Lambert J, Jonlet F, Vanhooteghem O, Richert B, de la Brassinne M
Service de Dermatologie, Université de Liège.
Rev Med Liege. 2001 Feb;56(2):106-11.
Pyoderma gangrenosum (PG) is a rare chronic inflammatory skin disease characterized by the recurring development of necrotizing and painful ulcers. The skin lesions appear spontaneously or after minor traumatic injuries. Sites of predilection include the lower limbs and the trunk but any part of the body may be affected. PG is not an infectious disease; although the etiology is not completely understood, an immune disturbance is certainly involved. An underlying systemic disorder is associated in up to 50% of the cases, specially inflammatory bowel diseases, arthritis, paraproteinemias and hematologic malignancies. Chronic venous or arterial ulcers as well as bacterial gangrene are the most frequent false diagnoses. A right diagnosis, based upon the distinctive clinical features and a compatible histology, is essential to avoid surgical procedure that often tends to exacerbed the process. Because of its persistent and recurrent nature, systemic long-term therapy based upon corticosteroids associated with sulfones or immunosuppressive agents is required.
坏疽性脓皮病(PG)是一种罕见的慢性炎症性皮肤病,其特征是坏死性疼痛性溃疡反复出现。皮肤病变可自发出现或在轻微外伤后出现。好发部位包括下肢和躯干,但身体的任何部位都可能受累。PG不是传染病;虽然病因尚未完全明确,但肯定涉及免疫紊乱。高达50%的病例与潜在的全身性疾病有关,特别是炎症性肠病、关节炎、副蛋白血症和血液系统恶性肿瘤。慢性静脉或动脉溃疡以及细菌性坏疽是最常见的误诊。基于独特的临床特征和相符的组织学进行正确诊断,对于避免往往会使病情加重的外科手术至关重要。由于其持续性和复发性,需要基于皮质类固醇联合砜类药物或免疫抑制剂进行全身性长期治疗。