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对一名骨髓增生异常综合征患者发生的坏疽性脓皮病的成功治疗。

Successful treatment of pyoderma gangrenosum that developed in a patient with myelodysplastic syndrome.

作者信息

Yamauchi Takahiro, Ishida Kentaro, Iwashima Yoshiko, Ikegaya Satoshi, Kawai Yasukazu, Wakahara Mami, Kumakiri Masanobu, Ueda Takanori

机构信息

First Department of Internal Medicine, Fukui Medical University, 23, Shimoaizuki, Matsuoka, Fukui 910-1193, Japan.

出版信息

J Infect Chemother. 2003 Sep;9(3):268-71. doi: 10.1007/s10156-003-0254-6.

Abstract

We describe the successful treatment of pyoderma gangrenosum (PG) that developed in a patient with myelodysplastic syndrome (MDS). A 63-year-old Japanese man with MDS was admitted to our hospital because of a large skin ulcer on his neck in November 2001. The initial diagnosis was infectious dermatitis, and antimicrobial therapy was performed, using imipenem/cilastatin, isepamicin, and amphotericin B. However, this therapy was not effective, and the lesion worsened. Cultures of blood, throat swab, and ulcer pus yielded no microorganisms. A biopsy of the skin lesion revealed a severe infiltration of neutrophils in the dermis, without any evidence of infection. The lesion was finally diagnosed as PG, and systemic administration of corticosteroid hormone was started in December 2001. The patient was initially pulsed with 1 g methylprednisolone daily for 3 days. The dose was immediately reduced, and the treatment was maintained with 30 mg prednisolone daily. The skin lesion responded markedly to the therapy, and C-reactive protein became negative. The patient was discharged in February 2002 because the lesion was almost cured. Prednisolone administration was tapered after 6-month maintenance therapy. No recurrence of PG was seen, although his MDS transformed into leukemia in April 2003. Only 31 cases of MDS developing PG have been reported in the past 20 years in Japan. This report describes one such rare patient who was successfully treated with the use of high-dose pulse methylprednisolone and long-term maintenance therapy.

摘要

我们描述了一名骨髓增生异常综合征(MDS)患者发生坏疽性脓皮病(PG)后的成功治疗过程。一名63岁的日本男性MDS患者于2001年11月因颈部出现大面积皮肤溃疡入院。初步诊断为感染性皮炎,遂使用亚胺培南/西司他丁、异帕米星和两性霉素B进行抗菌治疗。然而,该治疗无效,病变恶化。血液、咽拭子和溃疡脓液培养均未发现微生物。皮肤病变活检显示真皮中有严重的中性粒细胞浸润,无任何感染迹象。最终病变被诊断为PG,并于2001年12月开始全身使用糖皮质激素。患者最初每天静脉注射1g甲泼尼龙,共3天。剂量立即减少,随后以每天30mg泼尼松龙维持治疗。皮肤病变对治疗反应明显,C反应蛋白转阴。由于病变几乎治愈,患者于2002年2月出院。维持治疗6个月后逐渐减少泼尼松龙用量。尽管他的MDS在2003年4月转化为白血病,但PG未复发。在过去20年里,日本仅报告了31例MDS合并PG的病例。本报告描述了这样一位罕见患者,他通过大剂量脉冲甲泼尼龙和长期维持治疗获得了成功治愈。

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