Atlas · Plate VII · General and Oral Pathology · 09

Genetic and systemic conditions

Some conditions show up first in the mouth. Down syndrome brings early severe periodontitis, macroglossia and a fissured tongue; ectodermal dysplasia gives missing or cone-shaped teeth; amelogenesis imperfecta affects only enamel, while dentinogenesis imperfecta causes opalescent teeth with obliterated pulps. Addison disease adds oral pigmentation, Peutz-Jeghers syndrome perioral freckles, and Sjögren syndrome a dry mouth and dry eyes.

High-yield facts · 7

  1. Down syndrome (trisomy 21): early severe periodontitis, macroglossia, fissured tongue, delayed eruption, hypodontia, heart defects.T5
  2. Ectodermal dysplasia: missing or cone-shaped teeth, sparse hair, few sweat glands.T5
  3. Amelogenesis imperfecta affects enamel only; dentinogenesis imperfecta gives opalescent teeth and obliterated pulps.T5
  4. Cleidocranial dysplasia: missing or underdeveloped clavicles, many unerupted supernumerary teeth.T5
  5. Addison disease causes bronze skin and oral melanin pigmentation.T5
  6. Peutz-Jeghers syndrome: perioral freckles and intestinal polyps.T5
  7. Sjögren syndrome: autoimmune dry mouth and dry eyes, often with rheumatoid arthritis; raises lymphoma risk.T5

Facts are written in our own words; each tag links to its source. Values marked "sources vary" differ between references, so check the one your program uses.