A Case of Atypical Maxillary Sinusitis Accompanied with Papillon-Lefevre Syndrome.
10.3342/kjorl-hns.2013.56.7.456
- Author:
Taejung PARK
1
;
Boyoung KIM
Author Information
1. Department of Otorhinolaryngology, Maryknoll Medical Center, Busan, Korea. entkby@naver.com
- Publication Type:Case Report
- Keywords:
Maxillary sinusitis;
Papillon-Lefevre syndrome
- MeSH:
Humans;
Incidence;
Keratoderma, Palmoplantar;
Killer Cells, Natural;
Lymphocytes;
Maxillary Sinus;
Maxillary Sinusitis;
Monocytes;
Papillon-Lefevre Disease;
Periodontitis
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
2013;56(7):456-459
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
