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糖尿病大疱病:一种罕见的伴有免疫球蛋白G(IgG)沉积相关血管病变的表现。病例报告及重点综述

Bullosis Diabeticorum: A Rare Presentation with Immunoglobulin G (IgG) Deposition Related Vasculopathy. Case Report and Focused Review.

作者信息

Sonani Hardik, Abdul Salim Sohail, Garla Vishnu V, Wile Anna, Palabindala Venkataraman

机构信息

Department of Medicine, University of Mississippi Medical Center, Jackson, MS, USA.

Department of Nephrology, University of Mississippi Medical Center, Jackson, MS, USA.

出版信息

Am J Case Rep. 2018 Jan 15;19:52-56. doi: 10.12659/ajcr.905452.

Abstract

BACKGROUND Bullosis diabeticorum (BD) is a condition characterized by recurrent, spontaneous, and non-inflammatory blistering in patients with poorly controlled diabetes mellitus. While etiopathogenesis remains unclear, roles of neuropathy, vasculopathy and UV light are hypothesized. Most literature reports negative direct and indirect immunofluorescence findings in diabetics with bullous eruptions. Porphyria cutanea tarda, bullous pemphigoid, epidermolysis bullosa, and pseudoporphyria are other differential diagnoses of bullous lesions, and they must be excluded. CASE REPORT We present a 42-year-old African American male with long standing poorly controlled insulin dependent diabetes mellitus with blisters on his left hand and feet. The blisters were noticed three weeks prior to presentation and, thereafter, rapidly increased in size and spontaneously ruptured. Physical examination revealed a multitude of both roofed and unroofed bullous painless skin lesions. Hematoxylin and eosin (H&E) staining dramatized the dermal-epidermal blistering and re-epithelization process. Direct Immunofluorescence (DIF) was positive for 2 + IgG deposition in the already thickened basement membrane of the capillaries of the superficial vascular plexus. After debridement, his wounds greatly improved with over three months of aggressive wound care. CONCLUSIONS Primary immunologic abnormality likely plays no role in the onset of BD. To date, only one article has reported nonspecific capillary-associated immunoglobulin M and C3. This is the first case of BD with IgG deposition in the superficial capillary basement membrane. Positive findings on DIF suggest vasculopathy. Dermal microangiopathy, secondary to immunologic abnormality, is a possible underlying pathogenesis to bullae formation. Punch biopsy with DIF can be an additional diagnostic modality in the management of such cases.

摘要

背景

糖尿病性大疱病(BD)是一种在糖尿病控制不佳的患者中表现为反复、自发且非炎症性水疱形成的病症。虽然其发病机制尚不清楚,但推测与神经病变、血管病变和紫外线有关。大多数文献报道糖尿病水疱疹患者的直接和间接免疫荧光检查结果均为阴性。迟发性皮肤卟啉病、大疱性类天疱疮、大疱性表皮松解症和假性卟啉病是水疱性病变的其他鉴别诊断,必须予以排除。病例报告:我们报告一例42岁的非裔美国男性,患有长期胰岛素依赖型糖尿病且控制不佳,左手和足部出现水疱。水疱在就诊前三周被发现,此后迅速增大并自发破裂。体格检查发现大量有疱顶和无疱顶的无痛性皮肤病变。苏木精和伊红(H&E)染色显示了真皮 - 表皮水疱形成和再上皮化过程。直接免疫荧光(DIF)显示浅表血管丛毛细血管已经增厚的基底膜中有2 + IgG沉积。清创后,经过三个多月积极的伤口护理,他的伤口有了很大改善。结论:原发性免疫异常可能在BD的发病中不起作用。迄今为止,仅有一篇文章报道了非特异性的与毛细血管相关的免疫球蛋白M和C3。这是首例BD患者浅表毛细血管基底膜中有IgG沉积的病例。DIF阳性结果提示血管病变。继发于免疫异常的真皮微血管病变可能是水疱形成的潜在发病机制。DIF的打孔活检可作为此类病例管理中的一种额外诊断方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6ba8/5776741/a3129d6ab26e/amjcaserep-19-52-g001.jpg

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