Total hip replacement in an adolescent patient with harlequin ichthyosis; a case report.

Harlequin ichthyosis (HI) is a rare autosomal recessive skin disorder. No orthopaedic procedure has previously been described on a patient with HI. We report the case of a 17-year-old patient with HI who presented with bilateral juvenile idiopathic arthritis of the hips who underwent bilateral total hip replacements. Our standard operative and postoperative regime was followed and no complications occurred. One year after the second procedure our patient had a Oxford hip score of 43 and was very satisfied with the result.