Expression génitale isolée du syndrome de Stevens-Johnson

Stevens-Johnson syndrome (SJS) is a dermatological and systemic disease. It is a potentially life-threatening condition, almost always triggered by medications, and characterized by extensive skin destruction. It is sometimes associated with the involvement of other epithelia. Its immunological mechanisms are only partially elucidated. Current hypothesis is: delayed-type drug hypersensitivity involving drug-specific cytotoxic T lymphocytes in the blood and skin of patients. Clinically, symptoms occur 1 to 3 weeks after the incriminated drug is started. The patient develops prodromal symptoms including a feeling of discomfort, fever, headache, cough, and keratoconjunctivitis. Macules, often showing a “cockade” pattern, suddenly appear. These lesions appear simultaneously elsewhere on the body, coalesce into large flaccid bullae and slough over a period of 1 to 3 days. Diagnosis is often based on lesions occurrence and rapid progression of symptoms. Histological examination of sloughed skin shows typical necrotic epithelium. Treatment is based on supportive care, cyclosporin, plasmapheresis or IV immunoglobulins, early corticotherapy and TNF (tumor necrosis factor)-alpha inhibitors. Mortality rate is high. The particularity of this case is the isolated genital involvement, 6 days after the intake of a non-steroidal anti-inflammatory drug. Despite prompt management in the Intensive Care Unit, the patient had multi-visceral failure. He died due to refractory shock.