Mahmood-Rao Hamzah, Gandhi Nirav, Ding Tina
Royal Derby Hospital, Derby, UK.
BMJ Case Rep. 2017 Jun 29;2017:bcr-2017-220300. doi: 10.1136/bcr-2017-220300.
Cutaneous polyarteritis nodosa (CPAN) is a rare diagnosis which is distinct from polyarteritis nodosa (PAN). PAN is a medium-vessel vasculitis which can affect multiple organs and classically produces microaneurysms in the vasculature. CPAN is limited to the skin mainly affecting small vessels. There is an absence of microaneurysms in CPAN and it does not affect internal organs. However, the histopathological findings on the skin are similar to PAN. CPAN rarely progresses to PAN but relapses more often. We will illustrate a challenging case of a patient with CPAN who developed gangrenous infarcts despite initial immunosuppressive treatment with high-dose steroids and azathioprine. His treatment had to be escalated to intravenous cyclophosphamide which induced disease remission.
皮肤结节性多动脉炎(CPAN)是一种罕见的诊断,与结节性多动脉炎(PAN)不同。PAN是一种中等血管的血管炎,可影响多个器官,典型地在血管系统中产生微动脉瘤。CPAN局限于皮肤,主要影响小血管。CPAN中不存在微动脉瘤,也不影响内脏器官。然而,皮肤的组织病理学表现与PAN相似。CPAN很少进展为PAN,但更容易复发。我们将举例说明一个具有挑战性的CPAN病例,该患者尽管最初使用大剂量类固醇和硫唑嘌呤进行免疫抑制治疗,但仍出现了坏疽性梗死。他的治疗不得不升级为静脉注射环磷酰胺,从而诱导疾病缓解。