Surgical Treatment of Syndactyly of Harlequin Ichthyosis

Ahn,  Sung Jae; Hong,  Jong Won

doi:2018.23.3.184

Arch Hand Microsurg > Volume 23(3); 2018 > Article

Case Report

Archives of Hand and Microsurgery 2018;23(3):184-189.
Published online September 1, 2018.
DOI: https://doi.org/10.12790/ahm.2018.23.3.184

Surgical Treatment of Syndactyly of Harlequin Ichthyosis

Sung Jae Ahn¹, Jong Won Hong^1,2

¹Department of Plastic and Reconstructive Surgery, Yonsei University College of Medicine, Seoul, Korea. saturn@yuhs.ac
²Institute for Human Tissue Restoration, Yonsei University College of Medicine, Seoul, Korea.

Received: 24 April 2018 • Revised: 16 May 2018 • Accepted: 28 May 2018

Abstract

Harlequin ichthyosis (HI) is a rare and most severe form of autosomal recessive congenital ichthyoses characterized by excessive hyperkeratosis. The skin anomalies affect the shape of eyes, ears, nose, mouth, and distal extremities. Since HI is very fatal, there is a lack of data that documents successful treatment of HI patients with syndactyly. We successfully managed syndactyly in a HI patient by using a conventional method without any complication and we noticed an improvement functionally and aesthetically.

Key Words: Lamellar ichthyosis, Syndactyly, Hand deformities, Skin transplantation