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      Papillon-Lefèvre Syndrome: A Series of Six Cases in the Same Family

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          Abstract

          Papillon-Lefèvre syndrome (PLS) is a rare, autosomal recessive heterogeneous disorder, which is characterized by palmoplantar hyperkeratosis, early loss of primary and permanent teeth, and associated calcification of the dura mater. Herein we described six cases of PLS in the same family. In this series, six cases (two females and four males) with the mean age of 15.6 ± 10.4 years were recruited. Palmoplantar hyperkeratosis was detected in all of the cases, leading to a difficult and painful walking in two cases due to lesions on the soles. Skin lesions were sharply distinct from adjacent normal skin in all cases. Other skin lesions were located in the external malleolus (5/6), knee (4/6), elbow (4/6), toe and dorsal fingers (3/6), and the thighs (2/6). In three cases, all permanent teeth were exfoliated. In three others, no primary teeth remained. Severe gingivitis was observed in three patients. Radiologic study confirmed alveolar bone destruction in five cases. Delayed diagnosis and insufficient treatment of PLS patients can affect patient's life of by causing edentulism at a young age and may impose PLS patients to increased risk of social, psychological, and economical burdens.

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          Most cited references16

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          THE SYNDROME OF PALMAR-PLANTAR HYPERKERATOSIS AND PREMATURE PERIODONTAL DESTRUCTION OF THE TEETH. A CLINICAL AND GENETIC ANALYSIS OF THE PAPILLON-LEF'EVRE SYNDROME.

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            Papillon-Lefèvre syndrome.

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              Papillon-Lefèvre syndrome: a review of the literature and report of 4 cases.

              This report describes 4 cases of Papillon-Lefèvre syndrome (PLS) affecting 2 Jordanian families with a total of 8 children. In family A, 3 girls out of 6 siblings were affected; and in family B, the eldest of 2 brothers. The patients were between 4 1/2 and 12 years old, and their parents, who are first cousins, were not affected. Diagnosis was determined by history, and clinical, laboratory, and radiographic examinations. All present cases exhibited the typical clinical features of PLS: hyperkeratosis of the palms and soles and the presence of advanced destructive periodontitis resulting in early loss of both the primary and permanent dentitions. In Case 4, cutaneous lesions were noticed at age 6 months and the child was edentulous by the age of 12 years. In all cases, there was a relationship between increased severity of skin lesions and seasonal variations and intensified periodontal destruction. There was an early eruption of the permanent teeth. The teeth were caries-free with no sign of root resorption. Dentists play a significant role in the diagnosis and management of PLS patients.
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                Author and article information

                Journal
                ISRN Dermatol
                ISRN Dermatol
                ISRN.DERMATOLOGY
                ISRN Dermatology
                International Scholarly Research Network
                2090-4592
                2090-4606
                2012
                3 December 2012
                : 2012
                : 139104
                Affiliations
                1Division of Gastroenterology and Hepatology, School of Medicine, The Johns Hopkins University, 1800 Orleans Street, Sheikh Zayed Building, Room 7125 B, Baltimore, MD 21287, USA
                2Aliabad-e-Katoul Hygiene Center, Golestan University of Medical Sciences, Gorgan 4934174515, Iran
                3Zainaldin Martyr Research Center, Gorgan University of Agricultural Sciences and Natural Resources, P.O. Box 15739-49138, Gorgan, Iran
                4Department of Dermatology, Golestan University of Medical Sciences, Gorgan 4934174515, Iran
                5Department of Dermatology, Tehran University of Medical Sciences, P.O. Box 14155-6447, Tehran, Iran
                Author notes
                *Ali Kord Valeshabad: ali_kord2006@ 123456yahoo.com

                Academic Editors: F. Guarneri and C.-C. Lan

                Article
                10.5402/2012/139104
                3518962
                23251811
                c3cbdea3-ae97-457c-b13c-aee86cb671f3
                Copyright © 2012 Ali Kord Valeshabad et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 19 October 2012
                : 7 November 2012
                Categories
                Clinical Study

                Dermatology
                Dermatology

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