Dermatology · Genodermatoses and Rare Disorders

Which triad defines the classic form of hypohidrotic ectodermal dysplasia?

  • A Hypohidrosis, cataract, sensorineural deafness
  • B Hyperhidrosis, nail dystrophy, palmoplantar keratoderma
  • C Hypohidrosis, hypotrichosis, hypodontia with peg-shaped teeth
  • D Hypotrichosis, leukoplakia, nail dystrophy
Correct answer: C. Hypohidrosis, hypotrichosis, hypodontia with peg-shaped teeth

Explanation

The classic triad of hypohidrotic ectodermal dysplasia is hypohidrosis, hypotrichosis and hypodontia with conical peg-shaped teeth; most cases are X-linked recessive due to EDA mutations, and affected boys have unexplained fever from heat intolerance. Option D describes the triad of dyskeratosis congenita, which also includes bone marrow failure later in life.

Reference: Fitzpatrick's Dermatology, 9th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.

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