Clinicopathologic profile of normocomplementemic and hypocomplementemic urticarial vasculitis: a study from South India

J Eur Acad Dermatol Venereol. 2008 Jul;22(7):789-94. doi: 10.1111/j.1468-3083.2007.02641.x. Epub 2008 Mar 7.


Background: This study aims to study the clinical and histopathological characteristics of hypocomplementemic and normocomplementemic urticarial vasculitis (HUVS and NUV) among dermatology clinic attendees in a tertiary care hospital in South India.

Patients and methods: A prospective study was conducted in the dermatology department from February 2003 to May 2004. Seventy-five patients met the inclusion criteria for UV. Sixty-eight patients in whom complement levels were available were classified into either NUV or HUVS groups. Clinical features, laboratory parameters and histological features were compared, and the significance of differences was established using Pearson's Chi-squared test.

Results: There was a female preponderance among patients with HUVS. Wheals > 24 h were seen in 90% of patients, and in 54.4% of patients, the wheals were partially blanching or non-blanching. Angioedema was more prevalent in patients with NUV than HUVS (44.4% vs. 21.4%). Systemic involvement was seen in 64.3% of patients with HUVS and 44.4% of patients with NUV. Fever, ANA positivity and systemic lupus erythematosus (SLE) were significantly associated with HUVS. In most cases of UV, a provoking factor could not be identified. Neutrophilic small vessel vasculitis was seen in 42.9% of patients with HUVS and 16.6% patients with NUV. Direct immunofluorescence test showing immunoreactants at the dermo-epidermal junction were present in 60% of patients with HUVS and 33.3% patients with NUV.

Conclusion: The clinical features of Indian patients with UV were similar to those reported from the West. Fever, ANA positivity and SLE were significantly associated with HUVS.

MeSH terms

  • Adolescent
  • Adult
  • Arthritis, Juvenile / epidemiology
  • Biopsy
  • Complement C1q / deficiency
  • Complement C1q / metabolism
  • Complement C3 / deficiency
  • Complement C3 / metabolism
  • Complement C4 / deficiency
  • Complement C4 / metabolism
  • Complement System Proteins / deficiency*
  • Complement System Proteins / metabolism*
  • Dermis / blood supply
  • Dermis / pathology
  • Epidermis / pathology
  • Female
  • Fluorescent Antibody Technique, Direct
  • Humans
  • India / epidemiology
  • Infections / epidemiology
  • Lupus Erythematosus, Systemic / epidemiology*
  • Male
  • Middle Aged
  • Mixed Connective Tissue Disease / epidemiology
  • Neoplasms / epidemiology
  • Prevalence
  • Prospective Studies
  • Urticaria / blood
  • Urticaria / epidemiology*
  • Urticaria / pathology
  • Vasculitis / blood
  • Vasculitis / epidemiology*


  • Complement C3
  • Complement C4
  • Complement C1q
  • Complement System Proteins