Table of Contents
ISRN Dermatology
Volume 2012, Article ID 139104, 4 pages
http://dx.doi.org/10.5402/2012/139104
Clinical Study

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

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

Received 19 October 2012; Accepted 7 November 2012

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

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.

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 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.