Papillon-Lefevre syndrome

Revision as of 14:44, 20 August 2012 by WikiBot (talk | contribs) (Robot: Automated text replacement (-{{SIB}} +, -{{EH}} +, -{{EJ}} +, -{{Editor Help}} +, -{{Editor Join}} +))
(diff) ← Older revision | Latest revision (diff) | Newer revision → (diff)
Jump to navigation Jump to search
Papillon-Lefevre syndrome
OMIM 245000
DiseasesDB 9583
MeSH D010214

WikiDoc Resources for Papillon-Lefevre syndrome

Articles

Most recent articles on Papillon-Lefevre syndrome

Most cited articles on Papillon-Lefevre syndrome

Review articles on Papillon-Lefevre syndrome

Articles on Papillon-Lefevre syndrome in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Papillon-Lefevre syndrome

Images of Papillon-Lefevre syndrome

Photos of Papillon-Lefevre syndrome

Podcasts & MP3s on Papillon-Lefevre syndrome

Videos on Papillon-Lefevre syndrome

Evidence Based Medicine

Cochrane Collaboration on Papillon-Lefevre syndrome

Bandolier on Papillon-Lefevre syndrome

TRIP on Papillon-Lefevre syndrome

Clinical Trials

Ongoing Trials on Papillon-Lefevre syndrome at Clinical Trials.gov

Trial results on Papillon-Lefevre syndrome

Clinical Trials on Papillon-Lefevre syndrome at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Papillon-Lefevre syndrome

NICE Guidance on Papillon-Lefevre syndrome

NHS PRODIGY Guidance

FDA on Papillon-Lefevre syndrome

CDC on Papillon-Lefevre syndrome

Books

Books on Papillon-Lefevre syndrome

News

Papillon-Lefevre syndrome in the news

Be alerted to news on Papillon-Lefevre syndrome

News trends on Papillon-Lefevre syndrome

Commentary

Blogs on Papillon-Lefevre syndrome

Definitions

Definitions of Papillon-Lefevre syndrome

Patient Resources / Community

Patient resources on Papillon-Lefevre syndrome

Discussion groups on Papillon-Lefevre syndrome

Patient Handouts on Papillon-Lefevre syndrome

Directions to Hospitals Treating Papillon-Lefevre syndrome

Risk calculators and risk factors for Papillon-Lefevre syndrome

Healthcare Provider Resources

Symptoms of Papillon-Lefevre syndrome

Causes & Risk Factors for Papillon-Lefevre syndrome

Diagnostic studies for Papillon-Lefevre syndrome

Treatment of Papillon-Lefevre syndrome

Continuing Medical Education (CME)

CME Programs on Papillon-Lefevre syndrome

International

Papillon-Lefevre syndrome en Espanol

Papillon-Lefevre syndrome en Francais

Business

Papillon-Lefevre syndrome in the Marketplace

Patents on Papillon-Lefevre syndrome

Experimental / Informatics

List of terms related to Papillon-Lefevre syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Papillon-Lefèvre syndrome (PLS) is an autosomal recessive[1] genetic disorder caused by a deficiency in cathepsin C.[2]

File:Autorecessive.svg

Symptoms

PLS is characterized by periodontitis and palmoplantar keratoderma.[3] The severe destruction of periodontium results in loss of most primary teeth by the age of 4 and most permanent teeth by age 14. Hyperkeratosis of palms and soles of feet appear in first few years of life.

Treatment

Retinoids and antibiotics have been used.[4]

Eponym

It is named for M. M. Papillon and Paul Lefèvre.[5][6]

References

  1. Ullbro C, Crossner CG, Nederfors T, Alfadley A, Thestrup-Pedersen K (2003). "Dermatalogical and oral findings in a cohort of 47 patients with Papillon-Lefevre syndrome". J Am Acad Dermatol. 48 (3): 345–351. doi:10.1067/mjd.2003.197. PMID 12637913.
  2. Wani A, Devkar N, Patole M, Shouche Y (2006). "Description of two new cathepsin C gene mutations in patients with Papillon-Lefèvre syndrome". J Periodontol. 77 (2): 233–237. doi:10.1902/jop.2006.050124. PMID 16460249.
  3. Cagli NA, Hakki SS, Dursun R; et al. (2005). "Clinical, genetic, and biochemical findings in two siblings with Papillon-Lefèvre Syndrome". J. Periodontol. 76 (12): 2322–9. doi:10.1902/jop.2005.76.12.2322. PMID 16332247. Unknown parameter |month= ignored (help)
  4. Ahuja V, Shin RH, Mudgil A, Nanda V, Schoor R (2005). "Papillon-Lefèvre syndrome: a successful outcome". J. Periodontol. 76 (11): 1996–2001. doi:10.1902/jop.2005.76.11.1996. PMID 16274321. Unknown parameter |month= ignored (help)
  5. Template:WhoNamedIt
  6. M. M. Papillon, P. Lefèvre. Deux cas de kératodermie palmaire et plantaire symétrique familiale (maladie de Meleda) chez le frère et la soeur. Coexistence dans les deux cas d’altérations dentaires graves. Bulletin de la Société française de dermatologie et de vénéorologie, Paris, 1924, 31: 82-87.

External links


Template:WikiDoc Sources