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Skin and Connective Tissue Diseases > Skin Diseases > Skin Diseases, Genetic > Keratoderma, Palmoplantar > Papillon-Lefevre Disease

Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Skin Diseases, Genetic > Keratoderma, Palmoplantar > Papillon-Lefevre Disease

Skin and Connective Tissue Diseases > Skin Diseases > Keratosis > Keratoderma, Palmoplantar > Papillon-Lefevre Disease

Preferred term

Papillon-Lefevre Disease

Topical Descriptor

Keratoderma, Palmoplantar

Cathepsin C

Keratosis Palmoplantaris with Periodontopathia
Keratosis Palmoplantar Periodontopathies
Keratosis Palmoplantar Periodontopathy
Palmoplantar Periodontopathies, Keratosis
Papillon Lefevre Disease
Papillon Lefevre Syndrome
Papillon-Lefevre Syndrome

Rare, autosomal recessive disorder occurring between the first and fifth years of life. It is characterized by palmoplantar keratoderma with periodontitis followed by the premature shedding of both deciduous and permanent teeth. Mutations in the gene for CATHEPSIN C have been associated with this disease.

History note

91(75); was see under KERATOSIS PALMARIS ET PLANTARIS 1975-90 (which became KERATODERMA, PALMOPLANTAR 1993)

Papillon-Lèfevren tauti

Finnish
morbus Papillon-Lefevre
morbus Papillon-Lèfevre
Papillon-Lefevre
Papillon-Lèfevre
Papillon-Lefevren oireyhtymä
Papillon-Lèfevren oireyhtymä
Papillon-Lefevren syndrooma
Papillon-Lèfevren syndrooma
Papillon-Lefevren tauti
syndroma Papillon-Lefevre
syndroma Papillon-Lèfevre
synnynnäinen kämmen-jalkapohjakeratoosi
synnynnäinen kämmenen ja jalkapohjan keratoosi
Papillon-Lefevres sjukdom

Swedish

URI

http://www.yso.fi/onto/mesh/D010214

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