Turki S, Guillevin L, Dallot A, Jarrousse B, Vernier I, Laroche L, Pourrat J, Amouroux J
Service de Médecine Interne, Hôpital Avicenne, Bobigny, France.
Ann Dermatol Venereol. 1994;121(4):325-7.
Polyarteritis nodosa (PAN) is, in rare cases, associated with subcutaneous nodules and pathology does not usually show the presence of peri and extravascular granulomas. When present in patients with hepatitis B virus (HBV) related PAN these facts demonstrate that classification of PAN is not homogeneous. CASE REPORT. A patient infected by HBV developed a PAN demonstrated by clinical symptoms and pathology. The disease was characterized by the presence of subcutaneous nodules and histologically by peri- and extra-vascular granuloma which surrounded necrosis of medium-sized vessels. Outcome was also unusual in the patient who did not respond to the association of plasma exchanges and antiviral agents and was only slightly improved by steroids and cyclophosphamide. COMMENTS. HBV-related PAN is considered to be an immune complex disorder. In the present case report granuloma were present as observed in Churg Strauss syndrome or Wegener's granulomatosis which are the consequence of other pathogenetic mechanisms as anticytoplasmic neutrophil antibodies (ANCA). This case reports underlines the heterogeneity of the PAN group of vasculitis and the probable role for various pathogenetic mechanisms.
结节性多动脉炎(PAN)在罕见情况下与皮下结节相关,且病理检查通常未显示血管周围和血管外肉芽肿的存在。当乙型肝炎病毒(HBV)相关的PAN患者出现这些情况时,表明PAN的分类并不统一。病例报告。一名感染HBV的患者出现了经临床症状和病理证实的PAN。该疾病的特征是存在皮下结节,组织学表现为围绕中等大小血管坏死的血管周围和血管外肉芽肿。该患者的病情转归也不寻常,对血浆置换和抗病毒药物联合治疗无反应,仅使用类固醇和环磷酰胺后稍有改善。评论。HBV相关的PAN被认为是一种免疫复合物疾病。在本病例报告中,观察到肉芽肿的存在,类似于变应性肉芽肿性血管炎或韦格纳肉芽肿病,这是由其他致病机制如抗中性粒细胞胞浆抗体(ANCA)引起的。本病例报告强调了PAN组血管炎的异质性以及各种致病机制可能发挥的作用。