Kaplan Allen P, Greaves Malcolm W
Department of Medicine, Medical University of South Carolina, Charleston, South Carolina, USA.
J Am Acad Dermatol. 2005 Sep;53(3):373-88; quiz 389-92. doi: 10.1016/j.jaad.2004.09.032.
Although first described more than 130 years ago, the pathophysiology, origin, and management of the several types of angioedema are poorly understood by most dermatologists. Although clinically similar, angioedema can be caused by either mast cell degranulation or activation of kinin formation. In the former category, allergic and nonsteroidal anti-inflammatory drug-induced angioedema are frequently accompanied by urticaria. Idiopathic chronic angioedema is also usually accompanied by urticaria, but can occur without hives. In either case, an autoimmune process leading to dermal mast cell degranulation occurs in some patients. In these patients, histamine-releasing IgG anti-FcepsilonR1 autoantibodies are believed to be the cause of the disease, removal or suppression by immunomodulation being followed by remission. Angiotensin-converting enzyme inhibitor-induced angioedema is unaccompanied by hives, and is caused by the inhibition of enzymatic degradation of tissue bradykinin. Hereditary angioedema, caused by unchecked tissue bradykinin formation, is recognized biochemically by a low plasma C'4 and low quantitative or functional C'1 inhibitor. Progress has now been made in understanding the molecular genetic basis of the two isoforms of this dominantly inherited disease. Recently, a third type of hereditary angioedema has been defined by several groups. Occurring exclusively in women, it is not associated with detectable abnormalities of the complement system. Angioedema caused by a C'1 esterase inhibitor deficiency can also be acquired in several clinical settings, including lymphoma and autoimmune connective tissue disease. It can also occur as a consequence of specific anti-C'1 esterase autoantibodies in some patients. We have reviewed the clinical features, diagnosis, and management of these different subtypes of angioedema.
After completing this learning activity, participants should be aware of the classification, causes, and differential diagnosis of angioedema, the molecular basis of hereditary and non-hereditary forms of angioedema, and be able to formulate a pathophysiology-based treatment strategy for each of the subtypes of angioedema.
尽管血管性水肿早在130多年前就被首次描述，但大多数皮肤科医生对几种类型血管性水肿的病理生理学、起源及治疗了解甚少。血管性水肿虽然在临床上相似，但可由肥大细胞脱颗粒或激肽形成激活引起。在前一类中，过敏性和非甾体抗炎药诱导的血管性水肿常伴有荨麻疹。特发性慢性血管性水肿通常也伴有荨麻疹，但也可在无风疹块的情况下发生。在这两种情况下，一些患者会发生导致真皮肥大细胞脱颗粒的自身免疫过程。在这些患者中，释放组胺的IgG抗FcepsilonR1自身抗体被认为是疾病的病因，通过免疫调节去除或抑制后病情缓解。血管紧张素转换酶抑制剂诱导的血管性水肿不伴有风疹块，由组织缓激肽的酶促降解受抑制引起。遗传性血管性水肿由不受控制的组织缓激肽形成引起，通过低血浆C'4以及低定量或功能性C'1抑制剂在生化上得以识别。目前在了解这种显性遗传病两种亚型的分子遗传基础方面已取得进展。最近，几个研究小组定义了第三种类型的遗传性血管性水肿。它仅发生于女性，与补体系统可检测到的异常无关。由C'1酯酶抑制剂缺乏引起的血管性水肿也可在多种临床情况下获得，包括淋巴瘤和自身免疫性结缔组织病。在一些患者中，它也可能是特定抗C'1酯酶自身抗体导致的结果。我们回顾了这些不同亚型血管性水肿的临床特征、诊断及治疗。
完成本学习活动后，参与者应了解血管性水肿的分类、病因及鉴别诊断，遗传性和非遗传性血管性水肿的分子基础，并能够为每种血管性水肿亚型制定基于病理生理学的治疗策略。
