paid access
Hypocomplementemic urticarial vasculitis syndrome — clinical presentation, diagnosis and treatment
Affiliations- Department of Dermatology, Institute of Medical Sciences, Medical College of Rzeszow University
paid access
Abstract
Hypocomplementemic urticarial vasculitis syndrome (HVUS) belongs to the group of systemic small-vessel vasculitis and is characterized by persistent urticarial lesions lasting more than 24 hours, hypocomplementemia and frequent coexistence of organ involvement. Lungs, kidneys, joints, gastrointestinal tract, eyes and central nervous system may be involved in the course of the condition. The diagnosis of HUVS is based on the diagnostic criteria – fulfilment of both major criteria (the presence of chronic urticarial lesions with hypocomplementemia) and at least two minor criteria. HUVS may be associated with other systemic conditions. The diagnosis of the syndrome may also precede the development of systemic lupus erythematosus (SLE) in both the paediatric and adult populations. Due to the rarity of the disease, no randomized clinical trials are assessing the effectiveness of individual therapeutic options, and recommendations for the management of HUVS are based on case reports. Glucocorticosteroids (GCSs) are most commonly used as monotherapy or in combination with cyclosporine A, methotrexate, azathioprine, cyclophosphamide or mycophenolate mofetil. In the paper, the clinical picture, recommended diagnostics and therapeutic options for HUVS are discussed.
Abstract
Hypocomplementemic urticarial vasculitis syndrome (HVUS) belongs to the group of systemic small-vessel vasculitis and is characterized by persistent urticarial lesions lasting more than 24 hours, hypocomplementemia and frequent coexistence of organ involvement. Lungs, kidneys, joints, gastrointestinal tract, eyes and central nervous system may be involved in the course of the condition. The diagnosis of HUVS is based on the diagnostic criteria – fulfilment of both major criteria (the presence of chronic urticarial lesions with hypocomplementemia) and at least two minor criteria. HUVS may be associated with other systemic conditions. The diagnosis of the syndrome may also precede the development of systemic lupus erythematosus (SLE) in both the paediatric and adult populations. Due to the rarity of the disease, no randomized clinical trials are assessing the effectiveness of individual therapeutic options, and recommendations for the management of HUVS are based on case reports. Glucocorticosteroids (GCSs) are most commonly used as monotherapy or in combination with cyclosporine A, methotrexate, azathioprine, cyclophosphamide or mycophenolate mofetil. In the paper, the clinical picture, recommended diagnostics and therapeutic options for HUVS are discussed.
Full Text:
Add to basket: 49.00 PLN
Keywords
urticarial vasculitis; small-vessel vasculitis; HUVS; hypocomplementemia; anti-C1q
About this articleTitle
Hypocomplementemic urticarial vasculitis syndrome — clinical presentation, diagnosis and treatment
Journal
Issue
Article type
Review paper
Pages
4-7
Published online
2021-03-17
Page views
580
Article views/downloads
56
DOI
Bibliographic record
Forum Dermatologicum 2021;7(1):4-7.
Keywords
urticarial vasculitis
small-vessel vasculitis
HUVS
hypocomplementemia
anti-C1q
Authors
Magdalena Żychowska
References (17)- McDuffie FC, Sams WM, Maldonado JE, et al. Hypocomplementemia with cutaneous vasculitis and arthritis. Possible immune complex syndrome. Mayo Clin Proc. 1973; 48(5): 340–348.
- Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. 1992; 26(3 Pt 2): 441–448.
- Schwartz HR, McDuffie FC, Black LF, et al. Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease. Mayo Clin Proc. 1982; 57(4): 231–238.
- Zeiss CR, Burch FX, Marder RJ, et al. A hypocomplementemic vasculitic urticarial syndrome. Report of four new cases and definition of the disease. Am J Med. 1980; 68(6): 867–875.
- Al Musalhi B, Al Kamzari A, Al Kindi F, et al. Clinical Spectrum of Childhood-Onset Hypocomplementemic Urticarial Vasculitis in Oman: A Retrospective Multicenter Study. J Am Acad Dermatol. 2020 [Epub ahead of print].
- Chan RWY, Serpas L, Ni M, et al. Plasma DNA Profile Associated with DNASE1L3 Gene Mutations: Clinical Observations, Relationships to Nuclease Substrate Preference, and In Vivo Correction. Am J Hum Genet. 2020; 107(5): 882–894.
- Al-Mayouf SM, Sunker A, Abdwani R, et al. Loss-of-function variant in DNASE1L3 causes a familial form of systemic lupus erythematosus. Nat Genet. 2011; 43(12): 1186–1188.
- Jachiet M, Flageul B, Deroux A, et al. French Vasculitis Study Group. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients. Arthritis Rheumatol. 2015; 67(2): 527–534.
- Davis MD, Daoud MS, Kirby B, et al. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol. 1998; 38(6 Pt 1): 899–905.
- Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore). 1995; 74(1): 24–41.
- Grotz W, Baba HA, Becker JU, et al. Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge. Dtsch Arztebl Int. 2009; 106(46): 756–763.
- 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; 31(9): e395–e397.
- Fujii M, Kishibe M, Ishida-Yamamoto A. Case of hypocomplementemic urticarial vasculitis with Sjögren's syndrome successfully treated with oral corticosteroid and colchicine. J Dermatol. 2020 [Epub ahead of print].
- Alharbi S, Sanchez-Guerrero J. Successful Treatment of Urticarial Vasculitis in a Patient With Systemic Lupus Erythematosus With Rituximab. Clin Med Insights Arthritis Musculoskelet Disord. 2020; 13: 1179544120967374.
- Navarro-Navarro I, Jiménez-Gallo D, Villegas-Romero I, et al. Use of omalizumab in the treatment of hypocomplementemic urticarial vasculitis. Dermatol Ther. 2020; 33(2): e13237.
- Pérez Codesido S, Rosado Ingelmo A, Gómez de la Fuente E, et al. Successful Treatment of Hypocomplementemic Urticarial Vasculitis With Omalizumab: A Case Report. J Investig Allergol Clin Immunol. 2020; 30(3): 211–212.
- Nishijima C, Hatta N, Inaoki M, et al. Urticarial vasculitis in systemic lupus erythematosus: fair response to prednisolone/dapsone and persistent hypocomplementemia. Eur J Dermatol. 1999; 9(1): 54–56.
