Pediatric Dentistry · single question

A father reports his 3-year-old son has no teeth, periorbital wrinkling, and dry skin. The diagnosis is:

  1. Ectodermal dysplasia
  2. Palmoplantar keratosis
  3. Amelogenesis imperfecta
  4. Dentinogenesis imperfecta
Show answer and explanation

Correct answer: A. Ectodermal dysplasia

  • A. Correct. Anodontia/oligodontia with sparse hair, dry skin (hypohidrosis), and periorbital wrinkling is classic ectodermal dysplasia.
  • B. Palmoplantar keratosis affects palms/soles (e.g., Papillon-Lefèvre) and is not defined by missing teeth + dry skin in this way.
  • C. Amelogenesis imperfecta is an enamel defect; teeth are present but malformed.
  • D. Dentinogenesis imperfecta affects dentin (opalescent teeth), teeth are present.

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