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.
Highlights
Papillon-Lefevre syndrome (PLS) is a rare, autosomal recessive heterogeneous disorder, which was first described by Papillon Lefevre in 1924 [1]
PLS is characterized by palmoplantar hyperkeratosis, early loss of primary and permanent teeth, and associated calcification of the dura mater [2, 3]
We described six cases of PLS within the same family
Summary
Papillon-Lefevre syndrome (PLS) is a rare, autosomal recessive heterogeneous disorder, which was first described by Papillon Lefevre in 1924 [1]. PLS is characterized by palmoplantar hyperkeratosis, early loss of primary and permanent teeth, and associated calcification of the dura mater [2, 3]. Palmoplantar lesions are usually presented during the time of tooth eruption between the ages of six months to three years. Periodontal involvement is typically presented immediately after tooth eruption, accompanied by severe gingival inflammation, leading to exfoliation of primary teeth by age of four to five [1, 5]. Gingival inflammation is typically revealed after primary teeth exfoliation and is recurrent as the permanent teeth erupt. Most of the previous studies reported one to three cases of PapillonLefevre syndrome [8–10]. In this series, we described six cases of PLS within the same family
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
