후천성 혈우병이 동반된 스티븐존슨증후군 1예

Autoimmune diseases have been observed in patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN); however, acquired hemophilia, which can develop owing to autoimmune diseases, has not yet been reported SJS/TEN patients. A 74-year-old male patient, who had been treated for SJS after allopurinol exposure, was referred to our clinic due to hypotension and mucosal erosions. He was suspected to have septic shock due to widespread mucosal involvement caused by SJS, which improved after 1 week of intensive conservative treatment that included antibiotics and systemic corticosteroids. However, a rapid increase in prothrombin time (>60 seconds) and activated partial thromboplastin time (>120 seconds) was confirmed, and the mixture of the 2 materials did not improve decreased factor VIII activity (18.7%). The patient was diagnosed with acquired hemophilia, which was probably associated with SJS. In this case, acquired hemophilia, a fatal and rare autoimmune disease, occurred concurrently with SJS. KCI Citation Count: 0