Primary vasculitis in children: a clinical - epidemiological study

Resumen

Abstract

Introduction. Classifi cation of primary vasculitis is diffi cult and little is known about prevalence in childhood.

Objective. To determine frequency, epidemiological data and clinical features of primary vasculitis in pediatric patients between May 2000 and May 2008.

Design. Observational, retrospective and descriptive study.

Materials and methods. We reviewed medical record data base of primary vasculitis in pediatric patients of between 0 and 16 years of age that met the established inclusion criteria.

Results. 47 patients, 29 boys and 18 girls were included. The average presentation age was 4 years (range: 7 months to 13 years). The most frequent vasculitis was Henoch Schönlein purpura with 33 cases (70 percent), followed by Kawasaki disease with 9 patients (19 percent), acute hemorrhagic edema of infancy with 3 cases (6 percent) and cutaneous panarteritis nodosa and Churg-Strauss syndrome with 1 case each (2 percent).

Conclusions. Primary vasculitis is rare in childhood. Henoch Schönlein purpura was most frequent in our patients. Kawasaki disease was the vasculitis with highest morbidity. Acute hemorrhagic edema of infancy was less frequent, showed less morbidity and appeared in children under two years of age. We also identifi ed 2 cases of lower frequency vasculitis such as cutaneous panarteritis nodosa and Churg-Strauss syndrome

(Dermatol Argent 2009; 15(6):411-419).

Key words: vasculitis, vasculitides, childhood, children.