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显微镜下多血管炎患者的皮肤表现:两例病例报告及文献综述

Cutaneous manifestations in patients with microscopic polyangiitis: two case reports and a minireview.

作者信息

Kawakami Tamihiro, Soma Yoshinao, Saito Chihiro, Ogawa Hitoshi, Nagahuchi Yuko, Okazaki Takahiro, Ozaki Shoichi, Mizoguchi Masako

机构信息

Department of Dermatology, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, Kanagawa 216-8511, Japan.

出版信息

Acta Derm Venereol. 2006;86(2):144-7. doi: 10.2340/00015555-0034.

DOI:10.2340/00015555-0034
PMID:16648918
Abstract

Microscopic polyangiitis is a systemic small vessel vasculitis, which often has cutaneous and musculoskeletal features. Microscopic polyangiitis is a member of the family of anti-neutrophil cytoplasmic auto-antibody (ANCA)-associated vasculitides and is strongly associated with anti-myeloperoxidase (MPO)-ANCA. Titres of MPO-ANCA may reflect disease activity and play a pathogenic role. Patients with microscopic polyangiitis usually present with erythematous macules on the extremities as the first cutaneous manifestation. Skin biopsy specimens from the erythema reveal small-sized vessels that are infiltrated with neutrophils, consistent with leukocytoclastic vasculitis, in the deep dermis to the subcutaneous fat tissue. The cutaneous involvement is present at an early stage of microscopic polyangiitis with other non-specific symptoms, such as arthralgias and myalgias. The initial cutaneous manifestations are important in early diagnosis of possible ANCA-associated vasculitides with elevated ANCA titres.

摘要

显微镜下多血管炎是一种系统性小血管炎,常具有皮肤和肌肉骨骼特征。显微镜下多血管炎是抗中性粒细胞胞浆自身抗体(ANCA)相关血管炎家族的一员,与抗髓过氧化物酶(MPO)-ANCA密切相关。MPO-ANCA滴度可能反映疾病活动并起致病作用。显微镜下多血管炎患者通常以四肢出现红斑作为首发皮肤表现。红斑部位的皮肤活检标本显示,在真皮深层至皮下脂肪组织中有小血管被中性粒细胞浸润,符合白细胞破碎性血管炎。皮肤受累在显微镜下多血管炎早期出现,同时伴有其他非特异性症状,如关节痛和肌痛。最初的皮肤表现对于早期诊断ANCA滴度升高的可能的ANCA相关血管炎很重要。

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