Premium
Papillon‐Lefèvre Syndrome
Author(s) -
Landow R. Kenneth,
Cheung Herman,
Bauer Marjorie
Publication year - 1983
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/j.1365-4362.1983.tb03359.x
Subject(s) - medicine , hyperkeratosis , dermatology , calcification , autosomal recessive inheritance , oral hygiene , pathology , dentistry , genetics , biology , gene
A patient, with Papillon‐Lefevre syndrome, presented with a premature loss of both deciduous and permanent teeth and hyperkeratosis palmaris et plantaris. Other abnormalities Such as psoriasiform hyperkeratosis. calcification of the falx, and frequent infections can be seen. Although no etiologic factor is known for this aberration, an autosomal recessive inheritance is established. Treatment usually consists of dental hygiene only since dermatologic therapy is usually unrewarding. A new finding resulted when fibroblasts from involved gingiva were compared with either uninvolved gingiva or controlled gingiva. The protein and collagen synthesis of the involved tissue was about twice that of the latter two groups.