Successful treatment of fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions

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.We propose that granulocytes pockets transfusions will be beneficial to heal EG especially in severely immunocompromised patients.Leukocyte adhesion deficiency I (LAD I) is an autosomal recessive disorder characterized by defect in CD18 expression on neutrophils, leading to leucocytosis with high numbers of circulating neutrophils and an accumulation of abnormally low number of neutrophils at sites of infection. Recurrent bacterial and fungal infections count among clinical complications of such disorder [1]. Nonetheless, ecthyma gangrenosum (EG) which represents a cutaneous infection mainly associated with pseudomonal sepsis in immunocompromised patient suffering extreme neutropenia [2,3], was never reported in the setting of LAD I.We report a case of a child affected by LAD-1, who experienced a fusarium solani EG lesion. 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.A 9 years old girl affected by LAD-1 developed after a fall an abscess on her knee. Cultures taken from the lesion revealed a Pseudomonas aeruginosa infection. She was treated with amikacine 400 mg/day and imipenem 400 × 2 mg/day for three weeks and local disinfection. Lesion cultures performed one month later were negative. As all patients affected by such immunodeficiency, she was constantly under prophylactic treatment [25 mg/Kg/2 days (sulfamethoxazole + trimetoprime) and 10 mg/Kg/day Itraconazole].Three months later, the patient was readmitted with 7 cm diameter erythematous and edematous lesion around