title: Coexistence of pemphigus foliaceus and acquired hemophilia A: A case report.
abstract: Pemphigus foliaceus (PF) is an autoimmune bullous dermatosis with anti-desmoglein-1 autoantibodies. Acquired hemophilia A (AHA) is a rare coagulation disorder with a high mortality rate, caused by anti-factor VIII immunoglobulin G antibodies leading to spontaneous severe hemorrhages into skin, muscles or soft tissues. This coagulopathy may be associated with malignancies, drug reactions and autoimmune disorders including bullous dermatoses. Herein, we demonstrate a first report of AHA in the course of pemphigus foliaceus. A 55-year-old woman presenting with extensive, erosive, crusted, scaly skin lesions was diagnosed with PF based on histopathological and immunofluorescent examination, confirmed by the presence of anti-desmoglein-1 antibodies on enzyme-linked immunoassay. She developed extensive internal hemorrhages and prolonged external bleeding after laparotomy. Based on coagulation tests, AHA was diagnosed. Simultaneous remission of pemphigus and coagulopathy occurred with immunosuppressants and recombinant activated factor VII.
authors: Department of Dermatology, Venerology and Paediatric Dermatology, Lublin, Poland.
authors: Aleksandra
authors: A
authors: Filipczak
authors: Department of Dermatology, Venerology and Paediatric Dermatology, Lublin, Poland.
authors: Małgorzata
authors: M
authors: Michalska-Jakubus
authors: Department of Haemotooncology and Bone Marrow Transplantation, Medical University of Lublin, Lublin, Poland.|Department of Haemostatic Disorders and Internal Diseases, Institute of Haematology and Transfusion Medicine in Warsaw, Warsaw, Poland.
authors: Magdalena
authors: M
authors: Górska-Kosicka
authors: Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland.
authors: Katarzyna
authors: K
authors: Woźniak
authors: Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland.
authors: Cezary
authors: C
authors: Kowalewski
authors: Department of Medical Radiology, University Hospital No.1 in Lublin, Lublin, Poland.
authors: Witold
authors: W
authors: Krupski
authors: Department of Dermatology, Venerology and Paediatric Dermatology, Lublin, Poland.
authors: Aldona
authors: A
authors: Pietrzak
authors: 2nd Department of General, Gastroenterological and Alimentary Oncological Surgery, Medical University of Lublin, Lublin, Poland.
authors: Grzegorz
authors: G
authors: Wallner
authors: Department of Internal Diseases, University Hospital No.1, Medical University of Lublin, Lublin, Poland.
authors: Jerzy
authors: J
authors: Mosiewicz
authors: Dermatology Division, University of Rome G. Marconi, Rome, Italy.
authors: Torello
authors: T
authors: Lotti
authors: Department of Dermatology, Venerology and Paediatric Dermatology, Lublin, Poland.
authors: Dorota
authors: D
authors: Krasowska
mesh_terms: D001323:Autoantibodies / Q000097:blood; D005169:Factor VIII / Q000276:immunology*; D005260:Female; D006406:Hematoma / Q000209:etiology; D006467:Hemophilia A / Q000150:complications* / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008875:Middle Aged; D009135:Muscular Diseases / Q000209:etiology; D010388:Pelvis; D010392:Pemphigus / Q000150:complications*
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G; D005169:Factor VIII
keywords: acquired hemophilia A; hematomas; pemphigus foliaceus; prolonged activated partial thromboplastin time; spontaneous hemorrhages