Quoin Pharmaceuticals, Ltd.

Quoin is an emerging specialty pharmaceutical company dedicated to the development and commercialization of therapeutic products that treat rare and orphan diseases for which there are currently no approved treatments. Quoin’s lead product, QRX003, is a once daily, topical lotion to treat Netherton Syndrome, a rare hereditary skin disease. In addition, the company intends to pursue the clinical development of QRX003 in other orphan dermatological indications, including Peeling Skin Syndrome, SAM Syndrome and Palmoplantar Keratoderma. Clinical testing of QRX003 is currently underway in two studies under an open IND. Quoin’s second lead product, QRX004, is being developed as a potential treatment for Dystrophic Epidermolysis Bullosa. A further product, QRX008, is under development for scleroderma, a devastating rare disease for which there is currently no approved treatment or cure.  Quoin intends to The company will establish a sales infrastructure to commercialize its products in both US and Europe and seek to enter into strategic licensing partnerships for all other territories. Currently, eight such partnerships have been established covering 60 countries.

• Experienced Management Team with proven track record of success

• Focused Rare and Orphan Disease product pipeline

• Rare Pediatric Designation opportunity for lead products

• Targeting US/EU Approvals in 2024, 2025 and 2026.

• Company will establish sales infrastructure for both US and EU markets

• Differentiated polymer delivery technology with Strong IP Position

• Strong KOL support from leading experts

QRX003 is a once-daily topical lotion being developed for the treatment of Netherton Syndrome, a rare hereditary skin disease for which there is currently no approved treatment or cure. 

The active ingredient in QRX003 is a competitive broad-spectrum serine protease inhibitor whose mechanism of action is to target the serine proteases present in the skin, called Kallikreins, which are responsible for the process of skin shedding. As a result of the genetic mutation that causes Netherton Syndrome, these Kallikreins go unregulated and become hyperactive resulting in the uncontrolled desquamation that leads to the highly defective skin barrier in Netherton patients. When applied daily to the skin, QRX003 acts to regulate the activity of these Kallikreins leading to a more normalized skin shedding process and the formation of a stronger and more effective skin barrier. 

• Patented polymer delivery system

• Provides immediate barrier protection on skin

• Withstands frequent washing, allowing once daily application  

• Unique polymer combination both moisturizes and protects skin

An estimated 6,000-7,000 individuals in the US and Europe combined are believed to have Netherton Syndrome, with a birth incidence of approximately 1:200,000.  QRX003 has the potential to become the first approved treatment for Netherton Syndrome to reach the market both in the U.S. and Europe and may therefore likely be used in a large proportion of patients. It is intended that QRX003 will be applied once daily to the patient’s whole body for the remainder of their life thus providing a long-term opportunity for the product. Outside of Europe, there are additional attractive market opportunities in Canada, Australia, the Middle East and South East Asia. 

Quoin’s management believes the rare and orphan disease space represents an attractive commercial opportunity for several reasons. The overall success rate for obtaining regulatory approval for orphan drugs is estimated to be 26% compared to just 11% for all other indications. Worldwide orphan drug sales are forecast to grow at a compound annual growth rate (CAGR) of 12.3% from 2019-2024, essentially double the forecast for the non-orphan drug market. By 2024, orphan drug sales are expected to reach $242 billion and capture one-fifth of total worldwide sales. Furthermore, the mean drug price of the top 100 US orphan drugs was almost 4.5 times greater than the non-orphan drug price in 2018 and the small patient populations coupled with the lack of other treatment options greatly enhance the likelihood of insurance coverage and reimbursement.

Quoin intends to self-commercialize QRX003, and other rare disease products the company may develop, in both the US and Europe.  Because of the very low number of patients and the fact that diagnosis and treatment is generally provided by a relatively small number of board-certified dermatologists in major urban areas, this concentration of care will enable us to market QRX003 with a small, dedicated salesforce to target those patients and caregivers. Outside of the U.S and in Europe, Quoin intends to establish marketing partnerships for QRX003 in Canada, Australia, the Middle East and Asia.  Currently eight such partnerships have been established covering Australia, New Zealand, the Middle East, China, Taiwan, Hong Kong, Latin America as well as Central and Eastern Europe and Turkey.

Netherton Syndrome (NS) – a rare, hereditary skin disorder – is an autosomal recessive genetic disease caused by a mutation in the SPINK5 gene (serine protease inhibitor, Kazal Type 5). The SPINK5 gene, when behaving normally, encodes a protein, called Lympho- Epithelial Kazal Type Related Inhibitor or LEKTI, that serves to regulate the activity of certain proteases (enzymes that digest proteins) in the skin. These enzymes are called Kallikreins and consist of the KLK5, KLK7 and KLK14 proteases. For patients with NS, the LEKTI protein is absent due to the mutation of the SPINK5 gene. This results in the Kallikreins being completely unregulated, leading to excessive skin shedding and the highly porous and defective skin barrier that NS patients have.

Unmet Medical Needs in Netherton Syndrome
Currently, there is no cure for Netherton Syndrome nor are there any approved therapeutic treatments. What is available, such as skin moisturizers, are currently limited to providing symptomatic relief. Due to the highly compromised skin, even the selection of an appropriate moisturizer can be challenging. For example, moisturizers typically used for skin barrier repair, such as those containing lanolin and petrolatum, can cause further skin damage to NS patients due to friction or high shear forces upon application or removal. Topical steroids may have harmful rather than helpful effects and can further reduce the thickness of the skin barrier. Incidences of Cushing’s Syndrome have also been reported from the use of topical steroids. Other standard topical treatments, such as calcineurin inhibitors, can lead to dangerously high systemic blood levels due to the defective skin barrier. There is a critical need for a new and effective treatment for NS.

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