ABSTRACT
Bullous pemphigoid(BP)is an autoimmune blister disease caused by antibodies to the basement membrane zone.It usually exhibits tense bullae in skin and mucous membrane.BP patients can suffer from thrombosis due to hypercoagulation and/or hemorrhage due to the presence of anti-coagulation factors,which may be explained by the eosinophils,antibodies to the coagulation factors,and fibrinolytic system hyperactivity or inhibition.
Subject(s)
Blood Coagulation Disorders/complications , Pemphigoid, Bullous/complications , Autoantibodies , Blister , Blood Coagulation Factors , Eosinophils , Hemorrhage/etiology , Humans , Mucous Membrane/pathology , Skin/pathology , Thrombosis/etiologySubject(s)
Autoimmune Diseases/epidemiology , Skin Diseases, Vesiculobullous/epidemiology , Beijing/epidemiology , Female , Health Surveys , Humans , Male , Middle Aged , Pemphigoid, Benign Mucous Membrane/epidemiology , Pemphigoid, Bullous/epidemiology , Pemphigoid, Bullous/immunology , Sex Distribution , Skin Diseases, Vesiculobullous/immunologyABSTRACT
Epidermolysis bullosa acquisita (EBA) is a rare chronic subepidermal bullous autoimmune disease. The occurrence of acquired hemophilia A (AHA) is low and so the coexistence of EBA and AHA is extremely rare. We herein described a case of EBA coexisting with AHA and a case of EBA coexisting with AHA and hepatitis B. These EBA may be related to the pathogenesis of AHA. In this study, we analyzed the clinical features in the two Chinese cases of EBA coexisting with AHA, and found esophageal hemorrhage and hematemesis were the main symptoms of both patients. Cyclosporin, prednisone and lamivudine effectively control EBA with AHA and hepatitis B. The dose of cyclosporin should be more than 4 mg/kg per day and the period of treatment should be longer than 5 months to reduce the risk of EBA co-occurring with AHA.
