Wollina Uwe
Department of Dermatology, Hospital Dresden-Friedrichstadt, PO Box 120906, 01008 Dresden, Germany.
Am J Clin Dermatol. 2002;3(3):149-58. doi: 10.2165/00128071-200203030-00002.
Pyoderma gangrenosum is a noninfectious neutrophilic dermatosis that usually starts with sterile pustules which rapidly progress to painful ulcers of variable depth and size with undermined violaceous borders. In 17 to 74% of cases, pyoderma gangrenosum is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatological or hematological disease or malignancy. Diagnosis of pyoderma gangrenosum is based on a history of an underlying disease, typical clinical presentation and histopathology, and exclusion of other diseases that would lead to a similar appearance. Randomized, double-blinded prospective multicenter trials investigating the treatment of pyoderma gangrenosum are not available. The treatments with the best clinical evidence are systemic corticosteroids (in the initial phase usually 100 to 200 mg/day) and cyclosporine (mainly as a maintenance treatment). Combinations of corticosteroids with cytotoxic drugs such as azathioprine, cyclophosphamide or chlorambucil are used in patients with disease that is resistant to corticosteroids. The combination of corticosteroids with sulfa drugs, such as dapsone, or clofazimine, minocycline and thalidomide, has been used as a corticosteroid-sparing alternative. Limited experience has been documented with methotrexate, colchicine, nicotine, and mycophenolate mofetil, among other drugs. Alternative treatments include local application of granulocyte-macrophage colony-stimulating factor, intravenous immunoglobulins and plasmapheresis. Skin transplants (split-skin grafts or autologous keratinocyte grafts) and the application of bioengineered skin is useful in selected cases in conjunction with immunosuppression. Topical therapy with modern wound dressings is useful to minimize pain and the high risk of secondary infection. The application of topical antibacterials cannot be recommended because of their potential to sensitize and their questionable efficacy, but systemic antibacterial therapy is mandatory when infection is present. Despite recent advances in therapy, the prognosis of pyoderma gangrenosum remains unpredictable.
坏疽性脓皮病是一种非感染性嗜中性皮肤病，通常始于无菌脓疱，这些脓疱会迅速发展为深度和大小不一的疼痛性溃疡，边缘呈紫红色且有潜行。在17%至74%的病例中，坏疽性脓皮病与潜在疾病相关，最常见的是炎症性肠病、风湿性或血液系统疾病或恶性肿瘤。坏疽性脓皮病的诊断基于潜在疾病史、典型临床表现和组织病理学检查，并排除其他会导致类似表现的疾病。目前尚无针对坏疽性脓皮病治疗的随机、双盲前瞻性多中心试验。临床证据最佳 的治疗方法是全身使用皮质类固醇（初始阶段通常为每日100至200毫克）和环孢素（主要作为维持治疗）。对于对皮质类固醇耐药的患者，可将皮质类固醇与硫唑嘌呤、环磷酰胺或苯丁酸氮芥等细胞毒性药物联合使用。皮质类固醇与磺胺类药物（如氨苯砜）或氯法齐明、米诺环素和沙利度胺联合使用，已被用作减少皮质类固醇用量的替代方案。甲氨蝶呤、秋水仙碱、尼古丁和霉酚酸酯等药物的使用经验有限。替代治疗方法包括局部应用粒细胞巨噬细胞集落刺激因子、静脉注射免疫球蛋白和血浆置换。皮肤移植（分层皮片移植或自体角质形成细胞移植）以及生物工程皮肤的应用在选定病例中结合免疫抑制是有用的。使用现代伤口敷料进行局部治疗有助于减轻疼痛和降低继发感染的高风险。由于局部使用抗菌药物有致敏的可能性且疗效存疑，因此不推荐使用，但存在感染时必须进行全身抗菌治疗。尽管近年来治疗取得了进展，但坏疽性脓皮病的预后仍然不可预测。
