Orofacial manifestations in a child with congenital lamellar ichthyosis: Practical recommendations for dental management

Abstract

Ichthyosis is a group of rare Mendelian disorders which affects cornification of the skin characterized by hyperkeratosis and/or scaling. The incidence of lamellar ichthyosis is estimated to be approximately 1 in 300,000 live births with no known sex predilection. The presenting features comprise a wide array of dermatological manifestations. This case illustrates findings in a 5-year-old child with the rare disorder, i.e., congenital lamellar ichthyosis with a history of rickets with interesting array of orofacial manifestations associated with the disease. The dental management may include a multispecialty approach for rehabilitation while using delicate approach to manipulate soft tissues and skin. We share a few practical recommendations which were helpful for allaying challenges we faced in this case.