Lamellar Ichthyosis's Oral And Craniofacial Features In A Dental Setting

Abstract

Background: Hyperkeratosis and skin scaling are hallmarks of the uncommon autosomal recessive genodermatosis known as lamellar ichthyosis (LI). The oral and craniofacial manifestations are frequently disregarded, despite the prevalence of dermatological symptoms. Dental professionals might be the first to notice these symptoms, particularly in people who are undiagnosed or only slightly afflicted.

Case Presentation: We report a case of a young child who presented to the dental outpatient department with complaints of dry lips, bleeding gums, and limited mouth opening. Extraoral and intraoral examination revealed features consistent with lamellar ichthyosis, including angular cheilitis, ectropion, eclabium, gingival inflammation, and trismus. The patient had a prior diagnosis of LI confirmed by genetic testing (TGM1 mutation). A multidisciplinary care plan involving dermatology and dentistry was initiated.

Outcome and Follow-Up :Supportive dental care, such as emollients, scaling, and customized oral hygiene instructions, was well received by the patient. He still receives routine evaluations with coordinated dermatology assistance.

Conclusion: Dentists are essential in recognizing how systemic diseases like lamellar ichthyosis manifest orally. Improving quality of life and averting complications require early detection and interdisciplinary cooperation.