[The Papillon-Lefevre syndrome (keratosis palmoplantaris with periodontopathy). Treatment with etretinate].
The Papillon-Lefèvre syndrome is an autosomal recessive gene defect characterized by transgredient palmoplantar hyperkeratosis and periodontopathia leading to loss of the teeth. The syndrome is described in a 28-year-old man who had lost all of his teeth at the age of 10 years. Treatment with etretinate resulted in a marked improvement of the palmar and plantar skin lesions.
Wehrmann
W
W
Traupe
H
H
Happle
R
R
D000328:Adult; D005050:Etretinate / Q000627:therapeutic use*; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000188:drug therapy*; D008297:Male; D010214:Papillon-Lefevre Disease / Q000188:drug therapy* / Q000473:pathology; D012867:Skin / Q000473:pathology
D005050:Etretinate
Papillon-Lefèvre-Syndrom (Keratosis palmoplantaris mit Periodontopathie). Behandlung mit Etretinat.