Dermatosis ampollar por IgA lineal inducida por Vancomicina: reporte de un caso / Vancomycin-induced linear igabollous dermatosis: a case report

RESUMEN La dermatosis ampollar por IgA lineal del adulto (DLA) es una enfermedad autoinmune adquirida infrecuente, caracterizada por el depósito lineal de anticuerpos IgA en la membrana basal. La mayoría de los casos reportados son de causa idiopática, pero esta entidad también se ha visto asociada a ciertos fármacos, siendo la vancomicina el más frecuente. Se presenta un caso de DLA asociada a vancomicina, con extensa afectación cutánea y compromiso mucoso, tratado con dapsona y corticoides sistémicos con buena respuesta.

ABSTRACT Adult linear IgA bollous dermatosis (LABD) is a rare acquired autoimmune disease characterized by linear deposition of IgA antibodies on the basement membrane. Most of the reported cases are of idiopathic cause, but this entity has also been associated with certain drugs, vancomycin being the most frequent. We present a case of LABD associated with vancomycine, with extensive skin and mucosal involvement, treated with dapsone and systemic corticosteroids with a good response.

Correlation Between Cutaneous Manifestations and Functional Alterations in Chronic Venous Disease of the Lower Extremities / Correlación entre las manifestaciones cutáneas y las alteraciones funcionales en la insuficiencia venosa crónica en las extremidades inferiores

Background and aims Few studies have evaluated the correlation between the severity of cutaneous manifestations of chronic venous disease (CVD) of the lower limbs measured by the Clinical, Etiologic, Anatomic and Pathophysiologic classification (CEAP) and the Venous Clinical Severity Score (VCSS) combined, its risk factors, and venous reflux determined by Doppler ultrasonography. The purpose of this study was to compare the clinical severity assessed by CEAP and VCSS with known risk factors for CVD and the severity of venous reflux. Methods A prospective study was carried out on 250 patients with CVD characterized as C2 to C6 according to the CEAP classification, who attended the departments of Dermatology and Vascular Medicine at the Hospital Privado Universitario de Córdoba from April 2013 to December 2014. Chi-square test, Kruskal–Wallis analysis and multivariate logistic regression analysis were performed to examine the relations between these variables. Result Risk factors significantly associated with clinical severity included older age, hypertension, obesity, sedentarism, history of soft tissue infection, deep vein thrombosis (DVT), previous ulcer, and family history of venous ulcer. Both scores showed a good correlation between clinical severity and the presence of superficial, deep or perforating venous reflux. Older age, male gender and a history of DVT were significant risk factors for venous reflux in patients with mild disease. Conclusions In addition to venous reflux, modifiable risk factors such as obesity, sedentarism, and hypertension are associated with CVD severity. Mild cutaneous manifestations may accompany moderate to severe venous reflux, especially in middle-aged or older men with a history of DVT (AU)

Antecedentes y objetivos Pocos estudios han evaluado la correlación entre la gravedad de las manifestaciones cutáneas de la insuficiencia venosa crónica (IVC) en las extremidades inferiores medida conjuntamente utilizando la clasificación Clinical, Etiologic, Anatomic and Pathophysiologic classification (CEAP) y Venous Clinical Severity Score (VCSS), sus factores de riesgo y el flujo venoso determinado mediante ecografía Doppler. El objetivo de este estudio fue comparar la gravedad clínica evaluada mediante CEAP y VCSS y los factores de riesgo conocidos para IVC y la gravedad del reflujo venoso. Métodos Se llevó a cabo un estudio prospectivo de 250 pacientes con IVC caracterizada de C2 a C6 conforme a la clasificación CEAP, que acudieron a los departamentos de Dermatología y Medicina Vascular del Hospital Privado Universitario de Córdoba de abril de 2013 a diciembre de 2014. Se realizaron las pruebas χ2 y Kruskal-Wallis, así como un análisis de regresión logística multivariante para examinar las relaciones entre estas variables. Resultados Los factores significativamente asociados a la gravedad clínica fueron: edad avanzada, hipertensión, obesidad, sedentarismo, historia de infección de tejidos blandos, trombosis venosa profunda (TVP), úlcera previa y antecedentes familiares de úlcera venosa. Ambas puntuaciones reflejaron una buena correlación entre la gravedad clínica y la presencia de reflujo venoso superficial, profundo o perforante. La edad avanzada, el sexo masculino y los antecedentes de TVP fueron factores de riesgo significativos para el reflujo venoso en los pacientes con enfermedad leve. Conclusiones Además del reflujo venoso, los factores de riesgo moderado modificables, tales como la obesidad, el sedentarismo y la hipertensión, están asociados a la gravedad de la IVC. Las manifestaciones cutáneas leves pueden acompañar al reflujo venoso de moderado a severo, especialmente en los varones de mediana edad, o edad avanzada, con antecedentes de TVP (AU)

[Artículo traducido] Correlación entre las manifestaciones cutáneas y las alteraciones funcionales en la insuficiencia venosa crónica en las extremidades inferiores / Correlation Between Cutaneous Manifestations and Functional Alterations in Chronic Venous Disease of the Lower Extremities

Antecedentes y objetivos Pocos estudios han evaluado la correlación entre la gravedad de las manifestaciones cutáneas de la insuficiencia venosa crónica (IVC) en las extremidades inferiores medida conjuntamente utilizando la clasificación Clinical, Etiologic, Anatomic and Pathophysiologic classification (CEAP) y Venous Clinical Severity Score (VCSS), sus factores de riesgo y el flujo venoso determinado mediante ecografía Doppler. El objetivo de este estudio fue comparar la gravedad clínica evaluada mediante CEAP y VCSS y los factores de riesgo conocidos para IVC y la gravedad del reflujo venoso. Métodos Se llevó a cabo un estudio prospectivo de 250 pacientes con IVC caracterizada de C2 a C6 conforme a la clasificación CEAP, que acudieron a los departamentos de Dermatología y Medicina Vascular del Hospital Privado Universitario de Córdoba de abril de 2013 a diciembre de 2014. Se realizaron las pruebas χ2 y Kruskal-Wallis, así como un análisis de regresión logística multivariante para examinar las relaciones entre estas variables. Resultados Los factores significativamente asociados a la gravedad clínica fueron: edad avanzada, hipertensión, obesidad, sedentarismo, historia de infección de tejidos blandos, trombosis venosa profunda (TVP), úlcera previa y antecedentes familiares de úlcera venosa. Ambas puntuaciones reflejaron una buena correlación entre la gravedad clínica y la presencia de reflujo venoso superficial, profundo o perforante. La edad avanzada, el sexo masculino y los antecedentes de TVP fueron factores de riesgo significativos para el reflujo venoso en los pacientes con enfermedad leve. Conclusiones Además del reflujo venoso, los factores de riesgo moderado modificables, tales como la obesidad, el sedentarismo y la hipertensión, están asociados a la gravedad de la IVC. Las manifestaciones cutáneas leves pueden acompañar al reflujo venoso de moderado a severo, especialmente en los varones de mediana edad, o edad avanzada, con antecedentes de TVP (AU)

Background and aims Few studies have evaluated the correlation between the severity of cutaneous manifestations of chronic venous disease (CVD) of the lower limbs measured by the Clinical, Etiologic, Anatomic and Pathophysiologic classification (CEAP) and the Venous Clinical Severity Score (VCSS) combined, its risk factors, and venous reflux determined by Doppler ultrasonography. The purpose of this study was to compare the clinical severity assessed by CEAP and VCSS with known risk factors for CVD and the severity of venous reflux. Methods A prospective study was carried out on 250 patients with CVD characterized as C2 to C6 according to the CEAP classification, who attended the departments of Dermatology and Vascular Medicine at the Hospital Privado Universitario de Córdoba from April 2013 to December 2014. Chi-square test, Kruskal–Wallis analysis and multivariate logistic regression analysis were performed to examine the relations between these variables. Result Risk factors significantly associated with clinical severity included older age, hypertension, obesity, sedentarism, history of soft tissue infection, deep vein thrombosis (DVT), previous ulcer, and family history of venous ulcer. Both scores showed a good correlation between clinical severity and the presence of superficial, deep or perforating venous reflux. Older age, male gender and a history of DVT were significant risk factors for venous reflux in patients with mild disease. Conclusions In addition to venous reflux, modifiable risk factors such as obesity, sedentarism, and hypertension are associated with CVD severity. Mild cutaneous manifestations may accompany moderate to severe venous reflux, especially in middle-aged or older men with a history of DVT (AU)

Correlation Between Cutaneous Manifestations and Functional Alterations in Chronic Venous Disease of the Lower Extremities.

BACKGROUND AND AIMS: Few studies have evaluated the correlation between the severity of cutaneous manifestations of chronic venous disease (CVD) of the lower limbs measured by the Clinical, Etiologic, Anatomic and Pathophysiologic classification (CEAP) and the Venous Clinical Severity Score (VCSS) combined, its risk factors, and venous reflux determined by Doppler ultrasonography. The purpose of this study was to compare the clinical severity assessed by CEAP and VCSS with known risk factors for CVD and the severity of venous reflux. METHODS: A prospective study was carried out on 250 patients with CVD characterized as C2 to C6 according to the CEAP classification, who attended the departments of Dermatology and Vascular Medicine at the Hospital Privado Universitario de Córdoba from April 2013 to December 2014. Chi-square test, Kruskal-Wallis analysis and multivariate logistic regression analysis were performed to examine the relations between these variables. RESULTS: Risk factors significantly associated with clinical severity included older age, hypertension, obesity, sedentarism, history of soft tissue infection, deep vein thrombosis (DVT), previous ulcer, and family history of venous ulcer. Both scores showed a good correlation between clinical severity and the presence of superficial, deep or perforating venous reflux. Older age, male gender and a history of DVT were significant risk factors for venous reflux in patients with mild disease. CONCLUSIONS: In addition to venous reflux, modifiable risk factors such as obesity, sedentarism, and hypertension are associated with CVD severity. Mild cutaneous manifestations may accompany moderate to severe venous reflux, especially in middle-aged or older men with a history of DVT.

Prognostic biomarkers in primary progressive multiple sclerosis: Validating and scrutinizing multimodal evoked potentials.

OBJECTIVE: To validate the prognostic value of multimodal evoked potentials (mmEP) in primary progressive multiple sclerosis (PPMS) and to determine the most predictive EP-modalities. METHODS: Thirty-nine patients with PPMS (expanded disability status scale (EDSS): 2.0-6.5; mean clinical follow-up: 2.8 years) had visual (VEP), upper and lower limb somatosensory (SEP) and motor EP (MEP) at baseline. Quantitative EP-scores for single (qVEP, qSEP, qMEP) and combined modalities were correlated to EDSS and compared to previously published data of 21 PPMS patients. Predictors of EDSS-change were analyzed in pooled data by linear regression. RESULTS: Samples were comparable. Except qVEP, all EP-scores were correlated to EDSS at baseline (Rho: 0.45-0.69; p < 0.01) and follow-up (Rho: 0.59-0.80; p < 0.001). Combined EP-modalities significantly predicted EDSS-change (R2adj: 0.24), while EDSS and age did not. Tibial qSEP (R2adj: 0.22) and qMEP (R2adj: 0.26) were the best single modality predictors, outperformed by their combination (R2adj: 0.32). CONCLUSIONS: Quantitative EP-scores predict up to 32% of EDSS-change over three years. Modalities representing motor and long tract function carry the main prognostic information. SIGNIFICANCE: Replication of previous results corroborates the use of mmEP as a prognostic biomarker candidate in PPMS.

Síndrome Paraneoplásico: Dermatomiositis secundaria a carcinoma de vesícula biliar: Reporte de un caso / Paraneoplastic Syndrome. Dermatomyositis secondary to gallbladder carcinoma: A case report

RESUMEN La dermatomiositis paraneoplásica, y en particular vinculada a carcinoma de vesícula biliar es una entidad infrecuente, con menos de diez casos reportados a nivel mundial. Presentamos un paciente de 74 años con manifestaciones clínicas y dermatológicas asociadas a dermatomiositis, lo que impulsó a realizar estudios más específicos, los cuales permitieron diagnosticar finalmente un carcinoma de vesícula biliar. Haremos un repaso de esta patología en relación con el caso presentado, destacando la importancia del examen dermatológico completo como primer eslabón en el diagnóstico de neoplasias de órganos internos.

ABSTRACT Paraneoplastic dermatomyositis, particularly associated with gallbladder carcinoma, is an infrequent entity, with less than 10 cases reported worldwide. We present a 74-year-old patient with clinical and dermatological manifestations associated with dermatomyositis, which prompted more specific studies, to finally find gallbladder carcinoma. We will review this pathology in relation to the case presented, highlighting the importance of the complete dermatological examination as the first link in the diagnosis of neoplasms of internal organs.

Enfermedad de Paget extramamaria perianal, tratada con imiquimod tópico / Atypical presentation of perianal extramammary Paget's disease, treated with topical imiquimod

RESUMEN La enfermedad de Paget extramamaria (EPEM) perianal es un adenocarcinoma intraepitelial del margen anal de muy baja frecuencia. La forma primaria representa una neoplasia de origen apocrino, mientras que la forma secundaria representa una diseminación pagetoide de una neoplasia maligna en general anorectal. Debido a su similitud clínica con otras patologías de presentación frecuente, se requiere de un alto índice de sospecha para diagnosticarla. La resección quirúrgica amplia o la cirugía micrográfica de Mohs se considera comúnmente el pilar del tratamiento con altas tasas de recurrencia. Estudios previos han demostrado buena respuesta en la EPEM vulvar con imiquimod 5% en crema, pero su eficacia no ha sido bien descrita en la afectación perianal. En este artículo presentamos un caso de EPEM perianal primario con sobreinfección con HPV, al que se le indicó tratamiento tópico con imiquimod.

ABSTRACT Perianal extramammary Paget disease is a very low frequency intraepithelial adenocarcinoma of the anal margin. The primary form represents a neoplasm of apocrine origin, while the secondary form represents a pagetoid spread of a generally anorectal malignant neoplasm. Due to its clinical similarity with other pathologies with frequent presentation, a high index of suspicion is required to diagnose it. Wide surgical resection or Mohs micrographic surgery is commonly considered the mainstay of treatment with high recurrence rates. Previous studies have shown a good response in vulvar extramammary Paget disease with imiquimod 5% cream, but its efficacy has not been well described in perianal involvement. In this article we present a case of primary perianal extramammary Paget disease with HPV superinfection, which received topical treatment with imiquimod.

Características clínicas e histopatológicas de los pacientes con enfermedades ampollares autoinmunes: estudio retrospectivo en el Hospital Privado Universitario de Córdoba / Clinical and histopathological characteristics of autoimmune blister diseases: a retrospective study in Private University Hospital of Córdoba

RESUMEN Las enfermedades ampollares autoinmunes (EAA) comprenden un conjunto heterogéneo de patologías de baja frecuencia de presentación con evolución crónica, remisiones, exacerbaciones y una importante morbimortalidad aun con tratamiento temprano y adecuado. Existen pocos estudios epidemiológicos en nuestra población que incluyan todo el espectro de enfermedades ampollares. Objetivos : Determinar las características clínico-epidemiológicas de las EAA, describir tratamientos realizados, tiempo de control de actividad de la enfermedad y tiempo desde iniciado el tratamiento hasta la remisión completa. Determinar recaídas de las patologías estudiadas, evaluando si existe relación entre las mismas y algún factor reconocido. Materiales y métodos : Estudio retrospectivo y descriptivo. Se incluyeron todos los pacientes con diagnóstico clínico, histopatológico y de inmunofluorescencia de las EAA que hayan tenido seguimiento por al menos un año en el servicio de dermatología del HPUC, en el período de enero de 2008 hasta noviembre de 2018. Resultados : Se incluyeron 53 pacientes de los cuales el 50,9% tenía Penfigoide Ampollar (PA), 22,6% Dermatitis Herpetiforme (DH) y 17% de Pénfigo vulgar (PV). La edad de los pacientes se encontró entre 21 y 90 años. Las ampollas tensas y erosiones fueron las presentaciones más comunes. Los tratamientos sistémicos se realizaron en el 74% de los casos. Registramos recaídas en el 63% de los casos y dos casos de muerte durante el tratamiento. Se estableció más frecuencia de enfermedad cardiovascular en los pacientes con PA. Conclusiones : Es el primer trabajo retrospectivo sobre EAA en nuestro medio. Nuestros hallazgos permiten determinar una frecuencia importante de PA en la consulta dermatológica y de DH, hecho no reportado en otros estudios con poblaciones similares.

ABSTRACT The autoimmune bullous diseases (ASD) comprise a heterogeneous group of pathologies with a low frequency of presentation that presents a chronic evolution, with remissions, exacerbations and an important morbidity and mortality even with early and adequate treatment. There are few epidemiological studies in our population that include the entire spectrum of bullous diseases. Objectives : To determine the clinical-epidemiological characteristics of AAS, describe treatments performed, control time of disease activity and time from treatment initiation to complete remission. Determine relapses of the studied pathologies, evaluating if there is a relationship between them and some recognized factor. Materials and methods : Retrospective and descriptive study. All patients with clinical, histopathological and immunofluorescence diagnosis of the AAS who had followed up for at least one year in the dermatology service of the HPUC, from January 2008 to November 2018 were included. Results: 53 were included. patients of which 50.9% had Bullous Pemphigoid (BP), 22.6% Dermatitis herpetiformis (DH) and 17% Pemphigus Vulgaris (PV). The age of the patients was between 21 and 90 years. Tense blistering forms and erosions were the most common forms of presentation. Systemic treatments were performed in 74% of the cases. We recorded relapses in 63% of the cases and two cases of death during the treatment. More frequency of cardiovascular disease was established in patients with BP. Conclusions : It is the first retrospective work on EAA in our environment. Our findings allow determining an important frequency of PA in the dermatological consultation and DH, fact this last, not reported in other studies with similar populations.

SARS-CoV-2 and Guillain-Barré syndrome: AIDP variant with a favourable outcome.

BACKGROUND AND PURPOSE: The spectrum of COVID-19, caused by severe acute respiratory syndrome coronavirus 2 infection (SARS-CoV-2), includes different neurologic manifestations of the central and peripheral nervous system. METHODS: From March through April 2020, in two university hospitals located in western Switzerland, we examined three patients with Guillain-Barré syndrome (GBS) following SARS-CoV-2. RESULTS: These cases were characterized by a primary demyelinating electrophysiological pattern (Acute inflammatory demyelinating polyneuropathy or AIDP) and a less severe disease course compared to recently published case series. Clinical improvement was observed in all patients at week five. One patient was discharged from hospital after full recovery with persistence of minor neurological signs (areflexia). Two of the three patients remained hospitalized: one was able to walk and the other could stand up with assistance. CONCLUSIONS: We report three cases of typical GBS (AIDP) occurring after SARS-CoV-2 infection and presenting with a favourable clinical course. Given the interval between COVID-19-related symptoms and neurological manifestations (mean of 15 days) we postulate a secondary immune-mediated mechanism rather than direct viral damage.

Nevo epidérmico verrugoso inflamatorio lineal extenso. Reporte de un caso / Extensive linear inflammatory wart epidermal nevus. Case Report

Resumen El nevo epidérmico verrugoso inflamatorio lineal (NEVIL), es una variante rara de nevo epidérmico que aparece con mayor frecuencia al nacer o en la primera infancia. Clínicamente, se presenta como placas eritematosas, queratósicas muy pruriginosas siguiendo las líneas de Blaschko. La anatomía patológica cumple un rol esencial para el diagnóstico diferencial y el tratamiento constituye un desafío médico. Presentamos el caso de un paciente masculino de 38 años de edad, con manifestación extensa de la patología y poca respuesta al tratamiento.

Abstract Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare variant of epidermal nevus that appears most frequently at birth or in early childhood. Clinically, it is presented as pruritic keratotic erythematous plaques, following the lines of Blaschko. Histopatology plays an essential role for differential diagnosis and treatment constitutes a medical challenge. We present the case of a 38-year-old man, with extensive presentation of the pathology and little response to topical therapy.

Evolución clínica y supervivencia de pacientes con melanomas de novo y melanomas asociados a nevo / Clinical evolution and survival of patients with de novo melanomas and nevus-associated melanomas

RESUMEN Introducción: El porcentaje de melanomas asociados histológicamente a nevos (MN(+)) varía del 20,6% al 53%. Distintos estudios demuestran que los MN(+) tienen igual o mejor pronóstico que aquellos melanomas que no se asocian a nevos, también llamados melanomas de novo (MN(-)). Objetivo: Comparar la evolución clínica y la supervivencia de MN(-) y MN(+) en el Hospital Privado Universitario de Córdoba, analizando el estudio de ganglio centinela, la supervivencia libre de enfermedad al año y a 5 años y la mortalidad específica por melanoma a 5 años. Materiales y Métodos :Se realizó un estudio retrospectivo, transversal. Se incluyeron pacientes con diagnóstico de melanoma cutáneo realizado por el Servicio de Anatomía Patológica del hospital o con revisión del taco de biopsia en el hospital, desde enero del año 2000 hasta diciembre del 2015. Resultados: De 554 casos de melanoma, se incluyeron 208 melanomas cutáneos, con un seguimiento promedio de 2,44 (0,66 - 5, 35) años. El 47,1% de melanomas fueron MN(+). Los MN(+) se relacionaron de manera estadísticamente significativa con el tipo extensivo superficial, localización en tronco posterior, regresión, menor Breslow y Clark 2. La supervivencia libre de enfermedad a 5 años fue mayor en MN(+) y la ausencia de asociación a nevo fue un factor de riesgo independiente. No se observaron diferencias en edad, sexo, diámetro tumoral, antecedentes de síndrome de nevo displásico, ulceración, mitosis, resultado del estudio de ganglio centinela, supervivencia libre de enfermedad al año, ni mortalidad por melanoma a 5 años.

ABSTRACT Introduction: The percentage of melanomas associated with nevus (NM(+)) ranges from 20,6% to 53%. Some studies suggested that MN+ have the same or even better prognosis than those melanomas that are not nevus-associated. The latter are also called melanomas de novo (MN(-)). Objective: To compare clinical evolution and survival of patients with MN(-) and MN(+) at the University Private Hospital of Cordoba (Argentina), by analyzing sentinel lymph node status, disease-free survival at 1 and 5 years and 5-year melanoma specific mortality. Methods: This is a retrospective, transversal study of patients who were diagnosed with cutaneous melanoma from 2000 until 2015. The biopsy specimen was analyzed or revised by the hospital´s Department of Pathology. Results: Of 554 melanoma cases, 208 cutaneous melanomas were included in the study. The mean follow up was 2,44 (0,66- 5,35) years. MN(+) represented the 47,1% of cases and were significantly correlated with superficial spreading subtype, posterior trunk localization, regression, lower Breslow and Clark 2. 5-year disease-free survival was longer in patients with MN(+) and the absence of associated nevus was an independent risk factor. There were no differences in age, gender, tumor diameter, history of atypical mole syndrome, ulceration, mitosis, sentinel lymph node status, 1-year disease-free survival or 5-year melanoma specific mortality.

Liquen plano linealblashkoide, reporte de 2 casos / Linear lichen planus along the lines of Blaschko, report of 2 cases

RESUMEN El liquen plano es una dermatosis inflamatoria autolimitada, de etiología desconocida, relativamente común. Existen múltiples variedades clínicas,que se basan en la disposición, morfología y localización de las lesiones, siendo el liquen plano lineal a lo largo de las líneas de Blaschko una presentación de distribución rara. Se presentan dos casos en pacientes de sexo masculino de 22 y 21 años de edad, con confirmación histológica y buena respuesta clínica al tratamiento tópico.

SUMMARY Lichen planus is a self-limited inflammatory dermatosis, relatively common, with a unknown cause. There are multiple clinical varieties, based on the disposition, morphology and location of the lesions, being the linear lichen planar along the lines of Blaschko a rare distribution presentation. We report two cases in male patients of 22 and 21 years of age, with histological confirmation and a good clinical response to topical treatment.

Urticaria vasculitis como manifestación de Lupus Eritematoso Sistémico / Urticarial Vasculitis as a manifestation of Systemic Lupus Erythematosus

Resumen La urticaria vasculitis (UV) es una vasculitis de pequeños vasos caracterizada clínicamente por lesiones urticarianas que muestran en la histología una vasculitis leucocitoclástica. Puede ser normo o hipocomplementémica, y mayormente es de etiología idiopática. Presentamos un caso de una paciente de 15 años con un cuadro de urticaria vasculitis en el contexto de un lupus eritematoso sistémico (LES).

Abstract Urticarial vasculitis (UV) is a vasculitis of small vessels clinically characterized by urticarial lesion that show a leukocytoclastic vasculitis in the histology. It may be normal or hypocomplementemic, mostly of idiopathic etiology. Here we present a case of a female patient 15 years old, who presents a clinical history of urticaria vasculitis in the context of a systemic lupus erythematosus.

Fulminant multifocal relapse in a fingolimod-treated multiple sclerosis patient.

BACKGROUND: Fingolimod is the first approved oral disease-modifying treatment for relapsing-remitting multiple sclerosis. Fingolimod targets lymphocytes, exerting a modulator effect on cell-surface sphingosine-1-phosphate receptors and thus blocking lymphocytes egression from secondary lymphoid organs. Recent reports describe fingolimod cessation being followed by severe or pseudo-tumoral relapse, but it usually does not happen on continuous long-term treatment. CASE PRESENTATION: Here we present the case of a patient on continuous long-term fingolimod treatment who presented with fulminant atypical multifocal relapse involving over 30 new and active lesions. CONCLUSION: This case is unique since this fulminant multifocal relapse occurred in a patient with grade 3 lymphopenia and irreproachable adherence. This observation should be known as a possible side effect of fingolimod treatment.

Metástasis cutánea de carcinoma de mama primario / Cutaneous metastasis of primary breast carcinoma

RESUMEN: Se consideran metástasis cutáneas aquellas neoplasias que se extienden a la piel por diseminación linfática, hemática o por contigüidad, originadas generalmente de un tumor extracutáneo, casi siempre diseminado y agresivo. Las metástasis en la piel suelen constituir un evento tardío en el curso de la enfermedad neoplásica, pero también pueden presentarse como el primer signo de un tumor desconocido.1 El carcinoma de mama es la neoplasia maligna interna más frecuente en las mujeres y se manifiesta con mayor frecuencia entre la quinta y sexta década de la vida. El 45% de las metástasis en la piel de cáncer de mama se presentan entre 6 meses y 4 años después del diagnóstico del tumor primario, aunque pueden observarse lesiones metastásicas al cabo de 10 años, por lo que el riesgo de metástasis quedaría latente durante el resto de la vida.2 Constituye un factor pronóstico desfavorable de la enfermedad asociándose a la morbi-mortalidad de la paciente, ya que generalmente se encuentran metástasis concomitantes en órganos internos.

SUMMARY: Cutaneous metastases are considered to be those neoplasms that extend to the skin due to lymphatic and hematic dissemination, generally originating from an extra cutaneous tumor, almost always disseminated and aggressive. Metastases in the skin are usually a late event in the course of neoplastic disease, but they can also present as the first sign of an unknown tumor.1 Breast carcinoma is the most frequent internal malignant neoplasm in women, manifesting most frequently between the fifth and sixth decade of life. 45% of skin metastases of breast cancer occur between 6 months and 4 years after diagnosis of the primary tumor, although metastatic lesions can be observed after 10 years, so the risk of metastasis would remain latent during the rest of life.2 It is a prognostic factor of the disease associated with the morbidity and mortality of the patient, since concomitant metastases are usually found in distant organs.

Sarcoma de kaposi en pacientes vih: a proposito de dos casos / Kaposi´s sarcoma in hiv patients: regarding two case report

RESUMEN: El Sarcoma de Kaposi es unaneoplasia de origen vascular,asociado obligadamente al virus Herpes Humano tipo 8. Presenta manifestaciones cutáneas en primer lugar, mucosas, ganglionares y viscerales. Puede evolucionar de forma leve con lesiones cutáneas, hasta casos fulminantes con compromiso sistémico, en caso de no realizar tratamiento. Existen múltiples opciones terapéuticas, las cuales se definen según el compromiso de la enfermedad y la afectación del paciente por su patología de base.El diagnóstico se basa en la sospecha clínica y se confirma con una biopsia histopatológica. El sarcoma de Kaposi corresponde a la neoplasia más frecuente en los pacientes con VIHse encuentraprincipalmente en hombres homosexuales. En este artículo se presentan dos pacientes con VIH con diagnóstico de Sarcoma de Kaposi y se realiza una breve revisión de la bibliografía.

ABSTRACT: Kaposi´s sarcoma is a vascular origin neoplasm, obligatory associated with Human Herpes Virus 8. It presents cutaneous manifestations at a first place, mucous, ganglionic and viscerals. It may present a mild presentation with cutaneous manifestations, up to fulminant cases with systemicinvolvement,in case of not being treated. There are a number of therapeutic options, defined by the state of the illness and the involvement of the patient due to the primary pathology. The diagnosis is based on the clinical suspicion and it is confirmed by a histopathological biopsy. Kaposi´s sarcomais the most frequent neoplasm in patients with HIV, being more frequently in homosexual men. In this article two HIV patients with Kaposi´s sarcoma diagnosis are exposed and a brief revision of the literature is done.

Enfermedad de fabry / Fabry disease

RESUMEN La enfermedad de Fabry (EF) es un trastorno de almacenamiento lisosomal, hereditario, ligado al cromosoma X, causado por el déficit de la enzima alfa galactosidasa A. Presenta una gran variabilidad clínica por afección multisistémica de manera inespecífica. Por este motivo, el diagnóstico clínico de la enfermedad es difícil y suele retrasarse. Las manifestaciones cutáneas de esta patología, tienen un importante papel en el diagnóstico precoz de la enfermedad, ya que, permite la utilización de tratamiento de reemplazo enzimático oportuno, así como el consejo genético adecuado.

SUMMARY Fabry disease is a hereditary, lysosomal storage disorder linked to the X chromosome, caused by the deficiency of the enzyme alpha galactosidase A. It presents a great clinical variability due to multisystemic affection in an unspecific way. For this reason, the diagnosis of the disease is difficult and often delayed. The cutaneous manifestations of this pathology, have an important role in the early diagnosis of the disease, since it allows the use of opportune enzymatic replacement treatment, as well as the adequate genetic counseling.

Diagnostic tools to tackle infectious causes of encephalitis and meningoencephalitis in immunocompetent adults in Europe.

BACKGROUND: Encephalitis and meningoencephalitis imply inflammation of the brain parenchyma, and comprise many diagnostic entities, such as various infections and causes of dysimmunity. The cause remains unknown in around 50% of cases. OBJECTIVES: To summarize the main infectious causes of encephalitis and meningoencephalitis acquired in Europe, and the diagnostic means to identify them. SOURCES: PubMed, ECDC and WHO websites, personal experience. CONTENT: The principal infectious causes of encephalitis and meningoencephalitis acquired in Europe in adults are discussed in this review, with special emphasis on the microbiological and imaging diagnostic approaches. The role of electroencephalography in diagnosing encephalitis is also mentioned. Among infections, viruses are more frequent than other pathogen types, and their epidemiology varies according to geographic area. A few bacteria, such as Listeria monocytogenes and Mycobacterium tuberculosis, are also to be considered. In contrast, parasites and fungi are rare encephalitis causes in Europe. IMPLICATIONS: Identifying the causative pathogen of infectious encephalitis and meningoencephalitis is complex because of the variety of pathogens, the epidemiology of which is determined by geography and environmental factors. Furthermore, despite extensive microbiological testing, many cases of encephalitis remain of unknown origin. Brain magnetic resonance imaging and electroencephalography are useful complementary diagnostic tools, and newer unbiased sequencing technologies might help to fill in the diagnostic gap.

Diagnostic tools for immune causes of encephalitis.

BACKGROUND: Autoimmune encephalitis (AE) refers to a central nervous system (CNS) antibody-mediated entity characterized by a rapid onset behavioural and cognitive decline that can be associated with movement disorders, epileptic and dysautonomic features. Interestingly, it is thought to be as common as its infectious disease counterpart and can share some clinical, radiological, and laboratory findings. OBJECTIVES: The aim is to describe the main clinical features of AE caused by antibodies targeting cell-surface neuronal agents and the diagnostic means to identify them. Paraneoplastic syndromes, associated with intracellular antibodies, will not be tackled in this review. SOURCES: PubMed/MEDLINE were the sources. CONTENT: According to a recent population-based study, autoimmunity is one of the most frequent cause of encephalitis after infectious agents. Its diagnosis lies upon 'classic' clinical features, which are dominated by neuropsychiatric symptoms and epileptic seizures. Cerebral spinal fluid (CSF) and serum autoantibody testing can confirm AE. Complementary examination with magnetic resonance imaging (MRI) and electroencephalogram (EEG) may be helpful for excluding other causes and managing seizures. In addition, exclusion of infectious and other origins must be considered. IMPLICATIONS: AE misdiagnosis can lead to a delay in treatment onset and, thus, clinical worsening. In this sense, identifying the causative agent is of utmost importance. However, the absence of CSF or serum antibody detection does not exclude the diagnosis of AE. Despite extensive testing, many encephalitis cases remain of unknown origin. It is obvious that some autoantibodies have not yet been identified in AE. Since radiological and biological examinations are not always contributive, early symptom recognition might help to hasten the diagnostic process.