摘要：Henoch-Schonlein Purpura (HSP) is a systemic IgA-mediated vasculitis of small vessels common in children. The natural history of HSP is generally self-limiting, however, one third of patients experience recurrence of symptoms showing refractory courses. Our objective was to generate a systematic review of the use of dapsone in refractory HSP. A literature search of PubMed and MEDLINE databases was performed and 13 articles were finally identified published until June 14, 2018. The most common clinical feature was a palpable rash which was seen in all patients (100%) and joint pain was the following (69.2%). Treatment response within 1-2 days was observed in 6 of the 26 (23.1%) patients and 17 (65.4%) patients responded within 3 to 7 days. Relapse after discontinuation of treatment was reported in 17 patients (65.4%), whereas 3 patients (11.5%) reported no relapse of symptoms. Four of the 26 (15.4%) patients reported adverse effects of dapsone. The adverse effects included arthralgia (7.7%), rash (7.7%) and dapsone hypersensitivity syndrome (3.8%). This systematic study suggests that dapsone has an effect on refractory HSP. To determine the standard dosage of dapsone at initial treatment or tapering of treatment in HSP patients and evaluate whether dapsone has a significant benefit compared with steroids or other medication, multicenter, randomized placebo controlled clinical trials are necessary.
