BACKGROUND Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. Severe central nervous system (CNS) involvement is rare in HSP. CASE CHARACTERISTICS Three children with features of HSP presented with seizures and CNS dysfunction. OBSERVATION All three children had abnormalities on neuroimaging; 2 had complete remission but one was left with severe neurological damage. ...

OBJECTIVES To describe a group of patients treated at our service for Henoch-Schönlein purpura, with emphasis on recurrent and chronic cases, and to compare clinical and demographic characteristics of patients with monocyclic and recurrent disease. METHODS Data on 67 patients who had been treated since disease onset were analyzed. Twelve patients were excluded because they failed to return fo...

In the current report, we describe a patient with moderate nephritis and severe skin Henoch Schönlein purpura (HSP) who has been treated with rituximab. Complete and sustained skin and renal remission resulted from the treatment. Thus, further studies are required to confirm the efficacy of rituximab as first-line treatment in HSP, and it might be an interesting new therapeutic option.

Background Familial mediterranean fever (FMF) is an autoinflammatory disease been considered an autosomal recessive disease. 60% of patients are homozygotes. It can be associated with Henoch-Schönlein purpura and panarteritis nodosa. There is an increase in the diagnosis of heterozygosis form in FMF patients with other rheumatic diseases and it is believed that these genes could modulate the ex...

Henoch-Schönlein purpura (HSP) is an acute smallvessel leucocytoclastic vasculitis. HSP is the most common vasculitis in children, with an incidence of about 10 cases per 100 000 a year. In most series, boys are aff ected more often than girls. Although it can occur at any age, HSP is overwhelmingly a disease of childhood. The mean age of patients is 6 years; 75% of patients are under 8 years o...