Efficacy and safety of oral sirolimus for high-flow vascular malformations in real clinical practice.

BACKGROUND: Vascular malformations are a complex pathology with few treatment options. In previously published studies, oral sirolimus (rapamycin) has shown promising results in the treatment of low-flow vascular malformations, but its usefulness in high-flow vascular malformations is controversial. AIM: To evaluate the efficacy and safety of sirolimus for the treatment of high-flow vascular malformations in real-life practice. METHODS: In a unit specializing in vascular anomalies, patients treated with oral sirolimus for high-flow vascular malformations were located by consulting the drug dispensations. Reviewing the electronic medical records, data on patient demographics, vascular malformation characteristics, treatments, toxicity and clinical course were collected and statistically analysed. RESULTS: Nine patients with vascular malformations were included: eight had arteriovenous malformation and one had arteriovenous fistula. Six of these malformations were isolated while three were part of a syndrome. Sirolimus was initiated at a dosage of 1-4 mg/day to be taken as a single dose. Partial response was observed in eight of the nine patients (88.9%) with high-flow vascular malformation, while worsening was observed in the remaining patient. The treatment was well tolerated and at the most recent follow-up, five patients remained on treatment with oral sirolimus. CONCLUSION: Our results show that oral sirolimus is a well-tolerated therapeutic option, with an excellent safety profile, which can be useful in the long-term stabilization of patients with high-flow vascular malformations. Single-daily dosage may improve long-term adherence to treatment without worsening its effectiveness.

Microscopía confocal de fluorescencia ex vivo en escala de tres colores (mcf-3cs): una nueva técnica de imagen / Ex Vivo Fluorescence Confocal Microscopy on a 3-Color Scale: A New Imaging Technique

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Determinantes del tratamiento de las queratosis actínicas por médicos de atención primaria / Actinic Keratosis and Primary Care Physicians: Factors Affecting the Decision to Treat or Not

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FR- Exposición solar insuficiente y mortalidad global: ¿debemos recomendar o desaconsejar la exposición solar a nuestros pacientes? / RF- insufficient exposure to sunlight and global mortality: should we advise against or recommend sun exposure?

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Granuloma elastolítico anular de células gigantes en área no fotoexpuesta con respuesta a dapsona / Annular Elastolytic Giant Cell Granuloma in Sun-Protected Sites Responds to Dapsone

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Urticaria vasculitis: estudio retrospectivo de 15 casos / Urticarial Vasculitis: A Retrospective Study of 15 Cases

Introducción: La urticaria vasculitis (UV) es un subtipo de vasculitis caracterizada clínicamente por lesiones de tipo urticarial e histológicamente por vasculitis necrotizante. Objetivo: El objetivo de este trabajo es el estudio de los pacientes con urticaria vasculitis que han sido atendidos en el Servicio de Dermatología del Hospital Universitario Virgen del Rocío (Sevilla, España) para determinar las características de presentación clínicas e histológicas, así como el despistaje de hipocomplementemia, en estrecha relación con enfermedad sistémica asociada. Material y métodos: Se han extraído de la base de datos de nuestro servicio los casos de urticaria vasculitis confirmados histológicamente durante un período de 10 años. Resultados: Quince pacientes fueron diagnosticados de urticaria vasculitis (9 mujeres y 6 hombres, con una mediana de edad de 51 años). En 14 pacientes (93%) las lesiones persistían más de 24 horas. En 9 pacientes (60%) las lesiones se resolvían con púrpura e hiperpigmentación residual. Siete pacientes (47%) presentaron niveles disminuidos de complemento en sangre, 12 pacientes (80%) asociaron síntomas extracutáneos y 8 pacientes (53%) asociaban enfermedad sistémica, siendo la entidad más frecuente el lupus eritematoso sistémico. Conclusiones: La urticaria vasculitis constituye a menudo una entidad infradiagnosticada, con una variable respuesta al tratamiento y en la que la existencia de niveles de complemento disminuidos y de síntomas extracutáneos puede ser indicativa de enfermedad sistémica asociada (AU)

Introduction: Urticarial vasculitis is a subtype of vasculitis characterized clinically by urticarial lesions and histologically by necrotizing vasculitis. Objective: To study the clinical and histologic features of urticarial vasculitis in patients seen in the dermatology department of Hospital Universitario Virgen de Rocío in Seville, Spain, and to examine the association between hypocomplementemia and systemic disease. Material and methods: We performed a chart review of histologically confirmed cases of urticarial vasculitis in the database of our department covering a period of 10 years. Results: Fifteen patients (9 women and 6 men with a median age of 51 years) were included. In 14 patients (93%), the lesions persisted for more than 24 hours, and in 9 cases (60%) the lesions resolved leaving residual purpura or hyperpigmentation. Seven patients (47%) had low complement levels in the blood, 12 (80%) had extracutaneous symptoms, and 8 (53%) had associated systemic disease, the most common of which was systemic lupus erythematosus. Conclusions: Urticarial vasculitis may be underdiagnosed. Response to treatment is variable, and hypocomplementemia and extracutaneous symptoms may indicate the presence of associated systemic disease (AU)

Urticarial vasculitis: a retrospective study of 15 cases.

INTRODUCTION: Urticarial vasculitis is a subtype of vasculitis characterized clinically by urticarial lesions and histologically by necrotizing vasculitis. OBJECTIVE: To study the clinical and histologic features of urticarial vasculitis in patients seen in the dermatology department of Hospital Universitario Virgen de Rocío in Seville, Spain, and to examine the association between hypocomplementemia and systemic disease. MATERIAL AND METHODS: We performed a chart review of histologically confirmed cases of urticarial vasculitis in the database of our department covering a period of 10 years. RESULTS: Fifteen patients (9 women and 6 men with a median age of 51 years) were included. In 14 patients (93%), the lesions persisted for more than 24hours, and in 9 cases (60%) the lesions resolved leaving residual purpura or hyperpigmentation. Seven patients (47%) had low complement levels in the blood, 12 (80%) had extracutaneous symptoms, and 8 (53%) had associated systemic disease, the most common of which was systemic lupus erythematosus. CONCLUSIONS: Urticarial vasculitis may be underdiagnosed. Response to treatment is variable, and hypocomplementemia and extracutaneous symptoms may indicate the presence of associated systemic disease.