Lamellar ichthyosis

QUESTION 89

A 23-year-old woman presents for the evaluation of dry, scaly skin. She reports a history of extremely dry and scaly skin throughout her life, from birth. She has tried numerous topical emollients and keratolytics with only mild–moderate improvement. She reports a family history of dry, scaly skin, but does not think any family member has had cutaneous disease as severe as hers. On examination, there are large plate-like scales on the trunk and extremities that are centrally attached with raised borders, with some areas showing superficial fissures (89a, b).