ABSTRACT
Objectives: The 90-day double-blind phase (DBP) of the Phase 3 EASE study demonstrated accelerated wound healing for Oleogel-S10 (birch triterpenes) versus control gel in epidermolysis bullosa (EB). Here, we report safety and total wound burden results from the 24-month open-label phase (OLP) in which all patients received treatment with Oleogel-S10. Method(s): Total wound burden was assessed using EB Disease Activity and Scarring Index (EBDASI) and Body Surface Area Percentage (BSAP). Data are reported without visit windows to reflect a realworld situation more accurately, particularly considering the COVID- 19 pandemic. Result(s): The patient population was made up of dystrophic EB (n = 178, 86.8%) and junctional EB (n = 25, 12.2%);71.7% (n = 147) of patients were aged <18 years. 141 patients (68.8%) completed the OLP. The mean (SD) treatment duration for all patients was 584.7 (246.1) days. Adverse events were reported in 77.1% of all patients in the OLP versus 81.7% of those receiving Oleogel-S10 in the DBP. Mean BSAP for patients treated with Oleogel-S10 in the DBP reduced from 12.1% at study entry to 6.1% with 27 months of treatment. Similarly, the mean EBDASI skin activity score in the Oleogel-S10 group improved from 19.6 to 15.1 after 27 months. In addition, reductions in both BSAP and EBDASI from OLP baseline were observed in patients who transitioned from control gel to Oleogel-S10 in the OLP. Discussion(s): These data support a reassuring long-term safety profile of Oleogel-S10. Furthermore, the reduction in wound burden previously reported with 15 months of Oleogel-S10 treatment is maintained to the end of OLP. This is encouraging given the nature of this chronic genetic disorder in which there is regular cycling of patients' fragile wounds.
ABSTRACT
A novel SARS -CoV2 virus (COVID 19) that led to an outbreak of pneumonia in Wuhan, China is now known to involve multiple organ systems presenting with acute respiratory distress syndrome, gastrointestinal disease, cardiac disease, skin lesions, renal involvement, hepatic damage, and multi organ failure. Even though the mortality and morbidity of this disease is higher in the older age group, clusters of children having severe illness requiring critical care have also been identified. Severe disease in children infected with COVID 19 is characterized by a multisystem inflammatory condition and a clinical presentation similar to Kawasaki disease. COVID 19 and its multi organ involvement is due to the underlying hyperinflammatory syndrome leading to a surge in cytokine levels causing injury to several cell types including cardiac myocytes and endothelial cells. The binding of SARS CoV2 to the ACE 2 receptor expressed in the endothelial cells, pneumocytes and myocardial cells is responsible for the clinical manifestations occurring in skin, heart and other organs. © Springer Nature Switzerland AG 2021.