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ISSN 1405-1699 (Print)
Órgano Oficial del Colegio Mexicano de Alergia, Asma e Inmunología Pediátrica y de la Asociación Latinoamericana de Pediatría

1999, Number 6

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Alerg Asma Inmunol Pediatr 1999; 8 (6)

Papillon-Lefèvre syndrome

Jiménez-Córdoba I, Correa-Bautista Y, Hernández-Bautista V, Berrón-Pérez R

Language: Spanish
References: 26
Page: 170-173
PDF size: 245.89 Kb.


ABSTRACT

The connection between palmar plantar hyperkeratosis and severe periodontal disease was first reported in 1924 by Papillon and Lefèvre. Papillon-Lefèvre syndrome (PLS) is described as the association of palmar-plantar hyperkeratosis with precocious periodontal disease which results in exfoliation of primary and permanent dentitions. Periodontitis resulting from Papillon-Lefèvre syndrome has been known to cause early loss of primary dentition with subsequent involvement of the permanent dentition. The 2 major components of this syndrome (PLS) can also occur as distinct entities. The peripheral blood neutrophil chemotaxis and spontaneous migration were depressed in all patients with Papillon-Lefèvre syndrome. The decreased chemotaxis of peripheral blood neutrophils strongly suggests that the neutrophils may act as one of the important key determinants in the pathogenesis of severe periodontal destruction in patients with PLS. An increase in the CD29 lymphocytes and NK cells and a decrease in CD45RA lymphocytes. These findings are important to explain B lymphocyte activation and the pathogenesis of the PLS. The combination of retinoid therapy and periodontal treatment improved the dermatologic and periodontal conditions.


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