Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 6 3 2021 10 06 90 93 Kamran Balighi Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. Maryam Daneshpazhooh Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. Hamidreza Mahmoudi Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. Safoura Shakoei Department of Dermatology, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran. Zeinab Aryanian Autoimmune Bullous Disease Research Centre, Tehran University of Medical Sciences, Tehran, Iran. Soheil Tavakolpour Dana-Farber Cancer Insttute, Harvard Medical School, Boston, MA, United States. Arghavan Azizpour Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. 2020 07 14 2020 08 04 Acquired Hemophilia (AHA) is a relatively rare disease that occurs in patients with no previous family history of hemophilia. The spontaneous development of autoantibodies (IgG1 and IgG4) against factor VIII has been reported as the most probable cause of AHA. AHA has been reported in association with other conditions, including some autoimmune bullous skin diseases, such as bullous pemphigoid, pemphigus vulgaris, and pemphigus foliaceous. To the best of our knowledge, only 21 cases of AHA with skin autoimmune bullous diseases have been reported so far. Herein, we report a 63-year-old male with a previous history of pemphigus vulgaris who developed large ecchymotic areas on his lower abdomen and forearms after the second infusion of rituximab. Based on coagulation factors evaluation, he was diagnosed with AHA. Treatment with factor VII led to the improvement in his coagulation status, but unfortunately, he passed away because of inferior wall myocardial infarction four days later. https://crcp.tums.ac.ir/index.php/crcp/article/view/379 https://crcp.tums.ac.ir/index.php/crcp/article/download/379/408