Thelansis

 Netherton syndrome (NS) is a multisystemic genetic disease characterized by a triad of clinical manifestations: congenital ichthyosiform erythroderma, immune dysregulation, and hair shaft abnormalities. There are currently no specific therapies accessible for patients with NS due to the disease's complex pathogenesis. It is characterized by a triad of congenital ichthyosiform erythroderma (CIE) or ichthyosis linearis circumflexa(ILC), hair shaft abnormalities, and atopic diathesis. Mutations in the serine protease inhibitor Kazal-type 5 (SPINK5) gene, which encodes lymphoepithelial Kazal-type-related inhibitor (LEKTI), are responsible for NS. LEKTI is expressed in hair follicles and the granular layer of the epidermis. LEKTI deficiencies or abnormalities cause premature stratum corneum detachment and a defect in skin barrier function resulting from excessive serine protease activity. The phenotypic manifestations of NS vary widely like Bamboo hair (trichorrhexis invaginata), ichth...

  Netherton syndrome (NS) is a skin disorder that is distinguished by congenital ichthyosiform erythroderma (CIE), a specific hair shaft defect (trichorrhexis invaginata; TI), and atopic manifestations. Mutations in the SPINK5 gene (5q31-q32), which codes for the serine protease inhibitor LEKTI, cause NS. LEKTI deficiency causes an increase in trypsin-like hydrolytic activity in the stratum corneum (SC), resulting in SC premature desquamation and a severe skin barrier defect. Netherton syndrome (NS) incidence is estimated at 1/200,000 births. Thelansis’s “Netherton Syndrome (NS) Market Outlook, Epidemiology, Competitive Landscape , and Market Forecast Report – 2021 To 2032" covers disease overview, epidemiology, drug utilization, prescription share analysis, competitive landscape, clinical practice, regulatory landscape, patient share, market uptake, market forecast, and key market insights under the potential Netherton Syndrome (NS) treatment modalities options for eig...