DelveInsight’s Netherton Syndrome Market Insights, Epidemiology, and Market Forecast – 2030’ report delivers an in-depth understanding of the historical and forecasted epidemiology as well as the market trends of Netherton Syndrome in the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom), and Japan.
The Netherton Syndrome market report provides current treatment practices, emerging drugs, market share of the individual therapies, current and forecasted Netherton Syndrome market Size from 2018 to 2030, segmented by seven major markets. The report also covers the current Netherton Syndrome treatment practice/algorithm, market drivers, market barriers, and unmet medical needs to curate the best opportunities and assesses the underlying market potential.
Netherton syndrome (NS) is a rare and severe genetic autosomal recessive disorder of Ichthyosis. It is an inherited skin condition, often present at birth or in the first few weeks of life, where the skin is very red with fine dry scales. The condition tends to last for a lifetime, but the severity can vary from person to person.
It was described by Comèl (1949) and Netherton (1958). The syndrome is sometimes called Comèl-NS and is characterized by a clinical trial of ichthyosiform erythroderma, a specific hair shaft abnormality (trichorrhexis invaginata, or bamboo hair), atopic manifestations, and multisystemic complications.
NS is caused by loss of function mutations in the SPINK5 gene that encodes a serine peptidase inhibitor, Lympho-epithelial Kazal-type-related inhibitor (LEKTI). The loss of LEKTI leads to dysregulation of epidermal proteases and severe skin barrier impairment. Kallikrien-related peptidases, such as KLK7, and the epidermal elastase 2 (ELA2), which LEKTI inhibits, are reported to play major roles in NS pathology.
NS is diagnosed based on the symptoms and confirmed by genetic testing for changes in the SPINK5 gene. Other types of testing, such as close examination of the hair and a skin biopsy to obtain a small skin sample for examination under the microscope, can also be helpful for diagnosis. Often babies or children are not diagnosed for months or even years as red skin at birth can be a symptom of other conditions, such as other types of ichthyosis, severe eczema, or other forms of immune deficiency. Thus, differential diagnosis plays an important role.
There is no approved treatment for NS. Current approaches are all limited to symptom relief or supportive care with marginal efficacy and undesirable side effects. The treatment includes cleansing skin by non-detergent liquid cleansing oil, use of emollients, moisturizers, keratolytic, antipruritics, anti-histamines, antibiotics, topical corticosteroids, retinoids, calcipotriol, and Calcineurin inhibitors. Intravenous immunoglobulin and anti-TNF-α are therapeutic options for severe illness.
There is an urgent need for a targeted, effective and well-tolerated therapy. Further understanding of the underlying pathophysiology of skin changes will lead to more effective therapeutic modalities, including possible gene therapy.
Original Source:- Netherton Syndrome Market Research Report
