Dermatology

Question 1

Answer 1

Incontinentia Pigmenti

• XLD inheritance
  
  • Mutation in IKBKG - males usually fetal lethal
• Classic skin findings along the lines of Blaschko
  
  • Blistering rash in infancy
  • Swirling macular hyperpigmentation in childhood
  • Linear hypopigmentation in adulthood
• Diagnosis by molecular sequencing of IKBKG
• Evaluation:
  
  • Surveillance for seizures and retinal detachment
• Management:
  
  • Multidisciplinary = early intervention, dental, ophthalmology, and neurology

Question 2

Criteria for Referral to Burn Centre

Answer 2

Question 3

Answer 3

Fluorosis

• Mottled enamel from systemic fluoride consumption more than 0.05mg/kg/day during enamel formation
• Can be due to being in high area of fluoride in drinking water, swallowing fluoridated toothpaste or inapprop fluoride supp (shouldn’t give more than 120mg)
• Excessive fluoride during enamel formation = lacy white patches to severe brownish discolouration and hypoplasia

DDX:

• neonatal hyperbili (blue-black on primary teeth)
• porphyria (red-brown)
• tetracyclines (brown-yellow and hypoplasia)