Dominant Ichthyosis Vulgaris, also known as Autosomal Dominant Ichthyosis or Ichthyosis simplex, is a genetic skin disorder characterized by the abnormal buildup of dry and scaly skin. It is inherited in an autosomal dominant pattern, meaning that a person only needs to inherit one copy of the defective gene from one parent in order to develop the condition.
This dermatological condition is caused by a mutation in the filaggrin gene, which plays a crucial role in the formation of the skin's protective barrier. The faulty gene leads to a decrease in the production of filaggrin protein, resulting in an impaired skin barrier function. As a consequence, affected individuals experience excessive flaking and shedding of skin cells, leading to the characteristic appearance of thick, dry, and scaly skin.
The symptoms of Dominant Ichthyosis Vulgaris typically manifest during early childhood or infancy and may vary in severity among affected individuals. Most commonly, dry and scaling patches appear on the arms, legs, trunk, and sometimes the face, accompanied by itchiness and discomfort. The scales may range in color from white, gray, or brownish and may become more pronounced in dry or cold environments.
While the condition is chronic and presents significant challenges in terms of managing dry skin, it is not life-threatening and tends to improve with age. Treatment primarily focuses on moisturizing the skin to alleviate symptoms, which may involve the regular use of emollients, moisturizing creams, and ointments. In severe cases, additional interventions such as topical retinoids or keratolytic agents may be prescribed by a dermatologist to aid in the removal of excess skin cells and improve the skin's appearance and texture.
