The syndrome of palmar-plantar hyperkeratosis and premature periodontal destruction of the teeth

Gorlin, Robert J.; Sedano, Heddie O.; Anderson, Valerie

doi:10.1016/s0022-3476(64)80014-7

Cited by 250 publications

(201 citation statements)

References 30 publications

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“…16 -18 Other minor features of PLS include calcification of the dura, falx cerebri, tentorium cerebelli, and choroids plexus. 1,19 On presentation, our patient had the 2 major features of PLS. He had severe active periodontitis and PPK.…”

Section: Discussionmentioning

confidence: 82%

Pyogenic Liver Abscess and Papillon-Lefèvre Syndrome: Not a Rare Association

Almuneef¹,

Khenaizan

Ajaji³

et al. 2003

Pediatrics

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ABSTRACT. Papillon-Lefèvre syndrome is a rare, autosomal recessive disease comprising palmoplantar keratoderma and periodontitis. Pyogenic liver abscess is an increasingly recognized complication. We report a new case of this association and review the current literature. Pediatrics 2003;111:e85-e88. URL: http://www.pediatrics. org/cgi/content/full/111/1/e85; Papillon-Lefèvre syndrome, liver abscess.

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Section: Discussionmentioning

confidence: 82%

Pyogenic Liver Abscess and Papillon-Lefèvre Syndrome: Not a Rare Association

Almuneef¹,

Khenaizan

Ajaji³

et al. 2003

Pediatrics

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“…The combination of oral retinoids and antibiotics complemented with professional tooth cleaning are currently used in the treatment of severe periodontitis occurring with this disorder (Dhanrajani, 2009). PLS is an extremely rare inherited autosomal recessive disease with an estimated prevalence of 1 to 4 cases per million people, affecting children between the ages of 1 and 4 (Gorlin et al, 1964). It was first described by two French physicians, Papillon and Lefèvre (1924), as a form of "Mal de Meleda" and was only later classified as a distinct entity (Dekker and Jansen, 1956).…”

Section: Papillon-lefèvre Syndromementioning

confidence: 99%

Neutrophil Elastase, Proteinase 3, and Cathepsin G as Therapeutic Targets in Human Diseases

et al. 2010

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“…In addition to palmoplantar keratoderma and early periodontal destruction of primary and permanent dentition, other features may be associated with PLS, i.e., increased susceptibility to infection [16], increased risk of pyogenic liver abscess [40][41][42][43], calcification of the falx cerebri of the dura mater [44], excessive sweating (hyperhidrosis), and growth of fine body hair with the development of dirty-colored skin on the affected areas [45]. Recently, the first case of multiple brain abscesses in a child with PLS was reported [46].…”

Section: Secondary Features Associated With Plsmentioning

confidence: 99%

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 syndrome of palmar-plantar hyperkeratosis and premature periodontal destruction of the teeth

Cited by 250 publications

References 30 publications

Pyogenic Liver Abscess and Papillon-Lefèvre Syndrome: Not a Rare Association

Pyogenic Liver Abscess and Papillon-Lefèvre Syndrome: Not a Rare Association

Neutrophil Elastase, Proteinase 3, and Cathepsin G as Therapeutic Targets in Human Diseases

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

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