Wiesen Jonathan, Gonzalez-Estrada Alexei, Auron Moises
Department of Pulmonary and Critical Care, Cleveland Clinic, Cleveland, Ohio, USA.
BMJ Case Rep. 2014 Apr 10;2014:bcr2013201482. doi: 10.1136/bcr-2013-201482.
Hereditary angioedema (HAE) is an autosomal dominant disorder characterised by attacks of self-limited swelling affecting extremities, face and intra-abdominal organs, most often caused by mutations in the C1-inhibitor gene with secondary Bradykinin-mediated increased vascular permeability. We describe a 36-year-old man with a history of HAE who presented with painful interdigital bullae secondary to an acute oedema exacerbation. Biopsy and cultures of the lesions were negative and they resolved spontaneously. It is important to highlight and recognise the development of oedema blisters after resolution of a flare of HAE (only 1 previous case report), and hence avoid unnecessary dermatological diagnostic workup and treatment.
遗传性血管性水肿（HAE）是一种常染色体显性疾病，其特征为自限性肿胀发作，累及四肢、面部和腹内器官，最常见的病因是C1抑制剂基因突变，继发缓激肽介导的血管通透性增加。我们描述了一名36岁有HAE病史的男性，因急性水肿加重出现疼痛性指间水疱。病变活检和培养结果均为阴性，水疱自行消退。重要的是要强调并认识到HAE发作缓解后水肿性水疱的发生（此前仅有1例病例报告），从而避免不必要的皮肤科诊断检查和治疗。
