Oleogel-S10 in Dystrophic Epidermolysis Bullosa: A Case Series Evaluating the Impact on Wound Burden Over Two Years.

Epidermolysis bullosa (EB) is a group of rare, difficult-to-treat, inherited multisystem diseases affecting epithelial integrity. Impaired wound healing is central and can lead to serious clinical complications, deformities, and symptoms with a devastating impact on quality of life (QoL). Dressing changes and wound care are central to the management of EB. Recently Oleogel-S10 (also known as birch bark extract or birch triterpenes) was approved in Europe and the UK for treating EB wounds. This approval was based on data from the EASE phase 3 study, which demonstrated Oleogel-S10 accelerated wound healing, reduced total wound burden, and decreased the frequency of dressing changes in patients with EB. A retrospective analysis of medical records was conducted for up to 24 months in 13 patients with EB treated with Oleogel-S10 through an early access programme in Colombia. Effectiveness was assessed by measuring body surface area percentage (BSAP) and total body wound burden (EBDASI). Tolerability and safety were monitored throughout. This is the first report to evaluate the effectiveness of Oleogel-S10 in clinical practice. The results showed a reduction in percentage of BSA affected, from a mean of 27.3% at baseline to 10.4% at 24-month follow-up, despite treatment interruptions. A reduction in EBDASI skin activity score of - 16.2 (24 months) together with a reduced skin damage index score of - 15.4 (18 months) was also observed. Physicians, patients, and caregivers perceived faster wound closure. Adherence with therapy by patients was good, and patients expressed satisfaction with treatment and reported improvements in self-esteem, productivity, and social interaction. Oleogel-S10 was well tolerated; however, two patients reported worsening wounds related to gauze adherence. Two deaths during treatment interruption were reported and was not considered related to Oleogel-S10. This study supports the effectiveness of Oleogel-S10 in a real-world scenario in a country with scarce resources for the treatment of EB.

Linfoma primario cutáneo difuso de Células B grandes, tipo pierna / Primary cutaneous diffuse large B-cell Lymphoma, leg type (PCLBCL-LT)

El PCLBCL-LT es un trastorno linfoproliferativo infrecuente de mal pronóstico, que afecta principalmente ancianos con fuerte predominio en mujeres, caracterizado por nóduloso tumores rojo azulados, solitarios o agrupados en las piernas. El exámen histopatológico presenta células grandes como centroblastos e inmunoblastos, con fenotipo para linaje B y fuerte expresión para Bcl-2. El tratamiento para lesiones solitarias o poco numerosas consiste en radioterapia, y en casos de compromiso multifocal poliquimioterapia.Comunicamos un caso de una paciente de 74 años con PCLBCL-LT, con antecedente de leucemia linfocítica crónica (LLC), que consultó por tumores eritematosos de rápida evoluciónen las piernas y el brazo izquierdo. Se planteó como diagnóstico un linfoma cutáneo primario de células B, así como también se estableció diagnóstico diferencial con el síndrome de Richter, linfomas cutáneos CD 30 + y leucemia cutis...