Successful treatment of fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions
نویسندگان
چکیده
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.
منابع مشابه
Use of honey as a treatment for severe echtyma gangrenosum
For more than 25 years, honey has been employed to facilitate the healing of complex wounds with excellent results. Here, we report the case of a two-and-a-half-year-old agammaglobulinemia patient admitted in our unit for severe sepsis with extensive skin necrosis in the groin, penis and scrotum. An Ecthyma Gangrenosum was corroborated by the isolation of Pseudomonas aeruginosa in wound s...
متن کاملPeriodontal Involvement in Leukocyte Adhesion Deficiency: Review of the Literature and a Case Report
Objective: Leukocyte adhesion deficiency(LAD) is a scarce, autosomal recessive inherited disorder . LAD-I which is the most common type occurs due to mutations on the CD18 gene. This mutation leads to absence or severe reduction of leukocyte cell surface expression of ß2 integrin molecules which are necessary for the adhesion of the leukocytes to the endothelial cells, transendothelial mi...
متن کاملLeukocyte Adhesion Deficiency Type 1 Presenting as Leukemoid Reaction
The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over las...
متن کاملگزارش یک مورد سندرم نقص چسبندگی لکوسیتی نوع یک همراه با زمان مناسب افتادن بند ناف
Introduction: Leukocyte adhesion deficiency type 1 is a rare disorder of function of neutrophils which presents with recurrent bacterial and fungal infections. The patients usually have a history of delayed umbilical cord detachment. Case Report: The patient was a fourteen-month-old boy with recurrent bronchopneumonia, skin abscess and oral candidiasis. There was a history of abscess formati...
متن کامل