| Summary: | Harlequin ichthyosis is a rare congenital disorder, which causes restrictive circumferential encasement of the trunk and limbs. Patients usually develop compartment syndrome and sequential cyanosis of limbs and digits, leading to autoamputation. We report a case of harlequin ichthyosis in which all digits were preserved with an early escharotomy-like procedure. A 33–6/7-week-old preterm girl presented with whole body hyperkeratosis, constrictive bands on neck, chest, abdominal, limbs, and developed compartment syndrome. On the second day after birth, distal digits progressive swelling and ischemic change occurred. An escharotomy-like procedure was performed on all 4 extremities to the distal digits. All distal phalanges and nail plate were well preserved at 5-month follow-up. We concluded that for prevention of digits autoamputation in harlequin ichthyosis, early detection of compartment syndrome is necessary and an escharotomy-like procedure should be performed as soon as possible when ischemia occurs.
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