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

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 i...

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Publicado en:BMC Dermatology Vol. 10; pp. 10 - 11
Autores principales: Mellouli F, Ksouri H, Barbouche R, Maamer M, Hamed LB, Hmida S, Hassen AB, Béjaoui M, Mellouli, Fethi, Ksouri, Habib, Barbouche, Ridha, Maamer, Mongi, Hamed, Leila Ben, Hmida, Slama, Hassen, Assia Ben, Béjaoui, Mohamed
Formato: case study Journal Article
Publicado: BioMed Central 2010
Acceso en línea:Ver este registro en EBSCOhost
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      dt: 2010
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      pub: BioMed Central
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        10.1186/1471-5945-10-10
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        atl: Successful treatment of Fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions.
      aug:
        au:
          Mellouli F
          Ksouri H
          Barbouche R
          Maamer M
          Hamed LB
          Hmida S
          Hassen AB
          Béjaoui M
          Mellouli, Fethi
          Ksouri, Habib
          Barbouche, Ridha
          Maamer, Mongi
          Hamed, Leila Ben
          Hmida, Slama
          Hassen, Assia Ben
          Béjaoui, Mohamed
        affil: Service d'Immuno-Hématologie Pédiatrique, Centre National de Greffe de Moelle Osseuse, 2 rue Jebel Lakdhar, Tunis, Tunisia
      sug:
        subj:
          Pyoderma Immunology
          Pyoderma Microbiology
          Pyoderma Therapy
          Fungi
          Gangrene Immunology
          Gangrene Microbiology
          Gangrene Therapy
          Granulocytes Transplantation
          Child
          Female
          Immunocompromised Host
          Immunologic Deficiency Syndromes Complications
          Immunologic Deficiency Syndromes Immunology
          Neutropenia Complications
          Neutropenia Immunology
          Child: 6-12 years
          Female
      ab: 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.
      pubtype: Academic Journal
      doctype:
        case study
        Journal Article
      ougenre: Article
    language: English
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