Papillon – Lefevere Syndrome is characterized by a group of manifestations that causes Palmar-Plantar Keratosis. Its an autosomal disorder meaning that its inherited in families. However, the parents doesn’t usually show the symptoms, but their kids do!
Papillon – Lefevere Syndrome is characterized by dermatologic & oral manifestations. The dermatologic symptoms appear in the first 3 years of life & characterized by Palmar-Plantar Keratosis which is white hands and feet, this is where its most commonly appear. On the other hands, the whiteness can also occur in other areas like the legs, thighs, & trunk. The lesions usually appear as white, light, yellow, brown, or red plaques and patches that develop crusts, cracks, or deep fissures within the skin.
The oral manifestation on the other hands is the dramatic advanced and severe periodontitis that is seen in both the primary & permanent dentitions. Its characterized by rapid loss of bone that supports the teeth resulting in mobility & loss of the teeth eventually.
The presence of both manifestations is the key element for the diagnosis of Papillon – Lefevere Syndrome. Its of great importance that the dentist should take full appropriate family history of the patient along with proper examination to facilitate the treatment.
There is many theories and concepts for the treatment of Papillon – Lefevere Syndrome, & not all of them do work in all patients. It relies on the response of the patient afterall.
The skin manifestations are easily treated with Retinoids, such as etretinate, acitretin, & isotretinoin that should be prescribed by your Dermatologist. However, the oral manifestations are often of concern. Some times they do respond to the retinoids treatment as have been observed by the slowing down of the progression of the disease. Some times however, antibiotic therapy might work instead.
At the end, a rigorous oral hygiene, chlorhexidine mouth rinses, with frequent professional prophylaxis are all necessary for the long-term maintenance and its up to the patient response to the therapy!
Reference: Oral & Maxillofacial Pathology, Fourth Edition, by Neville, Damm, Allen, & Chi