Six pediatric NS patients are currently enrolled in the program, all under 10 years of age, with the youngest being just 6 months old. Four of the six children in the study were classified as ‘Improved' or ‘Significantly Improved' from baseline across key clinical endpoints at the time of this release.

Three of the four treatment responders achieved a minimum of a one-grade improvement from baseline according to an Investigator's Global Assessment (IGA) (Scale 0-clear to 4-severe), with one patient achieving a full 5-grade improvement from 4 (severe) at baseline to 0 (clear) at nine months, and the patient continues to remain clear at 15 months. Of these three responders, two achieved at least a 4-grade improvement from baseline for pruritus (scale 0-10), with one patient's pruritus being completely eliminated. The third responder started with pruritus of 1 and remained at 1 during treatment.

The fourth, and youngest patient responder is 6 months old and has only been on treatment for 3 weeks. Quoin is still waiting for the 6 week safety and efficacy data, however, at baseline, the patient required the application of emollients to their skin 12 times a day, every 2 hours. After 3 weeks of dosing with QRX003, as a result of improvements in the infant's skin, the parents are using almost no emollient on the treated body sites. In addition, prior to receiving QRX003, the infant's neutrophil levels were extremely low putting the infant at significant risk of infection complications. After just 3 weeks of treatment with QRX003, these levels have been fully restored to normal levels, which is yet another clear indication of a positive treatment effect.

Two of the six patients have not achieved any improvement to date, though for one of these the duration of treatment has been less than 8 weeks and the patient remains on treatment. An additional patient was enrolled into the program on June 15th and two others are scheduled to begin treatment by the end of September. Quoin believes the information generated from this growing cohort of pediatric patients could provide valuable supportive evidence of QRX003's longer term safety and efficacy in this key patient population.

There have been no reports of treatment related adverse events for any of the six patients in the program.

Quoin plans a comprehensive release of clinical data from this program and from its ongoing Phase 2 studies in the coming months.