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皮肤血管炎的组织学评估。

The histological assessment of cutaneous vasculitis.

机构信息

Department of Pathology, Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, NY, USA.

出版信息

Histopathology. 2010 Jan;56(1):3-23. doi: 10.1111/j.1365-2559.2009.03443.x.

Abstract

Vasculitis is defined as inflammation directed at vessels, which compromises or destroys the vessel wall leading to haemorrhagic and/or ischaemic events. Skin biopsy is the gold standard for the diagnosis of cutaneous vasculitis, whose manifestations include urticaria, infiltrative erythema, petechiae, purpura, purpuric papules, haemorrhagic vesicles and bullae, nodules, livedo racemosa, deep (punched out) ulcers and digital gangrene. These varied morphologies are a direct reflection of size of the vessels and extent of the vascular bed affected, ranging from a vasculitis affecting few superficial, small vessels in petechial eruptions to extensive pan-dermal small vessel vasculitis in haemorrhagic bullae to muscular vessel vasculitis in lower extremity nodules with livedo racemosa. Skin biopsy, extending to subcutis and taken from the earliest, most symptomatic, reddish or purpuric lesion is crucial for obtaining a high-yielding diagnostic sample. Based on histology, vasculitis can be classified on the size of vessels affected and the dominant immune cell mediating the inflammation (e.g. neutrophilic, granulomatous, lymphocytic, or eosinophilic). Disruption of small vessels by inflammatory cells, deposition of fibrin within the lumen and/or vessel wall coupled with nuclear debris allows for the confident recognition of small vessel, mostly neutrophilic vasculitis (also known as leukocytoclastic vasculitis). In contrast, muscular vessel vasculitis can be identified solely by infiltration of its wall by inflammatory cells. Extravasation of red blood cells (purpura) and necrosis are supportive, but not diagnostic of vasculitis as they are also seen in haemorrhagic and/or vaso-occlusive disorders (pseudovasculitis). Vasculitic foci associated with extravascular granulomas (palisaded neutrophilic and granulomatous dermatitis), tissue eosinophilia, or tissue neutrophilia signal the risk for, or co-existence of systemic disease. This essential histological information coupled with direct immunofluorescence and anti-neutrophil cytoplasmic data and clinical findings enables more precise and accurate diagnosis of localized and systemic vasculitis syndromes.

摘要

血管炎被定义为针对血管的炎症,它会损害或破坏血管壁,导致出血和/或缺血事件。皮肤活检是诊断皮肤血管炎的金标准,其表现包括荨麻疹、浸润性红斑、瘀点、紫癜、紫癜性丘疹、出血性水疱和大疱、结节、Racemosa 性网状青斑、深部(穿孔)溃疡和指(趾)干性坏疽。这些不同的形态直接反映了受累血管的大小和血管床的范围,从少数浅表小血管受累的瘀点性皮疹中的血管炎到广泛的全皮小血管血管炎的出血性大疱,再到下肢结节中的肌肉血管炎伴 Racemosa 性网状青斑。皮肤活检,延伸至皮下组织,取自最早、最有症状、红色或紫癜性病变,对于获得高产诊断样本至关重要。根据组织病理学,血管炎可以根据受累血管的大小和介导炎症的主要免疫细胞进行分类(例如中性粒细胞、肉芽肿性、淋巴细胞性或嗜酸性粒细胞性)。炎症细胞对小血管的破坏、管腔和/或血管壁内纤维蛋白的沉积以及核碎片的存在,使得能够明确识别小血管,主要是中性粒细胞性血管炎(也称为白细胞碎裂性血管炎)。相比之下,肌肉血管炎仅通过炎症细胞浸润其壁即可识别。红细胞外渗(紫癜)和坏死是支持性的,但不是血管炎的诊断,因为它们也见于出血和/或血管阻塞性疾病(假性血管炎)。与血管外肉芽肿(栅栏状中性粒细胞和肉芽肿性皮炎)、组织嗜酸性粒细胞或组织中性粒细胞相关的血管炎灶提示存在系统性疾病的风险或共存。这种基本的组织学信息,加上直接免疫荧光和抗中性粒细胞细胞质数据以及临床发现,能够更精确和准确地诊断局部和系统性血管炎综合征。

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