Dermatologic and oral findings in a cohort of 47 patients with Papillon-Lefèvre syndrome

Ullbro, Christer; Crossner, Claes-Göran; Nederfors, Tommy; Alfadley, Abdullah; Thestrup‐Pedersen, Kristian

doi:10.1067/mjd.2003.197

Cited by 80 publications

(94 citation statements)

References 18 publications

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“…The degree of dermatologic involvement may not be related to the level of periodontal infection [18]. Nail changes such as transverse grooving and fissuring are apparent in advanced cases [19].…”

Section: Introductionmentioning

confidence: 99%

Papillon Lefevre Syndrome: a case report with review

Kaur¹

2013

Dentistry

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Section: Introductionmentioning

confidence: 99%

Papillon Lefevre Syndrome: a case report with review

Kaur¹

2013

Dentistry

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“…Ullbro et al analyzed 47 patients with PLS and found, with no exception, that both skin and oral changes developed early in life in all the patients studied. Dermatologic involvement showed no correlation with age, whereas periodontal disease was significantly worse in young children with deciduous teeth [36]. No significant correlation, however, was demonstrated between the severity of the periodontal and of skin affections [36].…”

Section: Typical Features Of Papillon-lefévre Syndromementioning

confidence: 71%

“…Dermatologic involvement showed no correlation with age, whereas periodontal disease was significantly worse in young children with deciduous teeth [36]. No significant correlation, however, was demonstrated between the severity of the periodontal and of skin affections [36]. There was an early eruption of the permanent teeth, which were caries-free with no sign of root resorption [37].…”

Section: Typical Features Of Papillon-lefévre Syndromementioning

confidence: 91%

“…Keratosis may also affect other sites such as the elbows and knees [31]. Although there is a significant correlation between the severities of keratosis in the feet and hands, changes in the feet are more severe [36]. There also is a positive correlation between the severity of skin lesions and seasonal variations as well as intensified periodontal destruction [37].…”

Section: Typical Features Of Papillon-lefévre Syndromementioning

confidence: 93%

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Syndromes That Include Both Palmoplantar Keratoderma And Severe Periodontitis: A Review

Dababneh¹

2013

Dentistry

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Palmoplantar keratodermas (PPK) include a heterogeneous group of disorders with overlapping clinical features. The main aspect of PPK is thickening and hyperkeratosis of the palmar and plantar skin, that may be hereditary or acquired; diffuse, focal, or punctuate; and transgrediens or progrediens. PPKs are further distinguished by their mode of inheritance and by the presence of certain associated clinical features. Periodontitis was reported in association with more than one syndrome characterized by PPK. An extensively reported one is the Papillon-Lefévre syndrome (PLS) which is characterized by early onset of PPK and periodontitis affecting the primary and secondary dentitions. In addition to PLS, Haim-Munk, HOPP, Variant Carvajal and Weary-Kindler are other syndromes manifested by PPK and reported in association with severe periodontitis. Atypical cases of PLS were also reported, such as partial expression or a late presentation of the syndrome. The aim of this article is to critically review the literature concerned with Papillon-Lefévre syndrome in its typical and atypical clinical presentation, in addition to other syndromes manifested at the same time by PPK and severe periodontitis.Thorough history and medical examination, together with periodontal, dermatologic, and genetic counseling, are important to exclude other existing medical conditions or other syndromes that might need special attention and care.

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“…The CTSC gene is expressed at high levels in various immune cells, including polymorphonuclear leukocytes, macrophages, and their precursors (7,14). While periodontitis and palmoplantar hyperkeratosis are attributable to the same CTSC mutations, skin pigmentation has not been described previously as part of this complex of symptoms.…”

Section: Discussionmentioning

confidence: 99%

Papillon-Lefévre syndrome: a highly-suspected case

Ikeshima

2006

J Oral Sci

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Dermatologic and oral findings in a cohort of 47 patients with Papillon-Lefèvre syndrome

Cited by 80 publications

References 18 publications

Papillon Lefevre Syndrome: a case report with review

Papillon Lefevre Syndrome: a case report with review

Syndromes That Include Both Palmoplantar Keratoderma And Severe Periodontitis: A Review

Papillon-Lefévre syndrome: a highly-suspected case

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