Papillon-lefevre syndrome: a combined approach from the dermatologist and dentist - a clinical presentation.

[papillon-lefÃ¨vre syndrome]

Papillon-Lefevre syndrome (PLS ) is a rare disease characterized by skin lesions , which includes palmar -plantar hyperkeratosis and hyperhidrosis with severe periodontal destruction involving both the primary and the permanent dentitions . It is transmitted as an autosomal-recessive condition , and consanguinity of parents is evident in about one -third of the cases . This paper describes a 13 -year -old male patient who presented to the department of pedodontics , with rapidly progressing periodontitis . A general physical examination revealed scaling on the hands and feet , which had been medically diagnosed as PLS . The incidence of this rare entity is increasing in the recent times , which is associated with irreparable periodontal destruction at an early age , with not so prominent skin lesions in some cases . In such instances , the dentist has a more important role in diagnosing , treatment planning and preservation of the periodontal tissues and , at the same time , referring for the treatment of the skin lesions . This paper emphasizes the combined effort of the two specialities in order to maintain skin as well as dental conditions in health by early intervention and a synergistic treatment approach .

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Diseases presenting "which includes palmar-plantar hyperkeratosis and hyperhidrosis with severe periodontal destruction involving both the primary and the permanent dentitions" symptom

papillon-lefÃ¨vre syndrome

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