Diseases

Pachyonychia Congenita

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

MeSH Record

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MeSH Record

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MeSH Record

Synonyms

24 entry terms
  • Congenital Pachyonychia
  • Pachyonychia Congenita Syndrome
  • Pachyonychia, Congenital
  • 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 Tarda, Type 1
  • Pachyonychia Congenita Type 1
  • Pachyonychia Congenita, Jackson-Lawler Type
  • Pachyonychia Congenita, Jadassohn-Lewandowsky Type
  • Pachyonychia Congenita, Type 1
  • Pachyonychia Congenita, Type 2
  • 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
  • Syndrome, Jackson-Lawler (Pc-2)
  • Syndrome, Jadassohn-Lewandowski (Pc-1)

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2007

MeSH Record

Previous Indexing

  • Nails, Malformed (1964-2006)

MeSH Hierarchy

Tree Numbers

  1. C16w.131w.077w.350w.856w
  2. C16w.131w.831.350w.856w
  3. C16w.320w.850w.250w.856w
  4. C17.800w.529w.594w
  5. C17.800w.804.350w.856w
  6. C17.800w.827w.250w.856w

AMA Style

References

  1. National Library of Medicine. Pachyonychia Congenita. Medical Subject Headings (MeSH). 2026. Unique ID D053549. http://id.nlm.nih.gov/mesh/2026/D053549
  2. Pachyonychia Congenita. In: Wikipedia. https://en.wikipedia.org/wiki/Pachyonychia_congenita
  3. Pachyonychia Congenita. In: Wikidata. https://www.wikidata.org/wiki/Q3360152