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Abstract
Hyperkeratosis palmoplantaris with periodontosis (Papillon-Lefèvre syndrome) in a
10-year-old Negro boy is presented. Fewer than fifty cases have been reported in the
world's literature. The classic findings of hyperkeratotic skin lesions on the palms
and soles, associated with destruction of the periodontium of the deciduous and permanent
teeth leading to dental exfoliation, are shown. The acute gingival inflammation accompanying
the latter aspect of the syndrome is discussed. The etiologic role of parental consanguinity
and genetics is mentioned, although not proved in the case presented here. The disease
is progressive, resulting in loss of the permanent dentition (with the exception of
the third molars) and mild to severe lesions of the skin. Treatment is symptomatic
and not always satisfactory.
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References
- Deux cas de keratodermie palmaire et plantaire symetrique familiale (maladie de meleda) chez de frère et la loer; coexistance dans le deux cas d'alterations dentaíres graves.Bull. Soc. franç. dermat. et syph. 1924; 31: 82-87
- The Syndrome of Palmar-Plantar Hyperkeratosis and Premature Periodontal Destruction of the Teeth.J. Pediat. 1964; 65: 895-908
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- Palmer Plantar Hyperkeratosis With Periodontosis (Papillon-Lefèvre Syndrome).Arch. Dermat. 1964; 90 (and others): 330
- Hyperkeratosis Palmo-plantaris With Periodontosis (Papillon-Lefèvre).Arch. Dermat. 1963; 88: 207-209
- Keratosis Palmo-plantaris Congenita, With Periodontosis, Arachnodactyly and a Peculiar Deformity of the Terminal Phalanges.Brit. J. Dermat. 1965; 77: 42-54
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© 1967 Published by Elsevier Inc.
