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Pachyonychia Congenita (Jadassohn-Lewandowsky Syndrome)

A group of inherited ectodermal dysplasias whose most prominent clinical feature is hypertrophic nail dystrophy resulting in PACHYONYCHIA. Several specific subtypes of pachyonychia congenita have been associated with mutations in genes that encode KERATINS.
Also Known As:
Jadassohn-Lewandowsky Syndrome; Pachyonychia Congenita, Jackson-Lawler Type; Congenital Pachyonychia; Jackson-Lawler Syndrome (Pc-2); Jackson-Lawler Type Pachyonychia Congenita; Jadassohn-Lewandowski Syndrome (Pc-1); Pachyonychia Congenita Jackson Lawler Type; Pachyonychia Congenita Syndrome; Pachyonychia Congenita Tarda, Type 1; Pachyonychia Congenita, Jadassohn-Lewandowsky Type; Type 1 Pachyonychia Congenita; Type 2 Pachyonychia Congenita; Jackson Lawler Syndrome (Pc 2); Jackson Lawler Type Pachyonychia Congenita; Jadassohn Lewandowski Syndrome (Pc 1); Jadassohn Lewandowsky Syndrome; Pachyonychia Congenita, Jackson Lawler Type; Pachyonychia Congenita, Jadassohn Lewandowsky Type; Pachyonychia, Congenital; Syndrome, Jackson-Lawler (Pc-2); Syndrome, Jadassohn-Lewandowski (Pc-1); Pachyonychia Congenita Type 1; Pachyonychia Congenita, Type 1; Pachyonychia Congenita, Type 2
Networked: 162 relevant articles (8 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Palmoplantar Keratoderma (Keratosis Palmaris et Plantaris)
2. Hidradenitis Suppurativa
3. Psoriasis (Pustulosis Palmaris et Plantaris)
4. Darier Disease (Darier's Disease)
5. Epidermolysis Bullosa

Experts

1. Smith, Frances J D: 18 articles (12/2017 - 10/2006)
2. Kaspar, Roger L: 14 articles (02/2016 - 10/2005)
3. McLean, W H Irwin: 13 articles (12/2017 - 10/2006)
4. Hickerson, Robyn P: 10 articles (02/2016 - 10/2006)
5. Leachman, Sancy A: 10 articles (10/2012 - 12/2007)
6. Schwartz, Mary E: 9 articles (06/2019 - 09/2009)
7. Smith, F J D: 7 articles (01/2020 - 04/2005)
8. Smith, F J: 6 articles (07/2021 - 06/2000)
9. Sprecher, E: 6 articles (07/2021 - 11/2014)
10. Coulombe, Pierre A: 6 articles (01/2020 - 06/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pachyonychia Congenita:
1. Botulinum Toxins (Botulinum Toxin)IBA
2. Hydroxymethylglutaryl-CoA Reductase Inhibitors (HMG-CoA Reductase Inhibitors)IBA
3. Simvastatin (Zocor)FDA LinkGeneric
4. Keratin-6 (Keratin 6A)IBA
5. Small Interfering RNA (siRNA)IBA
6. Sirolimus (Rapamycin)FDA Link
7. AcitretinFDA Link
8. VitaminsIBA
9. Keratins (Keratin)IBA
10. Keratin-16 (Keratin 16)IBA

Therapies and Procedures

1. Therapeutics
2. Injections
3. Electrocoagulation (Electrocautery)
4. Intradermal Injections
5. Drug Therapy (Chemotherapy)