获得性血友病A与某些皮肤病的关系

Relationship between acquired hemophilia A and some cutaneous disorders

获得性血友病A是一类后天获得的凝血因子8缺乏性疾病，某些皮肤病如大疱性疾病、银屑病、系统性红斑狼疮、蕈样肉芽肿等可伴发获得性血友病A。患者可出现皮肤及其他各器官的出血．实验室检查活化的部分凝血活酶时间延长，第8因子活性降低。获得性血友病A与这些皮肤病合并的机制可能与免疫紊乱有关。可系统应用糖皮质激素、免疫抑制剂及凝血因子等治疗。

Acquired hemophilia A （ AHA ） is an acquired disease due to the lack of coagulation factor Ⅷ. Some cutaneous disorders, such as autoimmune bullous diseases, psoriasis, systemic lupus erythematosus and mycosis fungoides might be accompanied by AHA. Patients with cutaneous disorders associated with AHA may suffer from bleeding of skin and other organs, prolongation of activated partial thromboplastin time （ APTT ）and decrease of factor VIII activity. The pathogenesis of cutaneous disorders accompanied by AHA may be related to the disorder of immune system. Systemic corticosteroid, immunosuppressants and coagulation factors may be used in the treatment of AHA.