Pediatr Dermatol 2016 Sep 28;33(5):e327-32. Epub 2016 Jul 28.
Plastic and Reconstructive Surgery, Seattle Children's Hospital, Seattle, Washington.
Limb constriction or encasement in patients with harlequin ichthyosis can cause tissue injury resulting in necrosis and auto-amputation. Surgical release of constrictive plaques has been previously demonstrated, but the perioperative and intraoperative considerations surrounding this infrequent intervention have not been discussed in detail. This report documents a case of harlequin ichthyosis requiring surgical treatment, focusing on the importance of early surgical consultation, risks of surgery, indications for and timing of surgical intervention, and the details of the operation.