Background: Ecthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme\r\nneutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients.\r\nLeukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of the innate\r\nimmunity, is an autosomal recessive disorder characterized in its severe phenotype by a complete defect in CD18\r\nexpression on neutrophils, delayed cord separation, chronic skin ulcers mainly due to recurrent bacterial and fungal\r\ninfections, leucocytosis with high numbers of circulating neutrophils and an accumulation of abnormally low\r\nnumber of neutrophils at sites of infection.\r\nCase Presentation: We report at our knowledge the first case of a child affected by LAD-1, who experienced\r\nduring her disease course a multi-bacterial and fungal EG lesion caused by fusarium solani. Despite targeted\r\nantibiotics and anti-fungi therapy, the lesion extended for as long as 18 months and only massive granulocytes\r\npockets transfusions in association with G-CSF had the capacity to cure this lesion.\r\nConclusion: We propose that granulocytes pockets transfusions will be beneficial to heal EG especially in severely\r\nimmunocompromised patients.
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