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坏疽性脓皮病的临床特征:病例系列和文献回顾。

Clinical characteristics of pyoderma gangrenosum: Case series and literature review.

机构信息

Department of Dermatology, Beijing Chao-yang Hospital, Capital Medical University, Beijing, China.

出版信息

Medicine (Baltimore). 2024 Sep 13;103(37):e39634. doi: 10.1097/MD.0000000000039634.

Abstract

BACKGROUND

Pyoderma gangrenosum (PG) is a neutrophilic skin disease characterized by recurrent painful cutaneous ulcers, often accompanied by inflammatory bowel disease, joint pain, and other systemic damage. This disease is relatively rare in clinical practice and its diagnosis and treatment are often delayed, leading to secondary infections in the skin lesions, prolonged disease course, and increased disease burden on patients. This study retrospectively analyzed the clinical characteristics and treatment strategies of patients with PG admitted to our hospital and conducted a literature review, in order to improve the understanding of the disease among clinical doctors, enable patients to receive better diagnosis and treatment, and ultimately improve patient prognosis.

METHODS

Clinical data of patients diagnosed with PG and hospitalized in Beijing Chaoyang Hospital, Capital Medical University from January 2014 to December 2022 were retrospectively collected. The clinical manifestations, treatment strategies, efficacy, and disease outcomes were analyzed.

RESULTS

A total of 14 patients, including 8 males and 6 females, aged 14 to 66 years, were included. Skin lesion types: 13 cases were ulcer-type, 1 case was pustule combined with ulcer-type, and the lower limbs were the most commonly affected areas. All the 14 patients presented with comorbidities. All patients were treated with glucocorticoids, with a daily dose equivalent to 20 to 100 mg prednisone and a median dose of 40 mg. Among them, 3 patients were treated with minocycline in combination, 1 patient was treated with mycophenolate mofetil 0.5 twice daily in combination, 1 patient was treated with cyclophosphamide 0.1 once daily in combination, and 1 patient was treated with thalidomide 0.1 every night in combination.

CONCLUSION

PG is a relatively rare immune-related skin disease. Our small sample data analysis found that male PG is not uncommon in the Chinese population. Systemic glucocorticoids can quickly control the symptoms of PG in most patients with PG. In patients with poor efficacy or limited use of glucocorticoids, immunosuppressive drugs or novel targeted drugs such as biologics or small-molecule drugs should be used in combination as early as possible. Skin lesion care focuses on preventing infection, avoiding surgical debridement, and emphasizing pain management and the symptomatic treatment of comorbidities.

摘要

背景

坏疽性脓皮病(PG)是一种以反复发作的疼痛性皮肤溃疡为特征的中性粒细胞皮肤病,常伴有炎症性肠病、关节痛和其他全身损害。这种疾病在临床上相对少见,其诊断和治疗往往被延误,导致皮肤病变继发感染,疾病病程延长,患者疾病负担增加。本研究回顾性分析了我院收治的 PG 患者的临床特征和治疗策略,并进行了文献复习,以期提高临床医生对该病的认识,使患者得到更好的诊断和治疗,最终改善患者预后。

方法

回顾性收集 2014 年 1 月至 2022 年 12 月首都医科大学附属北京朝阳医院收治的 PG 患者的临床资料,分析其临床表现、治疗策略、疗效和疾病转归。

结果

共纳入 14 例患者,男 8 例,女 6 例,年龄 14~66 岁。皮损类型:溃疡型 13 例,脓疱并溃疡型 1 例,下肢最常见。14 例患者均合并基础疾病。所有患者均接受糖皮质激素治疗,等效泼尼松日剂量 20~100mg,中位剂量 40mg。其中 3 例联合应用米诺环素,1 例联合应用吗替麦考酚酯 0.5 次/d,1 例联合应用环磷酰胺 0.1 次/d,1 例联合应用沙利度胺 0.1 次/晚。

结论

PG 是一种相对少见的免疫相关性皮肤疾病。本小样本数据分析发现,中国人群中男性 PG 并不少见。全身糖皮质激素治疗能快速控制多数 PG 患者的 PG 症状。对于疗效不佳或糖皮质激素受限的患者,应尽早联合应用免疫抑制剂或新型靶向药物,如生物制剂或小分子药物。皮肤病变护理重点在于预防感染,避免外科清创,强调疼痛管理和合并症的对症治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70eb/11404947/56593a601295/medi-103-e39634-g001.jpg

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