Elsevier

Mayo Clinic Proceedings

Volume 89, Issue 11, November 2014, Pages 1515-1524
Mayo Clinic Proceedings

Original article
Incidence of Leukocytoclastic Vasculitis, 1996 to 2010: A Population-Based Study in Olmsted County, Minnesota

https://doi.org/10.1016/j.mayocp.2014.04.015Get rights and content

Abstract

Objective

To determine the population-based incidence of leukocytoclastic vasculitis (LCV).

Patients and Methods

This is a retrospective population-based study of all Olmsted County, Minnesota, residents with a skin biopsy–proven diagnosis of LCV from January 1, 1996, through December 31, 2010.

Results

A total of 84 patients (mean age at diagnosis, 48.3 years) with newly diagnosed skin biopsy–proven LCV (43 women and 41 men) were identified. The incidence rate (age and sex adjusted to the 2000 US white population) was 4.5 per 100,000 person-years (95% CI, 3.5-5.4). The incidence of LCV increased significantly with age at diagnosis (P<.001) and did not differ between female and male patients. Subtypes of LCV were cutaneous small-vessel vasculitis (CSVV), 38 patients (45%); IgA vasculitis, 25 (30%); urticarial vasculitis, 10 (12%); cryoglobulinemic vasculitis, 3 (4%); and antineutrophil cytoplasmic antibody–associated vasculitis, 8 (10%). LCV was idiopathic in 29 of 38 patients with CSVV (76%) and 24 of 25 patients with IgA vasculitis (96%). Thirty-nine of 84 patients (46%) had systemic involvement, with the renal system most commonly involved (17 of 39 [44%]). Twenty-four of 80 patients (30%) with follow-up data available had recurrent disease. Compared with the Minnesota white population, observed survival in the incident LCV cohort was significantly poorer than expected (P<.001), including the subset of patients with idiopathic CSVV (P=.03).

Conclusion

The incidence of LCV was higher than that reported in previously published studies. Idiopathic LCV was more common in our population-based cohort than that described previously. Overall survival was significantly poorer (P<.001) and should be explored further in future studies.

Section snippets

Patients and Methods

The study was approved by both the Mayo Clinic and Olmsted Medical Center institutional review boards, Rochester, MN. We used the Rochester Epidemiology Project (REP) database to retrieve the medical records of all the skin biopsy–proven cases of LCV between January 1, 1996, and December 31, 2010.

The REP was founded in 1966 and is a comprehensive database that links the medical records of Olmsted County, MN, residents.4 It is a computerized index system that contains medical diagnoses that have

Patient Population

Between January 1, 1996, and December 31, 2010, a total of 405 patients were identified from the REP as having a coded diagnosis that included 1 or more of the terms used to conduct the initial query of the REP. After exclusion of patients who did not have skin histopathologic confirmation of the diagnosis or who denied research authorization, the final analysis comprised 84 patients with a new diagnosis of skin biopsy–proven LCV.

The mean age at diagnosis for the 84 incident cases of LCV was

Patient Population and Epidemiology of LCV

Our study may be the first population-based study of histopathologically defined LCV. We found no statistical difference in the incidence of LCV in male and female patients. Chua et al8 reported that female patients outnumbered male patients by 2:1 in their retrospective analysis of cutaneous vasculitis. However, other studies have found that the disorder is more common in men.9 Our study also found a statistically significant increase in the incidence of LCV with increased age at diagnosis (P

Conclusion

Our study found that the incidence of LCV is 45 cases per million, which is higher than what has been previously published in the literature. To our knowledge, this is the first population-based study of LCV, thus adding essential knowledge to our present understanding of this disease entity. Idiopathic LCV was more common in our population-based cohort than that described previously. Systemic involvement was present in nearly one-half of the patients, and approximately 30% of the patients with

Acknowledgments

We thank David F. Fiorentino, MD, Department of Dermatology, Stanford University, and Richard D. Sontheimer, MD, Department of Dermatology, University of Utah, for their helpful comments regarding the design of the study. They received no compensation.

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  • Cited by (0)

    Grant Support: This study was made possible by the Rochester Epidemiology Project (grant number R01-AG034676; Principal Investigators: Walter A. Rocca, MD, MPH, and Barbara P. Yawn, MD, MSc).

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