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Stevens-Johnson syndrome (SJS) is a rare, but fatal and acute drug-induced cutaneous reaction which presents dramatic, unforgettable manifestations. Though the characteristic features of the disease are well known generally, many clinicians have not experienced a case of SJS frequently, which may result in delay in diagnosis and treatment of it. The authors report a rare case of SJS, which was initially misdiagnosed as Behcet’s disease, with literature review