M. Żychowska
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引用次数: 0

摘要

结节性多动脉炎(PAN)属于中度血管炎。两种亚型的疾病是典型的区分-全身变型和皮肤变型。全身性PAN是一种潜在的危及生命的疾病,而皮肤型PAN通常以病程轻微为特征。本文旨在综述PAN的症状、诊断和治疗,并引起人们对该病的重视。28-60%的全身PAN患者会发生皮肤变化。最常见的表现包括可触及的紫癜、网状红斑和炎症性结节,以及较少见的荨麻疹病变、短暂性红斑、远端坏死、浅表静脉炎和碎片性出血。皮肤PAN的皮肤病变通常位于下肢,较少见于上肢或躯干。疼痛的结节、网状增生和溃疡是皮肤PAN最常见的表现。溃疡愈合后通常留下象牙白色的星状疤痕(白色萎缩),周围是毛细血管扩张。该疾病的全身和皮肤亚型的诊断是基于诊断标准。全身性PAN的治疗包括诱导缓解和维持治疗。治疗方案的选择应基于疾病的严重程度和合并症,特别是乙型肝炎病毒感染。皮肤PAN的特点是良性过程,经常复发。一线治疗通常包括非甾体抗炎药、秋水仙碱和氨苯砜。考虑到PAN过程中皮肤病变的频繁发展,了解该病的临床表现、诊断和治疗是日常皮肤科实践的重要组成部分。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Guzkowe zapalenie tętnic — obraz kliniczny, diagnostyka i leczenie
Polyarteritis nodosa (PAN) belongs to the group of medium vessel vasculitis. Two subtypes of the disease are classically distinguished — systemic variant and cutaneous variant. Systemic PAN is a potentially life-threatening disease, while cutaneous subtype is usually characterized by mild course. The review aims to summarize the symptomatology, diagnostics and treatment of PAN and to draw attention to the dermatological manifestations of the disease. Skin changes develop in 28–60% of patients with systemic PAN. The most common manifestations include palpable purpura, livedo reticularis  and inflammatory nodules, and less commonly - urticarial lesions, transient erythema, distal necrosis, superficial phlebitis and splinter haemorrhages. Skin lesions in cutaneous PAN are typically located on the lower extremities, and less frequently — on the upper extremities or trunk. Painful nodules, livedo reticularis and ulcerations are the most frequent manifestations of cutaneous PAN. Ulcerations usually heal leaving ivory-white stellate scars ( atrophie blanche ) surrounded by telangiectasias. The diagnosis of both systemic and cutaneous subtype of the disease is based on diagnostic criteria. Management of systemic PAN consists of induction of remission and maintenance therapy. The choice of therapeutic option should be based on the severity of the disease and comorbidities, especially hepatitis B virus infection. Cutaneous PAN is characterized by a benign course with frequent relapses. First-line treatment usually consists of non-steroidal anti-inflammatory drugs, colchicine and dapsone. Considering frequent development of skin lesions in the course of PAN, knowledge of the clinical presentation, diagnosis and treatment of the condition is an important part of the everyday dermatological practice.
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