Papillon-Lefèvre syndrome, inherited as an autosomal recessive trait, is characterized by an erythematous palmoplantar keratoderma, and a periodontosis associated with the loss of teeth early in life.
The keratoderma is apparent before age 6 and extends up the sides of the feet.
- All stigmata, including the periodontosis, can be improved by the long term use of an oral retinoid, such as isotretinoin or acitretin.
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