6 research outputs found

    New Use of Rapamycin Stent in Non-Responding Facial Lymphatic Malformation

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    Introduction: Vascular anomalies represent a diagnostic and therapeutic difficulty. A lymphatic malformation (LM) corresponds to a low-flow vascular malformation. Sclerotherapy is the preferred treatment. Recently, angiogenesis inhibitors such as Rapamycin have been used with promising results. Case Report: A pediatric patient presented with a large facial LM and poor response to Sildenafil and sclerotherapy. After a persistent enlargement of the lesion, with painful ocular occlusion a new sclerotherapy was performed obtaining partial improvement. As a rescue treatment an IRS was installed. The patient achieved a rapid and evident response, with better ocular aperture and pain control. Discussion: LM correspond to vasculogenesis disorders with persistent growth over time. They can present high morbidity. The complications in the facial area are infection, hemorrhage, exophthalmos, and amblyopia. Rapamycin is a potent angiogenesis inhibitor. Rapamycin stents have been safely used in cardiology. They provide local concentration with less adverse effects. There are no reports on their use in lymphatic malformation. The patient had a favorable and persistent response. Conclusion: IRS should be considered as a therapeutic alternative in severe nonresponding lymphatic malformation. The necessity of a multidisciplinary approach is fundamenta

    Predictive Model for Response Rate to Narrowband Ultraviolet B Phototherapy in Vitiligo: A Retrospective Cohort Study of 579 Patients

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    Vitiligo is an acquired depigmenting disorder. To date, there is no predictive model for its response rate to narrowband ultraviolet B (NBUVB) phototherapy. The aim of this study was to investigate the different types of response of patients with non-segmental vitiligo undergoing NBUVB 3 times a week. Many patients who were previously considered non-responders were given the opportunity to continue the treatment. Longterm maintenance of treatment and follow-up of a cohort of 579 patients enabled different subtypes of response (very rapid, rapid, average, slow and “nonresponders”) to be described for the first time, and a predictive model of response to be constructed based on repigmentation rate in the first 48 sessions of NBUVB. Among those patients who did not respond during the first 48 sessions, a new subgroup of patients was found, termed “very-slow” responders, who achieved a low, but significant, level of repigmentation after 96 sessions of NBUVB.Versión Publicad

    Predictive model for response rate to narrowband ultraviolet B phototherapy in vitiligo: A retrospective cohort study of 579 patients

    No full text
    Vitiligo is an acquired depigmenting disorder. To date, there is no predictive model for its response rate to narrowband ultraviolet B (NBUVB) phototherapy. The aim of this study was to investigate the different types of response of patients with non-segmental vitiligo undergoing NBUVB 3 times a week. Many patients who were previously considered non-responders were given the opportunity to continue the treatment. Longterm maintenance of treatment and follow-up of a cohort of 579 patients enabled different subtypes of response (very rapid, rapid, average, slow and "non-responders") to be described for the first time, and a predictive model of response to be constructed based on repigmentation rate in the first 48 sessions of NBUVB. Among those patients who did not respond during the first 48 sessions, a new subgroup of patients was found, termed " very-slow" responders, who achieved a low, but significant, level of repigmentation after 96 sessions of NBUVB

    Bullous systemic lupus erythematosus: An uncommon manifestation in pediatric population

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    Bullous systemic lupus erythematosus (BSLE) is an autoimmune subepidermal blistering disease secondary to the presence of autoantibodies against type VII collagen of the basement membrane zone. It is considered a variant of Systemic Lupus Erythematosus (SLE) and is uncommon in the pediatric population. Objective: To describe the case of a pediatric patient with a bullous eruption compatible with BSLE. Clinical Case: A 16-year-old female patient of Mapuche descent with history of SLE diagnosed at age 10, undergoing treatment. She consulted due to a six-week history of a generalized bullous eruption with no systemic symptoms. Biopsy for histology and direct immunofluorescence (DIF) confirmed the diagnosis of BSLE. The patient responded favorably to dapsone 100 mg/day (associated with her baseline treatment), without new reactivations after 8 years of follow-up. Conclusion: BSLE is an infrequent manifestation of SLE. The clinical presentation is similar to other bullous dermatoses, but the histopathology and DIF in correlation with the presence of SLE confirm the diagnosis. Although indigenous ancestry is associated with SLE high-risk alleles, studies regarding the association of BSLE in this ethnic group are still lacking
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