Zoroquiain Pablo, González Sergio, Molgó Montserrat, Rodríguez Alejandra, Valbuena José R
Department of Anatomic Pathology, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Am J Dermatopathol. 2012 May;34(3):330-4. doi: 10.1097/DAD.0b013e3182318dd9.
Extensive necrotizing vasculitis (ENV) is a rare paraneoplastic phenomenon, and the majority of cases reported are associated with hematolymphoid neoplasms. Histologically, most cases of ENV represent leukocytoclastic vasculitis (LCV). Here we report the clinicopahological features of a 68-year-old man with ENV associated to a Epstein Barr virus-positive diffuse large B-cell lymphoma (EBV+DLBCL) of the elderly, a newly recognized lymphoproliferative disorder, most likely representing a paraneoplastic manifestation. The patient was treated with standard chemotherapy regimen for malignant lymphoma. Due to the extensive involvement of the extremities by ENV, surgical debridement was not feasible and a novel therapy based on CHITOSAN apposits was initiated with overall good response and subsequent re-epithelization of the skin lesions. The patient died of sepsis secondary to a Pseudomona pneumonia 17 months after diagnosis.
广泛坏死性血管炎（ENV）是一种罕见的副肿瘤现象，报告的大多数病例与血液淋巴系统肿瘤相关。组织学上，大多数ENV病例表现为白细胞破碎性血管炎（LCV）。在此，我们报告一名68岁男性ENV患者的临床病理特征，该患者与老年型爱泼斯坦-巴尔病毒阳性弥漫性大B细胞淋巴瘤（EBV+DLBCL）相关，这是一种新认识的淋巴增殖性疾病，很可能代表一种副肿瘤表现。该患者接受了恶性淋巴瘤的标准化疗方案治疗。由于ENV广泛累及四肢，手术清创不可行，因此启动了基于壳聚糖贴剂的新疗法，总体反应良好，皮肤病变随后重新上皮化。患者在诊断后17个月死于假单胞菌肺炎继发的败血症。
