Successful Treatment of Papillon Lefèvre Syndrome with a Combination of Acitretin and Topical-PUVA; A Four Year Follow Up

Abstract

Observations:

Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis, periodontitis which causes premature loss of both deciduous and permanent teeth and recurrent pyogenic infections. Topical keratolytics, antibiotics and retinoids, such as isotretinoin and acitretin, have been used in the treatment of PLS. We report a 5 years old girl with palmoplantar hyperkeratosis and chronic eye infection persisting over 1,5 years. She also defined recurrent skin lesions which began as purple lesions that evolved into abscesses and finally healed with scars on her hips, trunk and scalp. All deciduous teeth were previously lost due to chronic swelling and bleeding of the gums, except for two molars. Acitretin (10 mg/day) in combination with topical-PUVA therapy twice weekly was initiated. Additionally, she also used keratolytic preparation containing 5% salicylic acid in 10% urea and pure vaseline for palmoplantar hyperkeratosis. An improvement on the palmoplantar hyperkeratosis and chronic eye infection was observed after 4 sessions of topical PUVA therapy and within 6 weeks of therapy respectively. Four months after initiating treatment with acitretin, she had new permanent teeth.