Korean J Otorhinolaryngol-Head Neck Surg.
2013 Jul;56(7):456-459. 10.3342/kjorl-hns.2013.56.7.456.
A Case of Atypical Maxillary Sinusitis Accompanied with Papillon-Lefevre Syndrome
- Affiliations
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- 1Department of Otorhinolaryngology, Maryknoll Medical Center, Busan, Korea. entkby@naver.com
- KMID: 2277787
- DOI: http://doi.org/10.3342/kjorl-hns.2013.56.7.456
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.
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