Open AccessAcquired Hemophilia in Association With Pemphigus Vulgaris: An Uncommon Coexistence: A Case Report
Author(s) -
Kamran Balighi,
Maryam Daneshpazhooh,
Hamidreza Mahmoudi,
Safoura Shakoei,
Zeinab Aryanian,
Soheil Tavakolpour,
Arghavan Azizpour
Publication year - 2021
Publication title -
case reports in clinical practice
Language(s) - English
Resource type - Journals
eISSN - 2538-2691
pISSN - 2538-2683
DOI - 10.18502/crcp.v6i3.7124
Subject(s) - pemphigus vulgaris , medicine , pemphigus , bullous pemphigoid , dermatology , rituximab , pemphigus foliaceus , paraneoplastic pemphigus , autoantibody , pemphigoid , family history , immunology , lymphoma , surgery , antibody
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.