Immunoglobulin A (
IgA) vasculitis (
Henoch-Schonlein purpura (HSP)) is the most common
vasculitis in children. It is characterized by purpuric
rash,
arthritis, gastrointestinal, and/or renal involvement. Spontaneous resolution is the typical outcome. In chronic cutaneous manifestations of
IgA vasculitis,
dapsone seems to show a good effectiveness. Multiple case reports and case series about
dapsone in chronic
IgA vasculitis are available. However, no clear evaluation of its indications, its effectiveness, or its usage guidelines (optimal dosage or
duration of treatment) is available. We reviewed the published cases of
IgA vasculitis treated by
dapsone and compared them with 2 similar cases that we encountered. Seventeen patients (ranging from 22 months old to 16 years old) with severe or persistent clinical signs of
IgA vasculitis were included.
Dapsone showed good results on the resolution of cutaneous lesions but not on renal manifestations. Complications (
methemoglobinemia) were observed on 1 patient. Half of the patients relapsed
after treatment discontinuation. The difference between the time lapse before initiation and the duration of the treatment was not significant.Conclusion: We suggest that
dapsone can have a positive effect in chronic
IgA vasculitis when cutaneous manifestations last more than 6 weeks at the dosage of 1-2 mg/kg once per day during 1 week. What is Known: •
IgA vasculitis or
Henoch-Schonlein purpura is the most common
vasculitis in children and affects mostly small vessels of the skin, kidney, and gastrointestinal tract. It resolves spontaneously in most of the cases. Exceptionally, cutaneous lesions can last several weeks. •
Dapsone is a bacteriostatic antibacterial
sulfonamide drug found to be effective in the treatment of some inflammatory dermatological diseases like
IgA vasculitis. What is New: •
Dapsone is effective against chronic purpuric lesion (> 6 weeks) at the minimal dose of 1 mg/kg/day. • Relapse occurs frequently after discontinuation but responds after a second course of treatment. A longer
duration of treatment or a delay in treatment by
dapsone does not seem to influence the relapse rate.