Asociación entre penfigoide ampolloso e inhibidores de la dipeptidilpeptidasa-4: estudio de cohortes retrospectivo / Association Between Bullous Pemphigoid and Dipeptidyl Peptidase 4 Inhibitors: A Retrospective Cohort Study

ANTECEDENTES: La asociación entre los inhibidores de la dipeptidil peptidasa 4 (iDPP-4) y el penfigoide ampolloso (PA) se ha demostrado en varios estudios. El objetivo principal de este estudio era estimar el uso del tratamiento con iDPP-4i en pacientes diagnosticados de PA en nuestro entorno. MATERIAL Y MÉTODOS: Seleccionamos pacientes diagnosticados histológicamente de PA en nuestro departamento entre octubre de 2015 y octubre de 2018. Realizamos una revisión retrospectiva para evaluar los datos clínicos-epidemiológicos y los patrones de inmunofluorescencia directa (IFD). RESULTADOS: De los 70 pacientes diagnosticados con PA durante el período de estudio, el 50% eran diabéticos y el 88,57% de ellos estaban siendo tratados con un iDPP-4 en el momento del diagnóstico de PA. El iDPP-4 más frecuente era la linagliptina (utilizada en el 18,6% de los pacientes), seguida de la vildagliptina (el 17,1%). La mediana de tiempo de latencia entre el inicio del tratamiento con iDPP-4 y el diagnóstico de PA fue de 27,5 meses, siendo de 16 meses para la linagliptina y 39 meses para la vildagliptina (log Rank < 0,01). La IFD fue negativaUn resultado negativo de DIF fue significativamente más común en pacientes que no fueron tratados con un DPP-4i. El patrón DIF más fuertemente (y significativamente) asociado con el tratamiento con DPP-4i fueron los depósitos lineales de inmunoglobulina G a lo largo de la unión dermoepidérmica. El tratamiento con DPP-4i se retiró en el 87% de los pacientes y el 96% de ellos logró una respuesta completa. CONCLUSIÓN: El tratamiento con DPP-4i es muy común en pacientes con BP en nuestro entorno. El período de latencia entre el inicio del tratamiento y el inicio de la PA parece ser más corto con linagliptina que con otros tipos de gliptinas. Los pacientes que reciben tratamiento con DPP-4i pueden mostrar patrones DIF diferentes a los que no reciben tratamiento

BACKGROUND: The association between dipeptidyl peptidase 4 inhibitors (DPP-4i) and bullous pemphigoid (BP) has been demonstrated in several studies. The main aim of this study was to estimate the use of DPP-4i treatment in patients diagnosed with BP in our setting. METHODS: We selected patients histologically diagnosed with BP in our department between October 2015 and October 2018 and performed a retrospective chart review to assess clinical and epidemiological data and direct immunofluorescence (DIF) patterns. RESULTS: Of the 70 patients diagnosed with BP during the study period, 50% were diabetic and 88.57% of these were being treated with a DPP-4i when diagnosed with BP. The most common DPP-4i was linagliptin (used in 18.6% of patients), followed by vildagliptin (17.1%). The median latency period between initiation of DPP-4i treatment and diagnosis of BP was 27.5 months for all treatments, 16 months for linagliptin, and 39 months for vildagliptin (log rank < 0.01). A negative DIF result was significantly more common in patients not being treated with a DPP-4i. The DIF pattern most strongly (and significantly) associated with DPP-4i treatment was linear immunoglobulin G deposits along the dermal-epidermal junction. DPP-4i treatment was withdrawn in 87% of patients and 96% of these achieved a complete response. CONCLUSIONS: DPP-4i treatment is very common in patients with BP in our setting. The latency period between start of treatment and onset of BP seems to be shorter with linagliptin than with other types of gliptins. Patients receiving DPP-4i treatment may show different DIF patterns to those not receiving treatment

Association Between Bullous Pemphigoid and Dipeptidyl Peptidase 4 Inhibitors: A Retrospective Cohort Study. / Asociación entre penfigoide ampolloso e inhibidores de la dipeptidilpeptidasa-4: estudio de cohortes retrospectivo.

BACKGROUND: The association between dipeptidyl peptidase 4 inhibitors (DPP-4i) and bullous pemphigoid (BP) has been demonstrated in several studies. The main aim of this study was to estimate the use of DPP-4i treatment in patients diagnosed with BP in our setting. METHODS: We selected patients histologically diagnosed with BP in our department between October 2015 and October 2018 and performed a retrospective chart review to assess clinical and epidemiological data and direct immunofluorescence (DIF) patterns. RESULTS: Of the 70 patients diagnosed with BP during the study period, 50% were diabetic and 88.57% of these were being treated with a DPP-4i when diagnosed with BP. The most common DPP-4i was linagliptin (used in 18.6% of patients), followed by vildagliptin (17.1%). The median latency period between initiation of DPP-4i treatment and diagnosis of BP was 27.5 months for all treatments, 16 months for linagliptin, and 39 months for vildagliptin (log rank < 0.01). A negative DIF result was significantly more common in patients not being treated with a DPP-4i. The DIF pattern most strongly (and significantly) associated with DPP-4i treatment was linear immunoglobulin G deposits along the dermal-epidermal junction. DPP-4i treatment was withdrawn in 87% of patients and 96% of these achieved a complete response. CONCLUSIONS: DPP-4i treatment is very common in patients with BP in our setting. The latency period between start of treatment and onset of BP seems to be shorter with linagliptin than with other types of gliptins. Patients receiving DPP-4i treatment may show different DIF patterns to those not receiving treatment.

Papel de la resonancia magnética en niños con hemangiomas lumbosacros y perineales: informe de 3 casos clínicos y revisión de la literatura / Role of Magnetic Resonance Image in Children with Lumbosacral and Perineal Hemangiomas: Case Reports and Review of the Literature

Los hemangiomas infantiles son los tumores benignos más frecuentes en la población pediátrica. Cuando afectan al área lumbar y perineal, algunos casos pueden asociarse a alguna malformación subyacente como una disrafia espinal oculta. El manejo de estos hemangiomas carece de consenso. Describimos 3 casos de niños con hemangiomas lumbosacros y perineales con anomalías en la resonancia magnética y revisamos la literatura para valorar qué pruebas y en qué momento se deben realizar para completar el estudio en estos pacientes. Por lo general, se solicita una ecografía lo más precozmente posible, ya que esta técnica no es posible realizarla una vez que los elementos espinales posteriores se han osificado, lo que generalmente ocurre a los 6 meses de edad. La resonancia magnética es la prueba de referencia para diagnosticar una disrafia espinal oculta. De acuerdo con la literatura, la edad media para este examen debe ser alrededor de los 6 meses, cuando la formación de grasa en el filum terminale se ha visto incrementada. En nuestra opinión, se debería realizar una resonancia magnética a los 6 meses de edad en todos los niños con hemangioma lumbar o perineal, independientemente del tamaño de la lesión, la ausencia de síntomas neurológicos o los resultados de la ecografía

Cutaneous hemangiomas are the most frequent benign tumors in children. When they affect the lumbar and perineal area some cases can be associated with an occult spinal dysraphism. The management of these hemangiomas lack consensus. We report 3 cases of children with lumbosacral and perineal hemangiomas with magnetic resonance image abnormalities and we review the literature to find out the type and timing of tests that should be performed to complete the study in these patients. Ultrasound is typically requested as young as possible, as this imaging technique is not possible 11 the posterior spinal elements have ossified. MRI is the gold standard for diagnosing occult spinal dysraphism. According to the literature, the mean age for MRI screening should be around 6 months, when the fat formation in the filum terminale is expanded. In our opinion, an MRI scan should be performed at 6 months of age in every children with lumbar or perineal hemangioma regardless the lesion size, neurological symptoms or the ultrasound results

Aislamiento de Haemophilus spp. en exudados uretrales como posible agente etiológico de uretritis aguda: estudio de 38 casos / Haemophilus Species Isolated in Urethral Exudates as a Possible Causative Agent in Acute Urethritis: A Study of 38 Cases

Introducción: La incidencia de uretritis por Haemophilus está aumentando. Nuestro objetivo principal es describir las características clínico-microbiológicas de estos pacientes. Como objetivo secundario discutiremos el tratamiento más adecuado en función de las resistencias antibióticas testadas. Material y métodos: Seleccionamos los pacientes de la Unidad de Infecciones de Transmisión Sexual diagnosticados microbiológicamente de uretritis entre julio de 2015 y julio de 2018. De ellos, seleccionamos aquellos en los que se aisló un Haemophilus mediante cultivo agar chocolate. Las resistencias antibióticas se testaron mediante método de difusión disco-placa. De estos pacientes se recogieron los datos de forma transversal y prospectiva durante las visitas en consultas externas. Resultados: Se aisló un Haemophilus spp. en 33,6% de los pacientes diagnosticados de uretritis. De estos pacientes, la manifestación clínica más frecuente fue la supuración uretral (57,9%) y el 60% eran hombres que tienen sexo con hombres, siendo el aislamiento de este microorganismo más frecuente de forma estadísticamente significativa entre los hombres que tienen sexo con hombres que el aislamiento de Neisseria o Chlamydia. Haemophilus spp. se encontró de forma aislada en el 39,5% de los pacientes, siendo el más frecuente H. parainfluenzae en el 84,2%. El 34,2% de los casos de Haemophilus aislados fueron resistentes a azitromicina y el 26,3% eran resistentes tanto a azitromicina como a tetraciclinas. En los casos en los que no se perdió el seguimiento del paciente (n = 17; 44,7%), el tratamiento administrado de forma empírica consiguió una remisión clínica y microbiológica en 11 pacientes, mientras que en 6 fue necesario administrar una pauta de un nuevo antibiótico. Conclusiones: Haemophilus es un nuevo agente etiológico de uretritis no gonocócicas cuya incidencia está en aumento, especialmente entre hombres que tienen sexo con hombres que practican sexo oral sin protección. Estos pacientes pueden presentar una clínica similar a una uretritis gonocócica. Es necesario confirmar la erradicación debido al elevado número de resistencias antibióticas testadas en Haemophilus spp

Introduction: The incidence of urethritis due to Haemophilus species is increasing. The main aim of this study was to describe the clinical and microbiological characteristics of patients with this form of urethritis. A secondary aim was to discuss the adequacy of treatments in patients with different types of antibiotic resistance. Material and methods: We studied patients with a microbiologically confirmed diagnosis of urethritis seen at the Sexually Transmitted Infections Unit of our hospital between July 2015 and July 2018. We selected all patients in whom Haemophilus species were isolated on chocolate agar. Antibiotic resistance was tested using the disk-diffusion method. Cross-sectional data were collected prospectively during outpatient visits. Results: Haemophilus species were isolated in 33.6% of cases. The most common clinical manifestation was urethral discharge (57.6%); 60% of the patients were men who have sex with men and in this subgroup Haemophilus species were significantly more common than either Neisseria or Chlamydia species. Haemophilus species were found in isolation in 39.5% of patients and the most common one was Haemophilus parainfluenzae (isolated in 84.2% of cases). In total, 34.2% of patients were resistant to azithromycin and 26.3% were resistant to both azithromycin and tetracycline. Empirical treatment achieved clinical and microbiologic cure in 11 of the patients who were not lost to follow-up (n = 17; 44.7%). The remaining 6 patients required treatment with a new antibiotic. Conclusions: Haemophilus species are a new cause of nongonococcal urethritis, whose incidence is rising, particularly in men who have sex with men who engage in unprotected oral sex. The clinical manifestations are similar to those seen in gonococcal urethritis. Eradication of infection must be confirmed due to the high rate of antibiotic resistance associated with Haemophilus species

Haemophilus Species Isolated in Urethral Exudates as a Possible Causative Agent in Acute Urethritis: A Study of 38 Cases. / Aislamiento de Haemophilus spp. en exudados uretrales como posible agente etiológico de uretritis aguda: estudio de 38 casos.

INTRODUCTION: The incidence of urethritis due to Haemophilus species is increasing. The main aim of this study was to describe the clinical and microbiological characteristics of patients with this form of urethritis. A secondary aim was to discuss the adequacy of treatments in patients with different types of antibiotic resistance. MATERIAL AND METHODS: We studied patients with a microbiologically confirmed diagnosis of urethritis seen at the Sexually Transmitted Infections Unit of our hospital between July 2015 and July 2018. We selected all patients in whom Haemophilus species were isolated on chocolate agar. Antibiotic resistance was tested using the disk-diffusion method. Cross-sectional data were collected prospectively during outpatient visits. RESULTS: Haemophilus species were isolated in 33.6% of cases. The most common clinical manifestation was urethral discharge (57.6%); 60% of the patients were men who have sex with men and in this subgroup Haemophilus species were significantly more common than either Neisseria or Chlamydia species. Haemophilus species were found in isolation in 39.5% of patients and the most common one was Haemophilus parainfluenzae (isolated in 84.2% of cases). In total, 34.2% of patients were resistant to azithromycin and 26.3% were resistant to both azithromycin and tetracycline. Empirical treatment achieved clinical and microbiologic cure in 11 of the patients who were not lost to follow-up (n=17; 44.7%). The remaining 6 patients required treatment with a new antibiotic. CONCLUSIONS: Haemophilus species are a new cause of nongonococcal urethritis, whose incidence is rising, particularly in men who have sex with men who engage in unprotected oral sex. The clinical manifestations are similar to those seen in gonococcal urethritis. Eradication of infection must be confirmed due to the high rate of antibiotic resistance associated with Haemophilus species.

Role of Magnetic Resonance Image in Children with Lumbosacral and Perineal Hemangiomas: Case Reports and Review of the Literature. / Papel de la resonancia magnética en niños con hemangiomas lumbosacros y perineales: informe de 3casos clínicos y revisión de la literatura.

Cutaneous hemangiomas are the most frequent benign tumors in children. When they affect the lumbar and perineal area some cases can be associated with an occult spinal dysraphism. The management of these hemangiomas lack consensus. We report 3 cases of children with lumbosacral and perineal hemangiomas with magnetic resonance image abnormalities and we review the literature to find out the type and timing of tests that should be performed to complete the study in these patients. Ultrasound is typically requested as young as possible, as this imaging technique is not possible 11the posterior spinal elements have ossified. MRI is the gold standard for diagnosing occult spinal dysraphism. According to the literature, the mean age for MRI screening should be around 6 months, when the fat formation in the filum terminale is expanded. In our opinion, an MRI scan should be performed at 6 months of age in every children with lumbar or perineal hemangioma regardless the lesion size, neurological symptoms or the ultrasound results.

Dermatitis alérgica de contacto pediátrica. Estudio clínico-epidemiológico en un hospital terciario / Pediatric Allergic Contact Dermatitis: Clinical and Epidemiological Study in a Tertiary Hospital

Introducción: Los estudios epidemiológicos sobre incidencia de dermatitis alérgica de contacto en población pediátrica son escasos. Algunos trabajos consideran que se trata de una entidad infradiagnosticada, y que en muchos casos no se sospecha clínicamente, no realizándose pruebas epicutáneas. No obstante se han comunicado tasas prevalencia de hasta el 20% de sensibilización a alérgenos en la población pediátrica, por lo que probablemente debería ser tenida en cuenta como una posibilidad diagnóstica en este grupo de edad. Material y método: Se ha realizado un análisis retrospectivo de la base de datos de alergia cutánea del servicio de Dermatología del Hospital General seleccionando los casos diagnosticados en niños de entre 0 y 16 años durante los últimos 15 años (año 2000 hasta 2015). El estudio incluye variables epidemiológicas (edad, sexo, antecedentes de atopia) y clínicas (localización de las lesiones, baterías de alérgenos empleadas, alérgenos positivos y su relevancia). Resultados: De los 4.593 pacientes estudiados con pruebas epicutáneas en los últimos 15 años, 265 (6%) correspondían a niños de 0-16 años. Ciento cuarenta y cuatro pacientes (54,3%) mostraron positividad para al menos uno de los alérgenos parcheados. Los alérgenos más frecuentemente identificados fueron en orden decreciente: tiomersal, cloruro de cobalto, colofonia, parafenilendiamina, dicromato potásico, mercurio y níquel. La sensibilización fue considerada de relevancia presente en 177 casos (61,3%). Conclusión: Más de la mitad de los niños estudiados mostraron sensibilización a uno o más alérgenos, con un porcentaje importante de sensibilizaciones relevantes. Todo niño con sospecha clínica de dermatitis alérgica de contacto debería ser remitido para realización de pruebas epicutáneas. Al no existir pruebas estandarizadas en este colectivo se requiere un alto nivel de sospecha clínica y un conocimiento de los alérgenos más frecuentemente implicados de cara a seleccionar los alérgenos a parchar (AU)

Background: Few epidemiological studies have investigated the incidence of allergic contact dermatitis in children. Underdiagnosis has been observed in some studies, with many cases in which the condition is not suspected clinically and patch tests are not performed. However, the prevalence of pediatric sensitization to allergens has been reported to be as high as 20%, and the diagnosis should therefore be contemplated as a possibility in this age group. Material and methods: We performed a retrospective analysis of the skin allergy database of the Dermatology Department of Consorcio Hospital General Universitario de Valencia. Children between 0 and 16 years of age diagnosed with allergic contact dermatitis in the previous 15 years (between 2000 and 2015) were included in the analysis. Epidemiological (age, sex, history of atopy) and clinical (site of the lesions, allergen series applied, positive reactions, and their relevance) variables were gathered. Results: Patch tests had been performed on 4,593 patients during the study period. Of these, 265 (6%) were children aged between 0 and 16 years. A positive reaction to at least one of the allergens tested was observed in 144 (54.3%) patients in that group. The allergens most frequently identified were the following (in decreasing order of frequency): thiomersal, cobalt chloride, colophony, paraphenylenediamine, potassium dichromate, mercury, and nickel. The sensitization was considered relevant in 177 (61.3%) cases. Conclusions: More than half of the children studied showed sensitization to 1 or more allergens, with a high percentage of relevant sensitizations. All children with a clinical suspicion of allergic contact dermatitis should be referred for patch testing. As no standardized test series have been developed for this age group, a high level of clinical suspicion and knowledge of the allergens most commonly involved are required when selecting the allergens to be tested (AU)

Enfermedad xantogranulomatosa orbitaria del adulto. Formas clínicas, evaluación y manejo / Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management

La enfermedad xantogranulomatosa orbitaria del adulto comprende un grupo heterogéneo de síndromes clínicos con diferentes grados de afectación sistémica y pronóstico variable. Todas las formas se manifiestan clínicamente como lesiones amarillentas infiltradas orbitarias de crecimiento progresivo. Histológicamente se caracteriza por un infiltrado inflamatorio compuesto fundamentalmente por histiocitos espumosos y células gigantes multinucleadas tipo Touton. Estos histiocitos xantomatizados son CD68+, S100- y CD1a-. Existen 4 formas clínicas de enfermedad xantogranulomatosa orbitaria del adulto: el xantogranuloma orbitario del adulto, el asma del adulto asociado a xantogranulomas orbitarios, el xantogranuloma necrobiótico y la enfermedad de Erdheim-Chester. El tratamiento de las lesiones locales se basa fundamentalmente en corticosteroides sistémicos y otros inmunosupresores. En los casos con enfermedad sistémica vemurafenib, tocilizumab y sirolimus ofrecen resultados prometedores (AU)

Adult xanthogranulomatous disease of the orbit refers to a heterogeneous group of clinical syndromes with differing degrees of systemic involvement and distinct prognoses. The different syndromes all present clinically with progressively enlarging, yellowish lesions of the orbit. Histologically, the lesions are characterized by an inflammatory infiltrate of foam cells and Touton-type multinucleated giant cells. The xanthomatized histiocytes are CD68+, S100-, and CD1a-. There are 4 clinical forms of xanthogranulomatous disease of the orbit: adult xanthogranulomatous disease of the orbit, adult onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, and Erdheim-Chester disease. The treatment of local lesions are treated with systemic corticosteroids and other immunosuppressors. Vemurafenib, tocilizumab, and sirolimus have shown promising results in systemic disease (AU)

Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management. / Enfermedad xantogranulomatosa orbitaria del adulto. Formas clínicas, evaluación y manejo.

Adult xanthogranulomatous disease of the orbit refers to a heterogeneous group of clinical syndromes with differing degrees of systemic involvement and distinct prognoses. The different syndromes all present clinically with progressively enlarging, yellowish lesions of the orbit. Histologically, the lesions are characterized by an inflammatory infiltrate of foam cells and Touton-type multinucleated giant cells. The xanthomatized histiocytes are CD68+, S100-, and CD1a-. There are 4 clinical forms of xanthogranulomatous disease of the orbit: adult xanthogranulomatous disease of the orbit, adult onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, and Erdheim-Chester disease. The treatment of local lesions are treated with systemic corticosteroids and other immunosuppressors. Vemurafenib, tocilizumab, and sirolimus have shown promising results in systemic disease.

Pediatric Allergic Contact Dermatitis: Clinical and Epidemiological Study in a Tertiary Hospital. / Dermatitis alérgica de contacto pediátrica. Estudio clínico-epidemiológico en un hospital terciario.

BACKGROUND: Few epidemiological studies have investigated the incidence of allergic contact dermatitis in children. Underdiagnosis has been observed in some studies, with many cases in which the condition is not suspected clinically and patch tests are not performed. However, the prevalence of pediatric sensitization to allergens has been reported to be as high as 20%, and the diagnosis should therefore be contemplated as a possibility in this age group. MATERIAL AND METHODS: We performed a retrospective analysis of the skin allergy database of the Dermatology Department of Consorcio Hospital General Universitario de Valencia. Children between 0 and 16 years of age diagnosed with allergic contact dermatitis in the previous 15 years (between 2000 and 2015) were included in the analysis. Epidemiological (age, sex, history of atopy) and clinical (site of the lesions, allergen series applied, positive reactions, and their relevance) variables were gathered. RESULTS: Patch tests had been performed on 4,593 patients during the study period. Of these, 265 (6%) were children aged between 0 and 16 years. A positive reaction to at least one of the allergens tested was observed in 144 (54.3%) patients in that group. The allergens most frequently identified were the following (in decreasing order of frequency): thiomersal, cobalt chloride, colophony, paraphenylenediamine, potassium dichromate, mercury, and nickel. The sensitization was considered relevant in 177 (61.3%) cases. CONCLUSIONS: More than half of the children studied showed sensitization to 1 or more allergens, with a high percentage of relevant sensitizations. All children with a clinical suspicion of allergic contact dermatitis should be referred for patch testing. As no standardized test series have been developed for this age group, a high level of clinical suspicion and knowledge of the allergens most commonly involved are required when selecting the allergens to be tested.