Papillon-Lefèvre syndrome

Alternative eponyms

Related people

Symptoms and signs appearing within first 4 years of life include hyperkeratosis of palms and soles, usually diffuse type, seldom punctate type, generally not severe.

Description

Symptoms and signs appearing within first 4 years of life include hyperkeratosis of palms and soles, usually diffuse type, seldom punctate type, generally not severe, similar to those of Meleda syndrome.
In some cases, lesion more severe in winter and receding or disappearing during summer. Periodontosis becomes avident as soon as the last deciduous tooth has erupted, the teeth being involved in the sequence in which they erupt. Sometimes calcification of the choroid plexus and tentorium. Premature exfoliation of the teeth.

The syndrome is considered a variant of mal de Meleda, and is inherited as an autosomal recessive trait.

Bibliography

  • Paul-Henri Papillon, P. Lefèvre:
    Deux cas de kératodermie palmaire et plantaire symétrique familiale (maladie de Meleda) chez le frère et la soeur. Coexistence dans les deux cas d’altérations dentaires graves.
    Bulletin de la Société française de dermatologie et de vénéorologie, Paris, 1924, 31: 82-87.

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