Ruocco E, Sangiuliano S, Gravina A G, Miranda A, Nicoletti G
Department of Dermatology, Second University of Naples, Naples, Italy.
J Eur Acad Dermatol Venereol. 2009 Sep;23(9):1008-17. doi: 10.1111/j.1468-3083.2009.03199.x. Epub 2009 Mar 11.
Pyoderma gangrenosum is a rare, ulcerative, cutaneous condition. First described in 1930, the pathogenesis of pyoderma gangrenosum remains unknown, but it is probably related to a hyperergic reaction. There are various clinical and histological variants of this disorder. Pyoderma gangrenosum often occurs in association with a systemic disease such as inflammatory bowel disease, rheumatologic disease, paraproteinaemia, or haematological malignancy. The diagnosis, mainly based on the clinical presentation and course, is confirmed through a process of elimination of other causes of cutaneous ulcers. Local treatment may be sufficient for mild disease, while for severe cases, systemic immunosuppressants are the mainstay. Long-term treatment with these agents is often required, but this can expose patients to adverse side-effects.
坏疽性脓皮病是一种罕见的溃疡性皮肤病。1930年首次被描述,坏疽性脓皮病的发病机制尚不清楚,但可能与超敏反应有关。这种疾病有多种临床和组织学变体。坏疽性脓皮病常与全身性疾病如炎症性肠病、风湿性疾病、副蛋白血症或血液系统恶性肿瘤相关。诊断主要基于临床表现和病程,通过排除其他皮肤溃疡原因的过程来确诊。对于轻症病例,局部治疗可能就足够了,而对于重症病例,全身免疫抑制剂是主要治疗手段。通常需要长期使用这些药物进行治疗,但这可能会使患者面临不良副作用。