Volume 11, Issue 2 (8-1997)                   Med J Islam Repub Iran 1997 | Back to browse issues page

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MOIN M, FARHOUDI A, MOIN A. PAPILLON-LEFE'VRE SYNDROME: NEUTROPHIL MOTILITY AND KILLING DEFECT IN A CHILD WITH RECURRENT SEVERE INFECTIONS. Med J Islam Repub Iran. 1997; 11 (2) :149-152
URL: http://mjiri.iums.ac.ir/article-1-1122-en.html
From the Department of Clinical Immunology and Allergy, Children's Medical Center, Tehran University of Medical Sciences, Tehran
Abstract:   (2512 Views)
A case of palmoplantar hyperkeratosis with periodontosis and a history of recurrent severe pyoderma, pneumonia and multiple liver abscesses is described in a 12 year old girl. The patient demonstrated neutrophil dysfunction characterized by decreased random migration and chemotaxis and defective bactericidal activity. The exact immunopathological mechanism for susceptibility to infections in Papillon-Lefe'vre syndrome patients still remains to be determined. However, the mode of clinical presentation, laboratory findings and response to retinoid treatment, all support the speculation of Papillon-Lefe'vre syndrome as a primary immunologic disease with a variable defect in neutrophil motility and bactericidal activity. The pattern of clinical presentations as skin and periodontal lesions alone or with susceptibility to infection in other sites will change accordingly.
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Type of Study: case report | Subject: Immunology

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