Shavit Eran, Horev Amir, Berman Eitan, Halevy Sima
Department of Dermatology and Venereology, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.
Harefuah. 2012 Oct;151(10):558-61, 606.
Polyarteritis nodosa (PAN) is a multi-system disease, characterized by necrotizing vasculitis of medium-sized arteries that may affect any organ system. Cutaneous PAN is the cutaneous limited form of PAN. It affects 10% of all cases of PAN and usually demonstrates a benign and chronic course. We hereby describe a 47-year-old female with diabetes mellitus who presented with painful ulcers on both legs. The clinical and histological findings were consistent with PAN. A thorough investigation ruled out systemic PAN and cutaneous PAN was determined. Despite intensive therapies including corticosteroids and azathioprine, marked progression of the ulcers was noted and large areas of necrosis appeared. The patient underwent above-knee amputation of both legs and eventually died in less than three years. Although cutaneous PAN is known to have a benign and chronic course, we have presented an unusual progressive and severe course that resulted in the death of the patient.
结节性多动脉炎(PAN)是一种多系统疾病,其特征为中等大小动脉的坏死性血管炎,可累及任何器官系统。皮肤型PAN是PAN的皮肤局限性形式。它占所有PAN病例的10%,通常表现为良性慢性病程。我们在此描述一名47岁患有糖尿病的女性,她双下肢出现疼痛性溃疡。临床和组织学检查结果与PAN相符。全面检查排除了系统性PAN,确诊为皮肤型PAN。尽管采用了包括皮质类固醇和硫唑嘌呤在内的强化治疗,但溃疡仍明显进展,出现大面积坏死。患者接受了双下肢膝上截肢术,最终在不到三年的时间内死亡。虽然已知皮肤型PAN有良性慢性病程,但我们报告了一例不寻常的进行性严重病程,导致患者死亡。