A Case of Atypical Maxillary Sinusitis Accompanied with Papillon-Lefevre Syndrome / 대한이비인후과학회지
Korean Journal of Otolaryngology - Head and Neck Surgery
;
: 456-459, 2013.
Article
in Korean
| WPRIM
| ID: wpr-645884
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Papillon-Lefevre Disease
/
Periodontitis
/
Killer Cells, Natural
/
Lymphocytes
/
Monocytes
/
Maxillary Sinusitis
/
Incidence
/
Keratoderma, Palmoplantar
/
Maxillary Sinus
Type of study:
Incidence study
/
Prognostic study
Limits:
Humans
Language:
Korean
Journal:
Korean Journal of Otolaryngology - Head and Neck Surgery
Year:
2013
Type:
Article
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