ichthyoses

Summary, Conclusion and Recommendations The aim of this review IS to discuss the updates on classification,c1inical types and advances concerning, genetics,molecular biology and pathogenesis of hereditary and acquired ichthyoses. Also the recent advances in the management including gene therapy. Our understanding of how various genes are involved In causing ichthyosis has increased enormously over the last decade, revealing a spectrum of diverse pathogenic mechanisms that extend from abnormal structural proteins (keratins, filaggrin, loricrin, cornified cell envelope, etc.) to deficient enzymes or transport proteins essential for the lipid metabolism in the epidermis (cholesterol sulphatase, lipoxygenases, ABCA 12. etc.).The classification of the common types of ichthyoses is based on their clinical appearance, genetic back ground and histologic findings. Many classifications are present, the simplest one is to classify it into hereditary ichhyoses including: primary ichthyoses, ichthyosiform syndromes and still there are considerable numbers of isolated familial disorders that can be classified as ichthyosis;and acquired ichthyosis. The most common primary ichthyoses are ichthyosis vulgaris, X- Linked ichthyosis, ARCI (non bullous ichthyosiform erythroderma, Lamellar ichthyosis and Harlequin ichthyosis) and bullous congenital ichthyosiform erythroderma. Ichthyosiform syndromes includes;Netherton’s syndrome,sj?gren larsson syndrome,Refsum disease etc. Acquired ichthyosis may be associated with Mal ignancies as lymphoma, Metabolic diseases, Infectious diseases, Autoimmune! connective tissue diseases and drugs.