@ARTICLE{MOIN, author = {MOIN, M and FARHOUDI, ABOLHASAN and MOIN, ATHAR and }, title = {PAPILLON-LEFE'VRE SYNDROME: NEUTROPHIL MOTILITY AND KILLING DEFECT IN A CHILD WITH RECURRENT SEVERE INFECTIONS}, volume = {11}, number = {2}, abstract ={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. }, URL = {http://mjiri.iums.ac.ir/article-1-1122-en.html}, eprint = {http://mjiri.iums.ac.ir/article-1-1122-en.pdf}, journal = {Medical Journal of the Islamic Republic Of Iran}, doi = {}, year = {1997} }