Papillon-Lefèvre syndrome.
Papillon- Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinization characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition. The exact pathomechanism of these clinical events mainly remains speculative. This paper describes two cases of PLS with classic clinical features and briefly review the relevant literature.
Mahajan
Vikram K
VK
Department of Dermatology, Indira Gandhi Medical College, Shimla, India.
Thakur
Narbir S
NS
Sharma
Nand Lal
NL
D000015:Abnormalities, Multiple / Q000175:diagnosis; D000293:Adolescent; D002648:Child; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007194:India; D010214:Papillon-Lefevre Disease / Q000175:diagnosis* / Q000235:genetics*; D010808:Physical Examination; D011379:Prognosis; D018570:Risk Assessment; D012720:Severity of Illness Index