Abstract
AIM AND BACKGROUND: Lamellar ichthyosis (LI) is a rare type of ichthyosis characterized by accumulation of hyperkeratotic scales on skin surfaces. Literature on the involvement of oral structures is limited. This report highlights the clinical presentation of two siblings detected with LI. CASE DESCRIPTION: The first case is of a 14-year-old boy who reported with a chief complaint of decayed teeth. On examination, the child was found to have dental caries and enamel defects involving permanent molars. The second case, sibling of the first case, reported with decayed teeth. On examination, the child was found to have dental caries and enamel defects involving primary molars and permanent molars. Comprehensive dental care was provided to both patients. CONCLUSION: Enamel defects and dental caries are common findings in children with LI. With necessary precautions, dental treatment was completed. CLINICAL SIGNIFICANCE: Patients with LI can pose a challenge to clinicians. The presence of oral manifestation emphasizes the need for early dental care of these patients. HOW TO CITE THIS ARTICLE: D'Souza OK, Dukle SG, Naik R, et al. Oral Manifestations of Lamellar Ichthyosis: A Case Report of Two Siblings. Int J Clin Pediatr Dent 2025;18(5):606-609.