Successful treatment of fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions
Successful treatment of fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions
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DOI：
10.1186/1471-5945-10-10
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发表时间：
2010-10-07
期刊：
影响因子：
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通讯作者：
Bejaoui, Mohamed
中科院分区：
文献类型：
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作者：
Mellouli, Fethi;Ksouri, Habib;Bejaoui, Mohamed
背景：坏疽蜕皮病(EG)是一种严重影响中性粒细胞减少患者的皮肤损害，在免疫功能低下的患者中常与铜绿假单胞菌相关。白细胞黏附缺陷I(LAD I)是一种常染色体隐性遗传性疾病，属于先天免疫的主要免疫缺陷综合征之一，其严重表型包括中性粒细胞CD18表达完全缺陷、脐带分离延迟、主要由反复的细菌和真菌感染引起的慢性皮肤溃疡、循环中的大量中性粒细胞增多以及感染部位异常低的中性粒细胞聚集。病例介绍：据我们所知，我们报告了第一例感染LAD-1的儿童，她在病程中经历了由茄病镰刀菌引起的多细菌和真菌EG损害。尽管有针对性的抗生素和抗真菌治疗，但病变持续时间长达18个月，只有大量粒细胞袋输注联合G-CSF才有能力治愈这种病变。结论：粒细胞袋输注有利于EG的治愈，尤其是在免疫功能严重受损的患者。
Background: Ecthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme neutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients. Leukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of the innate immunity, is an autosomal recessive disorder characterized in its severe phenotype by a complete defect in CD18 expression on neutrophils, delayed cord separation, chronic skin ulcers mainly due to recurrent bacterial and fungal infections, leucocytosis with high numbers of circulating neutrophils and an accumulation of abnormally low number of neutrophils at sites of infection.Case Presentation: We report at our knowledge the first case of a child affected by LAD-1, who experienced during her disease course a multi-bacterial and fungal EG lesion caused by fusarium solani. Despite targeted antibiotics and anti-fungi therapy, the lesion extended for as long as 18 months and only massive granulocytes pockets transfusions in association with G-CSF had the capacity to cure this lesion.Conclusion: We propose that granulocytes pockets transfusions will be beneficial to heal EG especially in severely immunocompromised patients.