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以坏疽性脓皮病为表现的毛细胞白血病 1 例报告。

Hairy cell leukemia presenting with Ecthyma Gangrenosum- a case report.

机构信息

Department of Internal Medicine, St Antonius Hospital, Nieuwegein/ Utrecht, Koekoekslaan 1, 3435, CM, Nieuwegein, The Netherlands.

Department of Medical Microbiology an Immunology, St Antonius Hospital, Nieuwegein/Utrecht, The Netherlands.

出版信息

BMC Infect Dis. 2019 Jan 25;19(1):85. doi: 10.1186/s12879-018-3644-1.

Abstract

BACKGROUND

Ecthyma gangrenosum is a cutaneous infectious usually associated with P. aeruginosa. It usually develops In patients with an underlying immunodeficiency.

CASE PRESENTATION

A 50-year old mentally disabled white male with a history of epilepsy presented with fever and a painless red macule on his right arm which rapidly progressed to a painful ulcer. Blood and lesion cultures revealed P.aeruginosa, confirming our clinical diagnosis of ecthyma gangrenosum. Subsequently an underlying immune deficit was found, namely patient was diagnosed with hairy-cell leukemia. Despite adequate antibiotics no infection control could be achieved. After treating the underlying immune deficit as well, the infection and hairy-cell leukemia resolved completely.

CONCLUSION

Ecthyma gangrenosum is an important cutaneous infection to recognize, because it is it is typically associated with P.aeruginosa bacteremia. Recognizing this skin leasion should prompt empiric antimicrobial therapy including an agent with antipseudomonal activity. Furthermore, just like in our case, the presence of ecthyma gangrenosum can signal the presence of an occult immune deficit, warranting further investigation.

摘要

背景

坏疽性脓皮病是一种皮肤感染,通常与铜绿假单胞菌有关。它通常发生在有潜在免疫缺陷的患者中。

病例介绍

一名 50 岁的白人男性,智力残疾,有癫痫病史,发热,右上肢无痛性红斑,迅速发展为疼痛性溃疡。血液和病变培养显示铜绿假单胞菌,证实了我们对坏疽性脓皮病的临床诊断。随后发现潜在的免疫缺陷,即患者被诊断为毛细胞白血病。尽管使用了适当的抗生素,仍无法控制感染。在治疗潜在的免疫缺陷后,感染和毛细胞白血病完全消退。

结论

坏疽性脓皮病是一种需要识别的重要皮肤感染,因为它通常与铜绿假单胞菌菌血症有关。识别这种皮肤病变应促使进行经验性抗菌治疗,包括具有抗假单胞菌活性的药物。此外,就像我们的病例一样,坏疽性脓皮病的存在可能表明存在隐匿性免疫缺陷,需要进一步调查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c1f4/6346580/c73f1d4fe9df/12879_2018_3644_Fig1_HTML.jpg

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