Oral Manifestations and Conservative Clinical Management of a 2-year-old Child with Congenital Ichthyosis: A Case Report

Congenital Ichthyosis (CI) is an uncommon genetic condition affecting tissues of ectodermal origin, including the skin, nails and tooth enamel. The thickening of the stratum corneum impairs the skin’s ability to act as a protective barrier due to aberrant epidermal cell differentiation, as well as lipid synthesis, metabolism and transport. This set of keratinisation disorders is clinically and etiologically diverse, with severe forms characterised by skin dryness, flaking and peeling, leading to a wide range of complications. CI involves mutations in various genes and presents with different symptoms that can significantly affect the patient’s quality of life. Although it affects tissues of ectodermal origin, little is known about its oral manifestations. Lamellar Ichthyosis (LI) is a type of CI that typically manifests at birth, with the infant found to be covered in a membrane. Xerostomia is one oral symptom that may increase the risk of dental caries. While many individuals with CI may present with normal dentition, some may experience dental defects, delayed tooth eruption and a higher risk of dental caries. In the context of CI, the primary consideration in dental care should include the presence of friable and tender skin, which necessitates extra caution during handling. It is crucial to ensure that any procedure minimises irritation and injury to the skin, as it is more vulnerable than usual. The present case report highlights a case of a two-yearold female child diagnosed with CI who presented with early childhood caries. Management may include a conservative approach for rehabilitation, utilising delicate handling of soft tissues to reduce patient discomfort and facilitate successful treatment.