Abstract：
A case of a 28-year-old woman with known C1-inhibitor deficiency (functional, Type II) with persistent bilateral non-pruritic, mildly photosensitive facial rash for 10 months following delivery of her second child is presented. Histology of the skin was suggestive of tumid lupus erythematosus (LE), but no other features of systemic LE (ANA, dsDNA negative) were evident. She had stopped danazol which was controlling the underlying disease, and once this was restarted and treatment for tumid lupus was started, she improved. More rigorous control preventing all C1-inhibitor deficiency-related attacks proved successful. The hypothesis that uncontrolled classical pathway complement activation that led to the lupus-like skin lesions is being presented as a clinical case, highlighting the complex interrelationships between immunodeficiency and autoimmunity in inborn errors in immunity.
摘要：
一例28岁女性，已知C1抑制剂缺乏症（功能性，II型)伴持续性双侧非瘙痒,她的第二个孩子分娩后10个月出现轻度光敏性面部皮疹。皮肤组织学提示肿瘤性红斑狼疮（LE），但没有系统性LE的其他特征(ANA，dsDNA阴性)是明显的。她停止了控制潜在疾病的达那唑，一旦重新启动并开始治疗肿瘤狼疮，她改进了。更严格的控制预防所有C1抑制剂缺乏相关的攻击被证明是成功的。不受控制的经典途径补体激活导致狼疮样皮肤病变的假设正在作为临床病例提出。强调免疫缺陷和自身免疫之间复杂的相互关系在先天免疫错误。
