背景:自身免疫性大疱性疾病(AIBD)是临床上表现为皮肤和粘膜大疱和囊泡的器官特异性皮肤起泡疾病。皮肤屏障完整性的丧失使患者易于感染。坏死性筋膜炎(NF),文献中没有充分记录AIBD罕见但严重的感染性并发症.
方法:我们介绍一例51岁男性NF患者,最初误诊为带状疱疹。鉴于当地的地位,CT成像,和实验室参数,做出NF诊断,并对患者进行紧急手术清创。在进一步的发展中,偏远地区出现了新的大疱,并进行了周边活检,直接免疫荧光以及局部状态,病人的年龄,和非典型的介绍,最初诊断为大疱性表皮松解症。鉴别诊断为大疱性类天疱疮(BP)和大疱性系统性狼疮。在文学中,发现并审查了9个其他描述的病例。
结论:由于其临床表现不明确,坏死性筋膜炎本身表现为经常误诊的软组织感染。免疫抑制患者的实验室参数改变通常会导致NF的误诊和宝贵的时间损失。在生存中起着重要作用。鉴于AIBD表现为皮肤完整性丧失和免疫抑制治疗,这些患者可能比一般人群更容易患NF.
Autoimmune bullous diseases (AIBD) are organ-specific skin blistering diseases clinically manifesting as bullae and vesicles of the skin and mucous membranes. The loss of skin barrier integrity renders patients susceptible to infection. Necrotizing fasciitis (NF), a rare yet severe infectious complication of AIBD has been insufficiently documented in the literature.
We present a case of a 51-year-old male patient with NF initially misdiagnosed as herpes zoster. Given the local status, CT imaging, and laboratory parameters, NF diagnosis was made and the patient was taken for an urgent surgical debridement. In a further development, new bullae in remote areas erupted and a perilesional biopsy, direct immunofluorescence as well as local status, the patient\'s age, and atypical presentation, imposed an initial diagnosis of epidermolysis bullosa acquisita. Differential diagnoses were bullous pemphigoid (BP) and bullous systemic lupus. In the literature, 9 other described cases were found and are reviewed.
Due to its unspecific clinical picture, necrotizing fasciitis itself presents a frequently misdiagnosed soft tissue infection. Altered laboratory parameters in immunosuppressed patients often lead to misdiagnosing of NF and loss of precious time, which plays a major role in survival. Given the manifestation of AIBD as loss of skin integrity and immunosuppressive therapy, these patients could be more predisposed to NF than the general population.