Papillon-Lefevre syndrome: a case report.
We report the first diagnosed case of Papillon-Lefevre syndrome in Thailand. The patient is the youngest child of consanguinous parents, and she has had symmetrical hyperkeratotic plaques on both plantar surfaces since birth with a history of chronic gingivitis, periodontitis, and premature loss of primary dentition. The histologic study revealed compact hyperkeratosis with epidermal acanthosis. Radiologic studies of the skull were normal. The radiographic panoramic view of the oral cavity revealed generalized severe vertical and horizontal alveolar bone loss. The immunologic analysis of polymorphonuclear leukocyte phagocytic function by nitrobluetetrazolium test (NBT test) showed decreasing response to latex stimulation. Serum parathyroid hormone, calcium, phosphate, and alkaline phosphatase levels were within normal limits. The skin lesions were temporary relieved with topical keratolytic agents. The oral lesions were improved by the extraction of hopeless teeth and conventional periodontal treatments.
Pratchyapruit
Walai-orn
WO
Kullavanijaya
Preya
P
D000293:Adolescent; D003241:Consanguinity; D005260:Female; D006801:Humans; D010214:Papillon-Lefevre Disease / Q000175:diagnosis* / Q000235:genetics; D010375:Pedigree; D010510:Periodontal Diseases / Q000175:diagnosis* / Q000628:therapy; D013785:Thailand