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Q1: Definition and genetics (2 min)
Define incontinentia pigmenti. What is the mode of inheritance? Which gene is mutated, and why does the condition almost exclusively affect females?
Q2: Cutaneous stages (3 min)
Describe the four cutaneous stages of incontinentia pigmenti. What is the characteristic distribution? Which stage gives the disease its name, and what is seen histologically?
Q3: Extracutaneous features and complications (2 min)
What extracutaneous systems may be involved in incontinentia pigmenti? Which complication is the most serious and how can it be prevented?
Q4: Investigations and differential diagnosis (2 min)
How do you confirm the diagnosis of incontinentia pigmenti? What are the key mimics of the vesicular stage, and how would you distinguish them?
Q5: Management and counselling (1 min)
Outline the multidisciplinary management of a newborn with incontinentia pigmenti. What advice would you give the parents about future pregnancies?