PDF(Pubmed)
Abstract：
Granulomatosis with polyangiitis (GPA), formerly Wegener\'s granulomatosis, is a small- and medium-vessel vasculitis with characteristic cutaneous morphologic presentation and systemic involvement. Most patients have palpable purpura at some point in their disease course, but this is not always the presenting manifestation. This autoimmune disorder can affect a range of organs, with the upper and lower respiratory tract, kidneys, and nervous system being commonly implicated, while gastrointestinal and cardiac involvement is less frequent. This is a 44-year-old female presenting to the emergency department (ED) with polyarthralgia and palpable purpura. Palpable purpura was distributed on the oral palate, elbow, and lower back, and a punch biopsy revealed leukocytoclastic vasculitis (LCV). While this was an atypical distribution for leukocytoclastic vasculitis, the skin biopsy provided the necessary evidence to diagnose GPA. This case characterizes non-specific and atypical signs and symptoms of GPA that all providers should be aware of in order to diagnose the condition early in its disease course.
摘要：
肉芽肿性多血管炎（GPA），以前是韦格纳肉芽肿病，是一种中小血管血管炎，具有特征性的皮肤形态表现和全身受累。大多数患者在病程中的某个时候都有明显的紫癜，但这并不总是呈现的表现。这种自身免疫性疾病可以影响一系列器官，上呼吸道和下呼吸道，肾脏，神经系统通常被牵连，而胃肠道和心脏受累的频率较低。这是一名44岁的女性，因多发性关节痛和明显的紫癜前往急诊科（ED）。明显的紫癜分布在口腔，弯头,和下背部，穿刺活检显示白细胞碎裂性血管炎（LCV）。虽然这是白细胞碎裂性血管炎的非典型分布，皮肤活检为诊断GPA提供了必要的证据.该病例描述了所有提供者都应了解的GPA的非特异性和非典型体征和症状，以便在其病程早期诊断该疾病。
