Ichthyosis vulgaris

Question 1

Ichthyosis vulgaris: What type of inheritance pattern is associated with the condition described?

Answer 1

Autosomal dominant

Gene locus is unknown

Question 2

Ichthyosis vulgaris: What is the incidence rate of the condition?

Answer 2

1:250–1:2,000; M = F

Question 3

Ichthyosis vulgaris: At what age does the condition typically present?

Answer 3

Three months to 1 year of life

Question 4

Ichthyosis vulgaris: What is the pathogenesis of the condition?

Answer 4

Retention hyperkeratosis with normal epidermal proliferation; defect in profilaggrin synthesis

Results in decreased levels of profilaggrin in keratinocytes; most likely a polygenic disease

Question 5

Ichthyosis vulgaris: What are the key skin features of the condition?

Answer 5

Fine, whitish, adherent scale sparing flexures; increased involvement on extensor extremities; face usually spared but may involve cheeks and forehead

Other features include atopic dermatitis (50%), keratosis pilaris, and accentuated palmoplantar markings

Question 6

Ichthyosis vulgaris: What are some differential diagnoses for the condition?

Answer 6

• Atopic dermatitis
• Xerosis
• Acquired ichthyosis
• X-linked ichthyosis
• Lamellar ichthyosis

Question 7

Ichthyosis vulgaris: What investigations are recommended for diagnosis?

Answer 7

Skin biopsy from anterior shin—absent granular layer; electron microscopy—small, poorly formed keratohyalin granules

Question 8

Ichthyosis vulgaris: What management strategies are recommended for the condition?

Answer 8

Referral to dermatologist—topical emollients

Question 9

Ichthyosis vulgaris: What factors influence the prognosis of the condition?

Answer 9

Improves in summer and with age; also improves in warm, moist environment