Stevens-Johnson syndrome developing in a girl with systemic lupus erythematosus on high-dose corticosteroid therapy

Stevens-Johnson syndrome (SJS) is a vesiculobullous disease of the skin and mucosa. This generalized hypersensitivity reaction is well known to occur in association with certain drugs, particularly sulfonamides, nonsteroidal anti-inflammatory agents (NSAIDs), and anticonvulsants. The disease is usually seen in children and young adults and is often treated with corticosteroids. We present a 9-year-old girl with lupus nephritis who developed cutaneous bullae and mucositis while being treated with intravenous methylprednisolone. The initial differential diagnosis included bullous lupus, but skin biopsy specimen findings supported a diagnosis of SJS. She was treated with intravenous immunoglobulin (IVIg).