Keratitis, Ichthyosis, and Deafness(KID) 증후군 1례

A Case of Keratitis, Ichthyosis and Deafness(KID) Syndrome

저자들은 어린선과 과각화증의 특징적인 피부 소견과 결막염, 청력 소실로 KID 증후군이 진단된 환자에서 심한 성장 장애와 담즙 정체, 단백뇨가 동반된 1례를 경험하였기에 이를 보고하는 바이다.

KID syndrome was named after the initials of the major three symptoms of the disease; keratitis, ichthyosis, and deafness. The syndrome was first introduced by Dr. Burns in 1915 who described one of his patients with those symptoms. In 1981, Dr. Skinner and his colleagues reported 17 patients who had keratitis, ichthyosis, and deafness. They also called the disease KID syndrome for the first time. After that, there have been only 60 cases of KID syndrome reported. KID syndrome may be presented with neurosensory deafness, vascularizing keratitis and such skin disorders as ichthyosis, ichthyosiform skin eruptions, and alopecia. Also, those with KID syndrome may suffer from repetitive infections, dental affections, hypohidrosis, growth delay and hepatic and renal abnormalities. The authors report the first case of KID syndrome in Korea with some references. The patient presented with keratitis, neurosensory deafness, and such characteristic skin disorders as ichthyosis and hyperkeratosis.