Heritable Disorders: Ichthyosis Vulgaris

Question 1

Ichthyosis Vulgaris

Clinical Feature morphology

Answer 1

xerosis with fine scaling as well as large adherent scales (“fish-scales”)

Question 2

Ichthyosis Vulgaris common sites

Answer 2

affects arms, legs, palms, soles, back, forehead, and cheeks; spares flexural creases

Question 3

Ichthyosis Vulgaris course

Answer 3

improves in summer, with humidity, and as the child grows into adulthood

Question 4

Ichthyosis Vulgaris Pathophysiology

Answer 4

genetic deficiency in filaggrin protein leads to abnormal retention of keratinocytes (hyperkeratosis)
• scaling without inflammation

Question 5

Ichthyosis Vulgaris Epidemiology

Answer 5

1:300 incidence

Question 6

Ichthyosis Vulgaris inheritance

Answer 6

autosomal dominant inheritance

Question 7

Ichthyosis Vulgaris associated pathologies

Answer 7

with AD and keratosis pilaris

Question 8

Ichthyosis Vulgaris Investigations

Answer 8

electron microscopy: keratohyalin granules

Question 9

Ichthyosis Vulgaris topical Management

Answer 9

immersion in bath and oils followed by an emollient cream, humectant cream, or creams/oil containing
urea or alpha- or betha-hydroxy acids

Question 10

Ichthyosis Vulgaris systemic Management

Answer 10

intermittent systemic retinoids for severe cases