Keratosis Palmo-Plantaris Congenita, With Perio-Dontosis, Arachnodactyly and a Peculiar Deformity of the Terminal Phalanges.

Haim, S; Munk, J.

doi:10.1111/j.1365-2133.1965.tb14565.x

Cited by 85 publications

(69 citation statements)

References 16 publications

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“…In addition, he showed hepatosplenomegaly, hyperproteinemia, hyper gammaglobulinemia and a high titer of IgG. We have found only one similar observation [5], in which four brothers had PLS. All four had hyperpro teinemia as well as liver disorders (hepatomegaly, disturbed liver function tests, etc.)…”

Section: Discussionmentioning

confidence: 67%

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Papillon-Lefèvre Syndrome: Report of Two Familial Cases

Bravo-Piris¹,

Villarón²,

Martínez³

et al. 1976

Dermatology

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Two cases with Papillon-Lefèvre Syndrome (PLS) are reported. They are two brothers belonging to a family in 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.

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Section: Discussionmentioning

confidence: 67%

“…In addition to these constant features, other pathological findings have been reported in the literature: arachnodactyly [5], calcification of the dura [4], retardation of somatic development [12], susceptibility to infections [4][5][6], follicular hyperkeratosis [10], onychogryphosis [5,12].…”

Section: Introductionmentioning

confidence: 99%

Papillon-Lefèvre Syndrome: Report of Two Familial Cases

Bravo-Piris¹,

Villarón²,

Martínez³

et al. 1976

Dermatology

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“…Th is condition displays the same features of hyperkeratosis and severe aggressive periodontitis as Papillon-Lefevre syndrome, with the addition of arachnodactyly and a peculiar deformity of the terminal phalanges [34]. The Haim-Munk syndrome was also found to be linked to the cathepsin C gene.…”

Section: Monogenic Syndromesmentioning

confidence: 98%

Association of Periodontal Diseases with Genetic Polymorphisms

Gandhi¹,

Kothiwale²

2012

IJGE

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Periodontal diseases are multifactorial in nature. While microbial and other environmental factors are believed to initiate and modulate periodontal disease progression, there now exist strong supporting data that genetic polymorphis ms play a role in the predisposition to and progression of periodontal diseases. Variat ions in any number or combination of genes that control the development of the periodontal tissues or the competency of the cellular and humoral immune systems could affect an individual's risk fo r d isease. A corollary of this realization is that if the genetic basis of periodontal disease susceptibility can be understood, such information may have diagnostic and therapeutic value. This review aims to update the clin ician about various genetic polymorphis ms associated with periodontal diseases to aid in a better approach to the condition in the future.

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“…Other Syndromes Reported PPK in Association with Periodontitis (Table 1) Haim and munk syndrome Haim and Munk reported findings similar to Papillion-Lefevre syndrome in four sibs of a Jewish religious isolate from Cochin, India, on the Malabar Coast who later migrated to Israel [66]. Hence, the name Haim-Munk syndrome (HMS) was introduced.…”

Section: Atypical 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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Keratosis Palmo-Plantaris Congenita, With Perio-Dontosis, Arachnodactyly and a Peculiar Deformity of the Terminal Phalanges.

Cited by 85 publications

References 16 publications

Papillon-Lefèvre Syndrome: Report of Two Familial Cases

Papillon-Lefèvre Syndrome: Report of Two Familial Cases

Association of Periodontal Diseases with Genetic Polymorphisms

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

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