Papillon - Lefèvere Syndrome: A Case Report
Mohammed S. Al-Dhubaibi *
Department of Dermatology, College of Medicine, Qassim University, Buraidah, KSA.
Hamdy Metwaly
Department of Oral Pathology, Faculty of Dentistry, Tanta University, Tanta, Egypt and Department of Oral and Maxillofacial Surgery and Diagnostic Sciences, College of Dentistry, Qassim University, Buraidah, KSA.
Amr Elkarargy
Department of Oral Medicine and Periodontology, College of Dentistry, Qassim University, Buraidah, KSA.
*Author to whom correspondence should be addressed.
Abstract
Papillon-Lefèvere Syndrome (PLS) is one of the rare autosomal recessive disorders of keratinization (1-4 cases per million). It is inherited as an autosomal-recessive condition with one-third of the patients showing consanguinity of the parents and diagnosed in both sexes. Lesions are characterized by palmo-plantar hyperkeratosis and severe destructive periodontal disease affecting the primary and permanent teeth. This paper reports a case of a 13 -year-old African boy presented to the outpatient clinic of Qassim University with diffuse keratotic plaques of his soles extending to the dorsal surface, localized keratotic plaques of his palm and severe inflammation of the gingiva with premature teeth loss and looseness of remaining teeth.
Keywords: Papillon-Lefevre syndrome, severe periodontitis, autosomal