papillon-lefèvre syndrome responds to etretinate

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Papillon-Lefevre syndrome responds to etretinate The only familial cases treated for 6 years

The rare autosomal recessive genodermatosis, Papillon-Lelevre syndrome, was diagnosed in 4 siblings, representing the second largest sibship reported and the only familial cases that were treated with etretinate for 6 years (with over 3 years follow-up). The patients were aged between 8 and 12 years at the time of diagnosis, and received etretinate doses of 21-27 mg/day.

All the patients showed remarkable improvements in palmar and plantar hyperkeratosis, and clearing of keratotic plaques within 2-3 months. Improvements were maintained on maintenance doses of etretinate, but the hyperkeratotic cutaneous 'abnormalities recurred when the drug was discontinued. However, cutaneous abnormalities did not worsen after the age of 15 when etretinate was discontinued. Inflammatory gingival disease did not continue, although response was hard to assess. Serum triglyceride levels were elevated on several occasions. Kellum RE. Papillon-Lefevre syndrome in four siblings treated with etrinate. International Journal of Dermatology 28: 605-608, Nov 1989

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20 Jan 1990 0156-2703/90/0120-0012/0$01.00/0 © ADIS Press