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Results for “Papillon-Lefevre Disease”

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At least 19 recordsLinked to original sources
PubMed1978

[Papillon-lefèvre syndrome].

Papillon-Lefèvre syndrome in a 13-year-old boy is described. His two great-grandfathers were first-degree cousins (consanguinity). However, no other case of the syndrome nor of palmoplantar keratoderma exists in the family. The palmoplantar keratoderma, which had started before becoming 1 year of age, is usually more pronounced during spring and autumn as well as during feverish diseases. However, it is of the transgradient type and is not necessarily very severe. The loss of the deciduous and permanent teeth was a consequence of severe juvenile parodontitis and of bone destruction. The boy could extract his teeth easily by himself and has total prothesis since the age of 14 years. Every treatment was unsuccessful.

Adolescent↗
PubMed1978

A genetic analysis of the Papillon-Lefèvre syndrome in a Jewish family from Cochin.

The Papillon-Lefèvre syndrome (PLS) is segregating in a large kindred of a Jewish isolate originating from Cochin, India. The frequency of the gene responsible for PLS among the Cochin Jews, 0.1, was estimated from the number of unrelated carriers in the isolate who married into the kindred. The obvious discrepancy between this apparently high gene frequency and the total absence of PLS in other kindreds of the isolate suggests that the syndrome may not behave as a simple autosomal recessive trait.

Female↗
PubMed1979

The Papillon-Lefèvre syndrome: keratosis palmoplantaris with periodontopathy. Report of a case and review of the cases in the literature.

The Papillon-Lefévre syndrome (PLS) is an autosomal recessive trait characterized by diffuse transgredient palmar-plantar keratosis (PPK) and premature loss of both the deciduous and permanent teeth. In most cases, the PPK is noted within the first 3 years of life. The periodontal lesions begin shortly after the start of both the primary and the permanent dentitions. The teeth are affected in the order of their eruption, exhibiting inflammation of the periodontal tissue, bleeding of the gums, pocket formation, loosening, and finally spontaneous exfoliation without showing definite signs of root resorption. After an edentulous interval, the same process begins anew shortly after the second dentition. Ectopic intracranial calcifications, mental retardation, and increased susceptibility to infections have often been seen in PLS patients and may thus be regarded as facultative signs.

Child↗
PubMed1979

Papillon-Lefevre syndrome.

Two cases of Papillon-Lefevre Syndrome (PLS) in a family are reported. Association of recurrent infections, arachnodactyly, intracranial calcification and phalangeal abnormality have been observed. Because there is no history of consanguinity, and autosomal recessive pattern of inheritance is suggested.

Child↗
PubMed1978

Deficient phagocytic function in Papillon-Lefèvre syndrome.

The function of microphages has been studied in vitro in a 5 year-old girl with Papillon-Lefèvre syndrome. The results showed a weakness in chemotatic activity, a defect of intracellular killing of Staphylococcus aureus, and an almost normal phagocytosis but decreased intracellular killing of Candida albicans by the microphages.

Chemotaxis, Leukocyte↗
PubMed1976

Pipillon-Lefèvre syndrome: report of two familial cases.

Two cases with Papillon-Lefèvre Syndrome (PLS) are reported. They are two brothers belonging to a family which there is strong consanguinity. An autosomal recessive pattern of inheritance is suggested. Some pathological findings as congenital bronchiectasis observed in one of the patients as well as the PLS features are discussed and considered to be fortuitous defects. These observations are compared with those described in the literature.

Adolescent↗
PubMed1976

Papillon-Lefevre syndrome: report of two cases in the same family.

Two cases of Papillon-Lefevre syndrome have been seen in the same family. The characteristic features of the syndrome are shown to be present in both children and the main characteristics are hyperkeratosis of the palms of the hands and soles of the feet; destruction of the periodontium of the deciduous teeth with subsequent premature loss of the teeth; and destruction of the periodontium of the permanent teeth and the premature loss of these teeth usually before 16 years of age.

Child↗