^{[27, 28]}

Consultations

Consultation with the following specialists may be needed:

Dermatologist: Skin biopsy is evaluated by a dermatologist/dermatopathologist to confirm the diagnosis.
Rheumatologist: Consult a rheumatologist when SLE is suspected or if the patient has the hypocomplementemic variant with systemic symptoms.
Allergist/immunologist

Long-Term Monitoring

Once a diagnosis of urticarial vasculitis is made, follow-up care depends on the patient's complement levels. If complement level findings are normal, follow patients for symptoms and response to treatment. If complement level findings are low, follow patients for attendant complications related to the cause of their hypocomplementemia (see History).

Medication

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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Venzor J, Lee WL, Huston DP. Urticarial vasculitis. Clin Rev Allergy Immunol. 2002 Oct. 23(2):201-16. [QxMD MEDLINE Link].
Hamad A, Jithpratuck W, Krishnaswamy G. Urticarial vasculitis and associated disorders. Ann Allergy Asthma Immunol. 2017 Apr. 118 (4):394-398. [QxMD MEDLINE Link].
Raboudi A, Litaiem N, Jones M, Zeglaoui F. Hypocomplementemic urticarial vasculitis occurring in a patient with relapsing polychondritis. Int J Dermatol. 2018 May 20. [QxMD MEDLINE Link].
Sjöwall C, Mandl T, Skattum L, Olsson M, Mohammad AJ. Epidemiology of hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis). Rheumatology (Oxford). 2018 Apr 30. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Kallenberg CG. Anti-C1q autoantibodies. Autoimmun Rev. 2008 Sep. 7(8):612-5. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Jara LJ, Navarro C, Medina G, Vera-Lastra O, Saavedra MA. Hypocomplementemic urticarial vasculitis syndrome. Curr Rheumatol Rep. 2009 Dec. 11(6):410-5. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Manappallil RG, Pallivalappil B, Martin AM, Mampilly N, Rao A. Normocomplementemic Urticarial Vasculitis: An Unusual Presentation. Indian J Dermatol. 2020 May-Jun. 65 (3):208-210. [QxMD MEDLINE Link].
de Perosanz-Lobo D, Fernandez-Nieto D, Burgos-Blasco P, Selda-Enriquez G, Carretero I, Moreno C, et al. Urticarial vasculitis in COVID-19 infection: a vasculopathy-related symptom?. J Eur Acad Dermatol Venereol. 2020 Jun 8. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
de Castro FR, Masouye I, Winkelmann RK, Saurat JH. Urticarial pathology in Schnitzler's (hyper-IgM) syndrome. Dermatology. 1996. 193(2):94-9. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Ion O, Obrișcă B, Ismail G, Sorohan B, Bălănică S, Mircescu G, et al. Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome-A Case-Based Review. J Clin Med. 2020 Jul 6. 9 (7):[QxMD MEDLINE Link].
García-García B, Aubán-Pariente J, Munguía-Calzada P, Vivanco B, Argenziano G, Vázquez-López F. Development of a clinical-dermoscopic model for the diagnosis of urticarial vasculitis. Sci Rep. 2020 Apr 8. 10 (1):6092. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Al Kamzari A, Al Musalhi B, Al Abrawi S, Al-Zakwani I, Abdwani R. Urticarial vasculitis in pediatric systemic lupus erythematosus. Pediatr Dermatol. 2020 Jul. 37 (4):651-655. [QxMD MEDLINE Link].
Kolkhir P, Bonnekoh H, Kocatürk E, Hide M, Metz M, Sánchez-Borges M, et al. Management of urticarial vasculitis: A worldwide physician perspective. World Allergy Organ J. 2020 Mar. 13 (3):100107. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Ghadban R, Zenone T, Leveque-Michaud C, Louerat C, Rousset H. [Hypocomplementemic urticarial vasculitis]. Rev Med Interne. 2008 Nov. 29(11):929-31. [QxMD MEDLINE Link].
Mehregan DR, Gibson LE. Pathophysiology of urticarial vasculitis. Arch Dermatol. 1998 Jan. 134(1):88-9. [QxMD MEDLINE Link].

Media Gallery

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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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)

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.

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: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/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 these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

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.

Urticarial Vasculitis Treatment & Management

Medical Care

Consultations

Long-Term Monitoring

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