最初表现为皮肤结节性多动脉炎的典型结节性多动脉炎:病例报告

Sabina Adhikari
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引用次数: 0

摘要

典型结节性多动脉炎(PAN)是一种不常见的侵袭性全身血管炎,通常累及肾脏血管和其他内脏器官的中小型动脉,但肺部血管不受影响。皮肤型 PAN(c-PAN)仍然是一种罕见的中小动脉血管炎,它累及皮肤而不累及任何系统或内脏器官。然而,关节炎、关节痛、肌病和肌炎等皮外特征可与皮肤特征并存。组织病理学上,中型血管周围出现多形核白细胞,并伴有纤维素性坏死和血管分叉处的管腔血栓,即可确诊为 c-PAN。c-PAN 的病程为良性慢性过程,可通过小剂量短程口服类固醇或抗炎药物控制,而全身受累的病程则具有侵袭性,需要及时使用大剂量口服类固醇、环磷酰胺、利妥昔单抗或其他免疫抑制剂进行充分的免疫抑制。我们报告了一例 33 岁男性患者的病例,该患者在来我院门诊就诊时具有 c-PAN 的特征,在进一步检查时发现其患有肾梗塞。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Classic polyarteritis nodosa presenting initially as a case of cutaneous polyarteritis nodosa: A case report
Classic polyarteritis nodosa (PAN) is an uncommon form of aggressive systemic vasculitis involving small-to-medium sized arteries usually of renal vasculature and other visceral organs but characteristically sparing the lung vasculature. Cutaneous PAN (c-PAN) is still a rare type of small-to-medium vessel vasculitis with involvement of skin without any systemic or visceral involvement. However, extracutaneous features such as arthritis, arthralgia, myopathy, and myositis can co-exist with the cutaneous features. c-PAN can be confirmed histopathologically by the presence of polymorphonuclear leukocytes around the medium-sized vessels with evidence of fibrinoid necrosis and luminal thrombi at the bifurcations. While c-PAN has a benign but chronic course and can be managed with low doses of short-course oral steroids or anti-inflammatory drugs, systemic involvement has an aggressive course and needs adequate and prompt immunosuppression with high dose oral steroids, cyclophosphamide, rituximab, or other immunosuppressive. We present a case of a 33-year-old male who landed in our OPD with features of c-PAN and on further investigative workup was found to have a renal infarct.
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