Henoch-Schönlein purpura is the most common childhood vasculitis, but may also affect adults. This article reviews the literature since 2011 on advances in diagnosis, clinical disease manifestations, pathophysiology and treatment of Henoch-Schönlein purpura. The clinical manifestations are thought to arise from IgA depositions in blood vessel walls in the affected organs, mostly skin, gastrointestinal tract, joints and kidneys. Corticosteroids may be effective in rapid resolution of renal manifestations and treating joint and abdominal pain, but they are not proven effective for treating organ manifestations and complications, such as glomerulonephritis, bowel infarction or intussusception. Mycophenolate mofetil or cyclosporine A may be better treatment choices in case of renal involvement. Other immunosuppressive and immunomodulating drugs, such as rituximab and dapsone, are promising, but larger studies are needed to confirm these findings. Cancer screening should be considered in older males diagnosed with Henoch-Schönlein purpura.