Ichthyosis Etiology and Clinical Manifestation Update: A Review Article

Introduction: Ichthyosis a keratinization disorder, the skin becomes dry and scaly, might like fish appearance. The keratinization process cells in the stratum corneum are changing shape and number of cells. The process of epidermal differentiation is abnormal due to mutations of certain genes in various types of ichthyosis. Etiology genes identified as important in ichthyosis has increased. These genes cause changes in barrier function in the stratum corneum. The most common ichthyosis is ichthyosis vulgaris and has a fairly good prognosis. The rare and highest severity ichthyosis is harlequin ichthyosis and has a poor prognosis because of its high morbidity rate. The number of genes identified and shown to cause ichthyosis in human patients has reached eleven. Barriers function in the stratum corneum as an underlying pathogenetic mechanism. Discussion: The skin barrier in the stratum corneum can occur in three main components, namely the intercellular lipid layer, cornified cells and keratin / filaggrin degradation products. The first ichthyosis consensus conference was held to establish a consensus on the nomenclature and classification of inherited ichthyosis and an international consensus on the classification of inherited ichthyosis was achieved. The end product of the fully differentiated epidermis is the stratum corneum, which is composed of terminal keratinocytes called corneocytes, which are surrounded by an intercellular matrix. Corneocytes are enriched by protein, while the intercellular cellular matrix consists of a hydrophobic membrane enriched with keratin lipids. Corneocytes are thought to have good water resistance and retention properties in the stratum corneum, while their intercellular matrix has permeability barriers to water loss. The normal stratum corneum is desquamated regularly and is not visible. 1 The process of epidermal differentiation is complex and not fully understood. This disorder can be caused by many aspects and stages of this pr