肉芽肿合并多血管炎患者复发性结节样红斑

IF 1.1 Q4 ALLERGY

Journal of Cutaneous Immunology and Allergy Pub Date : 2022-10-13 DOI:10.1002/cia2.12283

Satomi Imamoto MD, Chie Miyabe MD, PhD, Ryujin Miyata MD, Yasuko Fukuya MD, PhD, Naoko Ishiguro MD, PhD

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引用次数: 0

摘要

多血管炎肉芽肿病(GPA)，以前称为韦格纳肉芽肿病，是一种抗中性粒细胞胞浆抗体(ANCA)相关的系统性血管炎，以坏死性肉芽肿性炎症为特征，主要影响中小型血管大约30% - 50%的gpa患者有皮肤症状。2皮肤表现各不相同;可触及的紫癜是最常见的发现，丘疹、出血性大疱、溃疡和皮下结节也经常发生;脓疱、囊泡、斑点和点较少见一个61岁的妇女被转介到我们诊所评估红色结节在她的下肢。她在9年前被诊断为GPA。初诊时表现为高热、鼻出血、组织病理学肉芽肿性炎症引起的肺结节、口腔溃疡和血尿。给予口服皮质类固醇和静脉注射环磷酰胺(IVCY)治疗。随访期间，患者复发时多次出现结节性红斑(EN)样皮肤疹。在整个随访期间，细胞质ANCA和蛋白酶3 ANCA均为阴性。在目前的表现中，在下肢观察到多个en样硬化红斑病变(图1A)。硬化红斑的皮肤活检显示淋巴细胞浸润皮下静脉，纤维内膜增生阻塞血管腔(图1B - D)。en样皮肤症状被认为是由GPA的皮下血管炎引起的。随后，患者接受IVCY，随后口服皮质类固醇和硫唑嘌呤。初次皮肤活检六年后，患者出现流感样症状后腿部出现皮疹，再次被转介到我们的诊所。检查时，在小腿和足背观察到多发en样疹(图1E)。皮肤活检示隔膜炎伴混合细胞浸润;然而，即使在连续切片上也未观察到血管炎的组织学证据(图1F,G)。给予较高剂量的口服皮质类固醇，这导致皮肤病变的改善。没有单一的皮肤表现被明确地确定为gpa的病理特征。然而，可触及的紫癜是最常见的皮肤症状(47%)，白细胞破坏性血管炎是最常见的病理类型(80%)en样出疹不被认为是典型的皮肤症状，在GPA中通常观察到多种皮肤症状。4据我们所知，这是第一个证明en样出疹是GPA中单独的血管相关皮肤表现的报告。先前的一份报告描述了一名GPA患者，表现为en样皮下结节和室间隔泛膜炎，但组织学上没有明显的血管炎这个病人也有可触及的紫癜，这是由皮肤小血管受累引起的。相比之下，我们的病人没有表现出任何与小血管炎相关的皮肤表现，包括紫癜、丘疹和大疱。我们的研究结果表明，常见于中等血管炎(如结节性多动脉炎)的ENlike爆发可能仅出现在GPA中。由于en样疹是GPA中相对罕见的皮肤表现，皮肤科医生应注意，如果仅影响皮下中等血管，en样疹也可能是GPA的皮肤症状。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

Relapsing erythema nodosum-like eruption in a patient with granulomatosis with polyangiitis

查看原文本刊更多论文

Relapsing erythema nodosum-like eruption in a patient with granulomatosis with polyangiitis

We report a rare case of granulomatosis with polyangiitis (GPA) who presented with relapsing erythema nodosum (EN)-like eruption as a solo vasculitis-associated cutaneous manifestation. We suggest that EN-like eruption alone can be a cutaneous symptom of GPA if subcutaneous medium-sized vessels are exclusively affected.

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来源期刊

Journal of Cutaneous Immunology and Allergy Medicine-Dermatology

CiteScore

0.60

自引率

10.00%

发文量

审稿时长

12 weeks