title: Papillon-Lefèvre syndrome in four siblings treated with etretinate. A nine-year evaluation.
abstract: Four siblings with Papillon-Lefèvre syndrome (3 boys and 1 girl), aged 8 to 12 at time of first diagnosis in 1978 are reported. These four patients represent the second largest sibship reported in the literature, and the only familial cases treated with etretinate for 6 years with an additional 3 1/2 year follow-up evaluation. No long-term side effects of etretinate were found in the children. All four patients are the product of a second cousin marriage; all demonstrate the B5 locus on HLA typing.
authors.affiliation: Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: R E
authors.initials: RE
authors.lastname: Kellum
mesh_terms: D002648:Child; D005050:Etretinate / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007645:Keratoderma, Palmoplantar; D008297:Male; D010214:Papillon-Lefevre Disease / Q000188:drug therapy* / Q000235:genetics
chemical_list: D005050:Etretinate