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Stem
A 2-week-old female infant is referred to the paediatric dermatology clinic with a linear vesicular eruption on the trunk and limbs. The lesions follow Blaschko's lines. She has a peripheral blood eosinophil count of 2.0 x 10^9/L. The family history is significant for the mother's two first-trimester male miscarriages. Skin biopsy shows intraepidermal spongiosis with numerous eosinophils and dyskeratotic keratinocytes.
Questions
a) What is the most likely diagnosis, and what is the inheritance pattern? (2 marks)
b) Name the four cutaneous stages of this condition and give the approximate age at which each occurs. (3 marks)
c) What extracutaneous systems may be involved, and which is the most serious? (2 marks)
d) Outline the key investigations and immediate management priorities for this infant. (3 marks)