A Case of Atypical Maxillary Sinusitis Accompanied with Papillon-Lefèvre Syndrome

Abstract

Received January 17, 2013 Revised March 29, 2013 Accepted April 3, 2013 Address for correspondence Boyoung Kim, MD Department of Otorhinolaryngology, Maryknoll Medical Center, 121 Junggu-ro, Jung-gu, Busan 600-730, Korea Tel +82-51-465-2205 Fax +82-51-461-0297 E-mail entkby@naver.com Papillon-Lefevre syndrome (PLS) is an extremely rare autosomal recessive disorder characterized by palmoplantar keratoderma and periodontitis and occuring with an estimated incidence of 1-4 cases per million. Patients with PLS are highly susceptible to infection. The etiology of an infective susceptibility is unknown; however, an association with defects in neurophil dysfunction, insufficient lymphocyte response to pathogens, defects in monocyte functions and impairment of NK cell cytotoxic function has been suggested. To our knowledge, this is the first case report of atypical maxillary sinusitis accompanied by PLS, and we represent the case with a review of the related literatures. Korean J Otorhinolaryngol-Head Neck Surg 2013;56:456-9 Key WordsZZMaxillary sinusitis ᆞPapillon-Lefevre syndrome.