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Papillon-Lefevre Disease

Rare, autosomal recessive disorder occurring between the first and fifth years of life. It is characterized by palmoplantar keratoderma with periodontitis followed by the premature shedding of both deciduous and permanent teeth. Mutations in the gene for CATHEPSIN C have been associated with this disease.
Also Known As:
Haim-Monk Syndrome; Keratosis Palmoplantar Periodontopathy; Keratosis Palmoplantaris with Periodontopathia; Papillon Lefevre Disease; Papillon-Lefevre Syndrome; Haim Monk Syndrome; Keratosis Palmoplantar Periodontopathies; Palmoplantar Periodontopathies, Keratosis; Papillon Lefevre Syndrome
Networked: 32 relevant articles (0 outcomes, 2 trials/studies)

Disease Context: Research Results

Related Diseases

1. Palmoplantar Keratoderma (Keratosis Palmaris et Plantaris)
2. Netherton Syndrome
3. X-Linked Dominant Hypophosphatemic Rickets (X-Linked Hypophosphatemia)
4. Pyogenic Liver Abscess
5. Erythropoietic Porphyria (Congenital Erythropoietic Porphyria)

Experts

1. Ahmed, Bilal: 1 article (05/2014)
2. Mushtaq, Mubashir: 1 article (01/2014)
3. Khan, Fayiza Yaqoob: 1 article (01/2014)
4. Jan, Suhail Majid: 1 article (01/2014)
5. Al Farraj AlDosari, Abdullah: 1 article (04/2013)
6. Shalini, K: 1 article (12/2012)
7. Sudha, Madhuri D: 1 article (12/2012)
8. Sreeramulu, B: 1 article (12/2012)
9. Kiran, G: 1 article (12/2012)
10. Haragopal, S: 1 article (12/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Papillon-Lefevre Disease:
1. Cathepsin CIBA
2. Histocompatibility Antigens Class IIIBA
3. Dental Implants (Dental Implant)IBA
4. Serine Proteases (Serine Protease)IBA
5. Cathepsin LIBA
6. Granzymes (Granzyme)IBA
7. AcitretinFDA Link
8. Isotretinoin (Accutane)FDA LinkGeneric
9. Vitamin DFDA LinkGeneric
10. TetracyclinesIBA

Therapies and Procedures

1. Prosthodontics
2. Photochemotherapy (Photodynamic Therapy)
3. Oral Hygiene (Dental Hygiene)
4. Drug Therapy (Chemotherapy)