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[一名原因不明的髓样化生患者的急性发热性嗜中性皮病(Sweet综合征)]

[Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with agnogenic myeloid metaplasia].

作者信息

Suzuki K, Utsumi H, Yamamoto K, Kamiyama T, Szuzki T, Miura O, Maruyama Y, Kato A, Murakami N, Murohashi I

出版信息

Rinsho Ketsueki. 1989 Feb;30(2):202-6.

PMID:2746875
Abstract

A 60-year-old man with a one-year history of agnogenic myeloid metaplasia was admitted to the hospital because of fever and a skin eruption. He had fever, anemia, hepatosplenomegaly, and a raised painful erythematous plaque in the face. The same kind of skin lesion developed thereafter at a venipuncture site in the left forearm. Bacterial cultures were negative. There was no response to antibiotic treatment. A biopsy specimen of the skin lesion revealed a dense dermal infiltration with mature neutrophils. A diagnosis of Sweet's syndrome was made. Fever and skin eruptions responded rapidly to prednisolone (PSL). Although the disease frequently recurred on rapid tapering of PSL, skin lesions cleared without scarring on a prolonged course of PSL. Four months after withdrawal of PSL, Sweet's syndrome recurred. A high dose PSL was given without benefit. He died of disseminated candidiasis.

摘要

一名患有特发性骨髓化生一年的60岁男性因发热和皮疹入院。他有发热、贫血、肝脾肿大,面部有一个凸起的疼痛性红斑斑块。此后,左前臂静脉穿刺部位出现了同样的皮肤病变。细菌培养结果为阴性。抗生素治疗无效。皮肤病变的活检标本显示真皮有密集的成熟中性粒细胞浸润。诊断为Sweet综合征。发热和皮疹对泼尼松龙(PSL)反应迅速。尽管在PSL快速减量时疾病经常复发,但在PSL长期治疗过程中皮肤病变消退且无瘢痕形成。停用PSL四个月后,Sweet综合征复发。给予高剂量PSL无效。他死于播散性念珠菌病。

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