Case Report

Year : 2021 | Volume : 6 | Issue : 1 | Page : 28-30

Orofacial Manifestations in a Child with Congenital Lamellar Ichthyosis: Practical Recommendations for Dental Management

Rishi Tyagi, Namita Kalra, Amit Khatri, Puja Sabherwal, Padma Yangdol

Department of Pedodontics and Preventive Dentistry, University College of Medical Sciences, Guru Teg Bahadur Hospital, New Delhi, India

Address for correspondence:

Dr. Puja Sabherwal,

Department of Pedodontics and Preventive Dentistry, University College of Medical Sciences, Guru Teg Bahadur Hospital, New Delhi, India.

E‐mail: sabherwalpuja@gmail.com

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.

Keywords: Autosomal recessive congenital ichthyosis, orofacial manifestations, practical recommendations for dental management, rare Mendelian disorder

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

How to cite this article: Tyagi R, Kalra N, Khatri A, Sabherwal P, Yangdol P. Orofacial manifestations in a child with congenital lamellar ichthyosis: Practical recommendations for dental management. Int J Pedod Rehabil 2021;6:28-30.