Objective To analyze the therapeutic outcomes and adverse effects of high-dose intravenous immunoglobulin (hd-IVIg) in the treatment of some severe skin diseases (toxic epidermal necrolysis, drug hypersensitivity syndrome, connective tissue disease, autoimmune bullous disease, acute graft-versus-host disease). Methods Twenty-five cases of severe skin diseases were treated with hd-IVIg (0.4 g/kg/day for a course of 5 days). Results The therapeutic outcomes were different from each other. Of all the cases, 21 improved, especially those of acute onset of toxic epidermal necrolysis and drug hypersensitivity syndrome; 1 adult dermatomyositis and 2 elder bullous pemphigoid were not relieved. A patient with acute graft-versus-host disease died. Three patients presented with minor adverse effects (headache and blood pressure rising). Conclusions hd-IVIg is effective and safe in the treatment of some severe skin diseases. More importantly, it has a substantial effect on shortening disease course and decreasing the dosage of glucocorticoids and immunosuppressants as well as on preventing infections.