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Papillon‐Lefèvre Syndrome in Four Siblings Treated with Etretinate A Nine‐Year Evaluation
Author(s) -
Kellum Robert E.
Publication year - 1989
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.1365-4362.1989.tb02539.x
Subject(s) - etretinate , medicine , girl , pediatrics , cousin , dermatology , developmental psychology , psychology , history , archaeology , psoriasis
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.