Actualización en malformaciones venosas

Venous malformations represent 2/3rds of all vascular malformations and are frequently much more complex than they appear to be. Patients with large venous malformations require a deep analytical and radiological study, as well as specific treatment to control any possible localised intravascular coagulation. If the lesions are extensive, especially in the lower member, a study should be made to detect the presence of an underlying osteoporosis with the idea of preventing pathological fractures. Equally, a check must be made for arthropathy, and an early prophylactic synovectomy must be considered when the radiological extension makes this advisable, with the idea of avoiding irreversible damage to the joints with the passage of time. Currently, microfoam scleropathy is favoured as the treatment of choice for low-flow vascular malformations. In the not too distant future, the use of selective antiangiogenic medicines, besides low-molecularweight heparins

Lesions of pemphigus vulgaris on irradiated skin

Summary Pemphigus vulgaris (PV) is an autoimmune blistering disease produced by IgG autoantibodies against desmoglein (Dsg)3. Lesions on the skin and mucosa can, in rare cases, be induced by radiotherapy. We report a patient with a history of microprolactinoma and PV, who had only oral lesions from the beginning of her illness but 2 months after treatment with radiotherapy for a breast neoplasia, developed skin lesions limited to the irradiated area. Over the following few months, she also developed autoantibodies against Dsg

Actualización en malformaciones venosas

Venous malformations represent 2/3rds of all vascular malformations and are frequently much more complex than they appear to be. Patients with large venous malformations require a deep analytical and radiological study, as well as specific treatment to control any possible localised intravascular coagulation. If the lesions are extensive, especially in the lower member, a study should be made to detect the presence of an underlying osteoporosis with the idea of preventing pathological fractures. Equally, a check must be made for arthropathy, and an early prophylactic synovectomy must be considered when the radiological extension makes this advisable, with the idea of avoiding irreversible damage to the joints with the passage of time. Currently, microfoam scleropathy is favoured as the treatment of choice for low-flow vascular malformations. In the not too distant future, the use of selective antiangiogenic medicines, besides low-molecularweight heparins

Terapia fotodinámica con luz de día para la prevención de nuevas queratosis actínicas y carcinomas queratinocíticos en trasplantados de órgano sólido. Ensayo clínico aleatorizado controlado con crioterapia

Introducción: Los pacientes trasplantados de órgano sólido (TOS) presentan un riesgo aumentado de la incidencia de queratosis actínicas (QA) y carcinomas queratinocíticos (CQs) debido a la terapia inmunosupresora. No hay ensayos clínicos que evalúen la efectividad de la modalidad de TFD con luz de día (TFDLD) para la prevención de nuevas QAs y CQs mediante el tratamiento repetido del campo de cancerización en pacientes TOS. Objetivo: Evaluar si el tratamiento repetido del campo de cancerización con TFDLD con metil aminolevulinato (MAL) puede prevenir la aparición de nuevas QAs y CQs en pacientes TOS. Evaluar la tolerancia al dolor, la preferencia de tratamiento, la satisfacción del paciente con el tratamiento, el resultado cosmético y la calidad de vida. Métodos: Ensayo clínico aleatorizado, controlado intraindividualmente, con evaluador ciego, desde abril de 2016 hasta octubre de 2018. Se incluyeron pacientes TOS mayores de 18 años, al menos un año post-trasplante, con al menos 5 QAs en cada hemicara/hemicuero cabelludo. Los criterios de exclusión fueron hipersensibilidad al fármaco, porfiria, embarazo y lactancia. Se realizó tratamiento del campo de cancerización en un lado de la cara y/o cuero cabelludo con TFDLD y el lado contralateral fue el control. El lado asignado aleratoriamente a TFDLD recibió un total de 6 sesiones, dos sesiones separadas 15 días al inicio del estudio, 2 sesiones separadas 15 días a los 3 meses del inicio y 2 sesiones separadas 15 días a los 9 meses del inicio. En el lado control no se trató el campo de cancerización, pero las QAs visibles se trataron con terapia dirigida a la lesion (doble ciclo de congelación- descongelación con crioterapia) al inicio, a los 3 y a los 9 meses (en total 3 sesiones). Se realizó seguimiento a los 15 y 21 meses. Resultados: De los 24 pacientes incluidos, 23 fueron analizados a los 3 meses y 21, a los 9, 15 y 21 meses. Todos los pacientes fueron hombres y de raza caucásica. La edad media fue 69.8 años (SD 9.2). El número total de lesiones (nuevas y persistentes) a los 21 meses fue menor en el lado de la TFDLD que en el lado control (media 4.7 [SD 4.3] versus 5.8 [5.0], respectivamente, P=0.09). A lo largo de todo el estudio hubo un menor número de lesiones nuevas en el lado que recibió tratamientos repetidos del campo de cancerización con TFDLD en comparación con el lado control. Estas diferencias fueron estadísticamente significativas a los 3 meses (media 4.2 [SD 3.4] versus 6.8 [4.8], p<0.001) , a los 9 meses (3.0 [3.3] versus 4.3 [3.4], P=0.04), y a los 15 meses (3.0 [4.6] versus 4.8 [5.0], P=0.02); y no significativas a los 21 meses (3.7 [3.5] versus 5 [4.5], P=0.06). El hecho de que hubiera una diferencia a los 15 y 21 meses (aunque no fuera estadísticamente significativa a los 21 meses) sugieren que el régimen de tratamientos repetidos podría prolongar el efecto preventivo de la TFDLD. La TFDLD fue significativamente mejor tolerada que la crioterapia. Los pacientes estuvieron más satisfechos con el resultado obtenido con la TFDLD y la mayoría prefirió la TFDLD a la crioterapia. No se encontraron diferencias significativas en el resultado cosmético (rejuvenecimiento cutáneo global, cambios en la pigmentación y cicatrices). No hubo diferencias en la escala de calidad de vida (Skindex-29) al comparar ambos lados pero si al comparar los cuestionarios antes del inicio de los tratamientos y al final del estudio. Conclusiones: La TFDLD es un tratamiento con potencial para prevenir nuevas QAs y CQs en pacientes TOS mediante tratamientos repetidos del campo de cancerización. La mayor preferencia de los pacientes por la TFDLD puede facilitar la adherencia al tratamiento a largo plazo necesario en estos pacientes por el carácter crónico de su patología

Queratosis actínicas en pacientes trasplantados de órgano sólido: revisión de la literatura

La inmunosupresión farmacológica de los pacientes trasplantados de órgano solido constituye un importante factor de riesgo tanto para la aparición de queratosis actínicas (QA) como para su progresión a carcinomas escamosos (CE). El tratamiento tanto de las lesiones clí- nicas como preclínicas en este grupo de pacientes es obligatorio debido a la elevada posibilidad de evolución a CE. Por otra parte, la prevención presenta también un papel importante que debemos tener en cuenta. Existen un gran número de estudios realizados en pacientes inmunocompetentes sobre el tratamiento y la prevención de QA, pero no en pacientes inmunosuprimidos. Esta revisión pretende resumir el conocimiento actual sobre los tratamientos y medidas de prevención de la QA en loas pacientes trasplantados de órgano sólido.Pharmacological immunosuppression in solid organ transplant recipients is a signifi- cant risk factor in the occurrence of actinic keratosis (AK) and later progression into squamous cell carcinomas (SCC). Treating clinical and preclinical lesions is mandatory in this group of patients due to the high changes of progression into SCC. On the other hand, prevention of AK should be considered because it plays a crucial role. Several studies have been published on immunocompetent patients, as well as on the mana- gement and prevention of AK, but not on immunosuppressed patients. This review aims to summarize the current knowledge on the management and prevention measures of AK in solid organ transplant recipients

Successful topical sirolimus treatment of epidermal nevus in a patient with phacomatosis spilosebacea

Epidermal nevi (EN) are hamartomas of keratinocytic or epidermal appendages which are most frequently seen as isolated birthmarks. EN syndromes (ENS) are a heteroge- neous group of disorders caused by somatic mosaicism and characterized by the presence of an EN with associated in- volvement of other organ systems.1 Phacomatosis spilose- bacea (PSS) is a rare ENS defined by the co-occurrence of papular speckled lentiginous nevus (or nevus spilus) and nevus sebaceus (NS), a common type of EN.2 Postzygotic- activating HRAS mutation in a multipotent progenitor cell has been described as the underlying pathogenetic mecha- nism of both types of nevi in PSS.2,3 Treatment of EN can be challenging. Although full-thickness excision is curative, these lesions may occur in locations where the surgery would result in disfigurement. Other treatments like topical corti- costeroids, retinoic acid and 5-fluorouracil or ablative laser have been used with different and limited results.1,

Daylight photodynamic therapy for prevention of new actinic keratosis and keratinocyte carcinomas in organ transplants. A cryotherapy-controlled randomized clinical trial

Background Organ transplant recipients (OTR) have a higher risk of actinic keratosis (AK) and keratinocyte carcinomas (KC). There are no clinical trials assessing the effectiveness of daylight photodynamic therapy (DPDT) to prevent new AK and KC in OTR. Objectives To determine whether repeated treatments of field cancerization with DPDT are effective in preventing new AK and KC in OTR. Methods A randomized, intra-subject controlled, evaluator-blind, split-face and/or scalp trial, from April 2016 to Octo- ber 2018. Participants were OTR older than 18 years, 1-year posttransplant, with at least 5 AK on each hemi-face/ hemi-scalp. One side received six field treatments with DPDT: two sessions 15 days apart at baseline, two at 3 months and two at 9 months after baseline. Control side received lesion-directed treatment with cryotherapy (double freeze– thaw) at baseline, 3 and 9 months. Total number of lesions (AK and KC) at 21 months, number of new AK and KC at 3, 9, 15 and 21 months and treatment preferences were analysed. Results Of 24 men included, 23 were analysed at 3 months; and 21, at 9, 15 and 21 months. Mean (SD) age was 69.8 years (9.2). The total number of lesions at 21 months was 4.7 (4.3) for DPDT and 5.8 (5.0) for control side; P = 0.09. DPDT showed significantly lower means [SD] of new lesions compared to control side at 3 months (4.2 [3.4] vs. 6.8 [4.8]; P < 0.001), 9 months (3.0 [3.3] vs. 4.3 [3.4]; P = 0.04) and 15 months (3.0 [4.6] vs. 4.8 [5.0]; P = 0.02), and non-signifi- cant at 21 months (3.7 [3.5] vs. 5.0 [4.5]; P = 0.06). Most participants preferred DPDT. Conclusion DPDT showed potential effectiveness in preventing new AK and KC in OTR by consecutive treatments of field cancerization. The preference for DPDT could facilitate adherence to the long-term treatment necessary in these patients

Daylight photodynamic therapy for prevention of new actinic keratosis and keratinocyte carcinomas in organ transplants. A cryotherapy-controlled randomized clinical trial

Background: Organ transplant recipients (OTR) have a higher risk of actinic keratosis (AK) and keratinocyte carcinomas (KC). There are no clinical trials assessing the effectiveness of daylight photodynamic therapy (DPDT) to prevent new AK and KC in OTR. Objectives: To determine whether repeated treatments of field cancerization with DPDT are effective in preventing new AK and KC in OTR. Methods: A randomized, intra-subject controlled, evaluator-blind, split-face and/or scalp trial, from April 2016 to October 2018. Participants were OTR older than 18 years, 1-year posttransplant, with at least 5 AK on each hemi-face/hemi-scalp. One side received six field treatments with DPDT: two sessions 15 days apart at baseline, two at 3 months and two at 9 months after baseline. Control side received lesion-directed treatment with cryotherapy (double freeze-thaw) at baseline, 3 and 9 months. Total number of lesions (AK and KC) at 21 months, number of new AK and KC at 3, 9, 15 and 21 months and treatment preferences were analysed. Results: Of 24 men included, 23 were analysed at 3 months; and 21, at 9, 15 and 21 months. Mean (SD) age was 69.8 years (9.2). The total number of lesions at 21 months was 4.7 (4.3) for DPDT and 5.8 (5.0) for control side; P = 0.09. DPDT showed significantly lower means [SD] of new lesions compared to control side at 3 months (4.2 [3.4] vs. 6.8 [4.8]; P < 0.001), 9 months (3.0 [3.3] vs. 4.3 [3.4]; P = 0.04) and 15 months (3.0 [4.6] vs. 4.8 [5.0]; P = 0.02), and non-significant at 21 months (3.7 [3.5] vs. 5.0 [4.5]; P = 0.06). Most participants preferred DPDT. Conclusion: DPDT showed potential effectiveness in preventing new AK and KC in OTR by consecutive treatments of field cancerization. The preference for DPDT could facilitate adherence to the long-term treatment necessary in these patients