Lamellar Ichthyosis

📋Overview

Lamellar ichthyosis is a rare inherited skin condition that typically presents at birth and is characterized by the development of large, plate-like scales across the body. This disorder is classified as a form of autosomal recessive congenital ichthyosis (ARCI), which encompasses several related genetic conditions that affect the development and function of the skin barrier.

Although the term is often used as a specific diagnosis, it is increasingly recognized as part of the broader ARCI clinical spectrum. The condition occurs when genetic mutations disrupt the normal processes of skin cell shedding and lipid production, leading to a persistent thickening of the outer skin layer.