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目的:强调坏疽性脓皮病(PG)的诊断和治疗困难的问题。材料和方法。对PG患者的临床检查结果进行文献回顾和分析。坏疽性脓皮病最常见于青年和中年人,多见于妇女。PG的发病机制尚未得到充分的研究。它被认为与基因突变、中性粒细胞功能障碍和免疫/炎症调节受损有关。Maverakis等人制定了PG的诊断标准。一线治疗旨在优化局部伤口护理。对于更严重的疾病,需要全身治疗。口服皮质类固醇(0.5-1 mg/kg/天)是治疗的基础,用于控制PG。环孢素可单独使用,也可与皮质类固醇合用。目前,越来越多的证据表明生物疗法作为治疗多种细胞因子的有效方法。结果和讨论。本文报告1例确诊为PG的患者,根据最新的指南,对全身性皮质类固醇治疗(Medrol起始剂量为48mg /d)、全身性抗炎和血管药物、局部治疗和治疗结果进行了报告。如果不进行治疗,这种疾病会持续数月甚至数年。溃疡有时生长得非常快,在几天内就会长得很大。一些溃疡愈合后,经常会出现新的溃疡。疼痛综合征,继发感染的增加和病理过程的持续进展导致患者生活质量下降,社会适应能力恶化。因此,及时充分的诊断、治疗和患者的完全依从性是成功的关键。
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Clinical case of pyodermia ulcerosa serpiginosa(pyoderma gangrenosum)
Objective — to highlight the issue of difficulties in the diagnosis and treatment of patients with pyoderma gangrenosum (PG). Materials and methods. A literature review and analysis of the results of the clinical examination of patients with PG was performed. Pyoderma gangrenosum is most commonly observed in young and middle-aged people, more frequently in women. The pathogenesis of PG has not been fully studied. It is believed to be related to genetic mutations, neutrophil dysfunction and impaired immunity/inflammation regulation. Maverakis and others developed diagnostic criteria for PG. First-line treatment is aimed at optimizing the local wound care. For more severe diseases, systemic therapy is required. Oral corticosteroids (0.5—1 mg/kg/day) are the basis of treatment and used for control of PG. Cyclosporine can be used either alone or in combination with corticosteroids. At present, there is an increasing evidence of effectiveness of biological therapy as treatment method for a number of cytokines. Results and discussion. This article presents a clinical case of a patient diagnosed with PG. Based on recent guidelines, systemic corticosteroid therapy (Medrol starting at 48 mg/d), systemic anti-inflammatory and vascular drugs, topical therapy and treatment results were presented. Conclusions. Without treatment, the disease lasts for months and years. Ulcers sometimes grow very fast, reaching huge sizes in a matter of days. After the healing of some ulcers new ones often emerge. Pain syndrome, the addition of a secondary infection and the continuous progression of the pathological process lead to a decrease in the patient’s quality of life, as well as to a deterioration in his social adaptation. Therefore, timely adequate diagnosis, treatment and full compliance of the patient are the key to success.
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