Vol 3, No 1 (2017)
Case report
Published online: 2017-02-22

open access

Page views 3669
Article views/downloads 2777
Get Citation

Connect on Social Media

Connect on Social Media

The content of this article is also available in the following languages:

Hypocomplementemic urticarial vasculitis syndrome — a case report

Dominik Samotij, Marta Wojciechowska-Zdrojowy, Adrian Pona, Adam Reich
Forum Dermatologicum 2017;3(1):8-11.

Abstract

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an uncommon small-vessel vasculitis presenting as a chronic urticaria with hypocomplementemia and the presence of anti-C1q autoantibodies. In most patients it is accompanied by a wide variety of extracutaneous manifestations such as arthritis, glomerulonephritis, ocular inflammation and pulmonary disease. We present a case of 49-year-old male with a 3-year history of recurrent urticaria accompanied by arthralgia, myalgia and gastrointestinal symptoms. The additional laboratory assessments revealed the presence of circulating anti-C1q autoantibodies in high titer. Histology of the lesional skin biopsy was consistent with leukocytoclastic vasculitis. The diagnosis of HUVS was confirmed in accordance to the currently adopted criteria. The described case emphasizes the necessity of high clinical suspicion when caring for patients with chronic urticaria and concomitant systemic symptoms.

Full content:

View HTML

References

  1. Gasim A.H., Jennette C. Hypocomplementemic urticarial vasculitis. Orphanet: http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=36412 (Last update: May 2013).
  2. Buck A, Christensen J, McCarty M. Hypocomplementemic urticarial vasculitis syndrome: a case report and literature review. J Clin Aesthet Dermatol. 2012; 5(1): 36–46.
  3. Grotz W, Baba HA, Becker JU, et al. Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge. Dtsch Arztebl Int. 2009; 106(46): 756–763.
  4. 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.
  5. Krause K, Mahamed A, Weller K, et al. Efficacy and safety of canakinumab in urticarial vasculitis: an open-label study. J Allergy Clin Immunol. 2013; 132(3): 751–754.e5.
  6. Kervarrec T, Binois R, Bléchet C, et al. [Hypocomplementaemic urticarial vasculitis with bullous lesions and pericardial involvement]. Ann Dermatol Venereol. 2015; 142(10): 557–562.
  7. Hérault M, Mazet J, Beurey P, et al. [Hypocomplementemic vasculitis treated with dapsone]. Ann Dermatol Venereol. 2010; 137(8-9): 541–545.
  8. Eiser AR, Singh P, Shanies HM. Sustained dapsone-induced remission of hypocomplementemic urticarial vasculitis--a case report. Angiology. 1997; 48(11): 1019–1022.
  9. Nürnberg W, Grabbe J, Czarnetzki BM. Urticarial vasculitis syndrome effectively treated with dapsone and pentoxifylline. Acta Derm Venereol. 1995; 75(1): 54–56.
  10. el Maghraoui A, Abouzahir A, Mahassine F, et al. [McDuffie hypocomplementemic urticarial vasculitis. Two cases and review of the literature]. Rev Med Interne. 2001; 22(1): 70–74.
  11. Sessler R, Hasche G, Olbricht CJ. [Hypocomplementemic urticarial vasculitis syndrome]. Dtsch Med Wochenschr. 2000; 125(34-35): 1003–1006.
  12. Al Mosawi ZS, Al Hermi BE. Hypocomplementemic Urticarial Vasculitis Syndrome in an 8-year-old Boy: A Case Report and Review of Literature. Oman Med J. 2013; 28(4): 275–277.
  13. DeAmicis T, Mofid MZ, Cohen B, et al. Hypocomplementemic urticarial vasculitis: report of a 12-year-old girl with systemic lupus erythematosus. J Am Acad Dermatol. 2002; 47(5 Suppl): S273–S274.