Abstract
Keratitis ichthyosis deafness （KID） syndrome is a congenital disorder characterized by ichthyosiform erythroderma, keratitis, and sensorineural hearing loss. The skin is highly susceptible to infection, often leading to severe recurrent infections. The patient was a 23-year-old female who had been treated for many years at a previous facility for KID syndrome. She presented with oral pain, and further evaluation at the previous facility had led to a diagnosis of mandibular gingival carcinoma. Due to KID syndrome, curative treatment for gingival cancer was judged to be difficult, and she was referred to our department. After consultation with the relevant departments, we decided to perform right mandibular segmental resection, right neck dissection, and reconstruction using a free rectus abdominis musculocutaneous flap. The postoperative course was uneventful with no signs of infection or delayed wound healing. She was discharged on postoperative day 21 and has been alive for 8 months without recurrence. Although KID syndrome presents severe skin symptoms, a favorable outcome can be achieved even in cases of advanced surgery with appropriate infection control, since patients are not in a systemic immunocompromised state.
