Carcinoma basocelular cutáneo: diagnóstico y tratamiento en atención especializada dermatológica. Guía de Práctica Clínica de la AEDV / Diagnosis and Treatment of Basal Cell Carcinoma in Specialized Dermatology Units: A Clinical Practice Guideline

ANTECEDENTES Y OBJETIVO: El carcinoma basocelular supone el cáncer de piel más frecuente en la población. Hay una gran variabilidad en su manejo y las diferentes guías extranjeras que existen son difícilmente aplicables en nuestro medio. El objetivo de la presente guía es servir de referencia a los dermatólogos españoles para mejorar el manejo de este tumor basándose en la evidencia actual. MATERIALES Y MÉTODOS: Se escogió a miembros del Grupo Español de Dermato-Oncología y Cirugía (GEDOC) con experiencia en el tratamiento de estos tumores y con interés en participar en la elaboración de la guía. Se hizo una adaptación de las guías de práctica clínica existentes mediante el método ADAPTE, se resumió el proceso de atención, y se elaboraron las preguntas clínicas relevantes. Se seleccionaron las guías mejor puntuadas mediante el instrumento AGREE II, realizando la búsqueda de las respuestas en dichas guías y elaborando posteriormente las recomendaciones. Finalmente se sometió la guía a revisión externa. RESULTADOS: Las guías con mejor puntuación fueron las de la British Association of Dermatologits (BAD), del National Comprehensive Cancer Network (NCCN), del European Dermatology Forum (EDF) y de la European Academy of Dermatology and Venereology (EADV). Se obtuvieron en total 11 preguntas clínicas, contestadas a partir de estas guías. CONCLUSIONES: Esta guía responde a preguntas habituales sobre el manejo del carcinoma basocelular en la práctica clínica diaria y sirve a los dermatólogos como referencia en la toma de decisiones, siempre teniendo en cuenta los recursos y las preferencias del paciente

BACKGROUND AND OBJECTIVE: Basal cell carcinoma (BCC) is the most common skin cancer in the general population. BCC is managed in a variety of ways, and available international guidelines are difficult to put into practice in Spain. This guideline aims to improve the management of BCC based on current evidence and provide a point of reference for Spanish dermatologists. MATERIAL AND METHODS: Members of the Spanish Oncologic Dermatology and Surgery Group (GEDOC) with experience treating BCC were invited to participate in drafting this guideline. The drafters used the ADAPTE collaboration process to develop the new guideline based on existing ones, first summarizing the care pathway and posing relevant clinical questions. They then searched for guidelines, assessed them with the AGREEII (Appraisal of Guidelines for Research and Evaluation) tool, and searched the selected guidelines for answers to the clinical questions. Finally, the recommendations were drafted and submitted for external review. RESULTS: The highest-scoring guidelines were from the Association of Dermatologists, the National Comprehensive Cancer Network, the European Dermatology Forum, and the European Academy of Dermatology and Venereology. A total of 11 clinical questions were answered. CONCLUSIONS: This new guideline answers the working group's clinical questions about the routine management of BCC in Spain. It provides dermatologists with a tool they can use for decision-making while taking into consideration the resources available and patient preferences

Diagnosis and Treatment of Basal Cell Carcinoma in Specialized Dermatology Units: A Clinical Practice Guideline. / Carcinoma basocelular cutáneo: diagnóstico y tratamiento en atención especializada dermatológica. Guía de Práctica Clínica de la AEDV.

BACKGROUND AND OBJECTIVE: Basal cell carcinoma (BCC) is the most common skin cancer in the general population. BCC is managed in a variety of ways, and available international guidelines are difficult to put into practice in Spain. This guideline aims to improve the management of BCC based on current evidence and provide a point of reference for Spanish dermatologists. MATERIAL AND METHODS: Members of the Spanish Oncologic Dermatology and Surgery Group (GEDOC) with experience treating BCC were invited to participate in drafting this guideline. The drafters used the ADAPTE collaboration process to develop the new guideline based on existing ones, first summarizing the care pathway and posing relevant clinical questions. They then searched for guidelines, assessed them with the AGREEII (Appraisal of Guidelines for Research and Evaluation) tool, and searched the selected guidelines for answers to the clinical questions. Finally, the recommendations were drafted and submitted for external review. RESULTS: The highest-scoring guidelines were from the Association of Dermatologists, the National Comprehensive Cancer Network, the European Dermatology Forum, and the European Academy of Dermatology and Venereology. A total of 11 clinical questions were answered. CONCLUSIONS: This new guideline answers the working group's clinical questions about the routine management of BCC in Spain. It provides dermatologists with a tool they can use for decision-making while taking into consideration the resources available and patient preferences.

Descripción de pacientes no aptos para ser sometidos a cirugía de Mohs tras valoración prequirúrgica: datos del registro español Regesmohs / Description of patients excluded for Mohs surgery after pre-surgical evaluation: data from the Regesmohs Spanish registry

ANTECEDENTES: El registro Regesmohs es un registro de ámbito nacional, de pacientes evaluados y sometidos a una cirugía de Mohs, en 17 centros españoles, desde julio de 2013. Como la cirugía de Mohs es el tratamiento que mejores resultados da para el manejo del carcinoma de células basales (CCB) de alto riesgo y otros tumores de la piel, queríamos describir los motivos por los que algunos pacientes fueron considerados no aptos para ser sometidos a este tratamiento y qué tratamientos alternativos recibieron. Estos datos pueden ser útiles para evitar excluir a pacientes aptos para ser sometidos a una cirugía de Mohs, para calcular la demanda que estos pacientes generan a nivel sanitario, así como la demanda que hay de tratamientos de inhibidores de la vía de Hedgehog en dicho grupo de pacientes. OBJETIVO: Describir a aquellos pacientes que fueron considerados no aptos para ser sometidos a una cirugía de Mohs tras valoración prequirúrgica y los tratamientos que recibieron. MÉTODOS: Regesmohs incluye a todos los pacientes consecutivos para ser sometidos a una cirugía de Mohs en los centros participantes, recogiendo datos sobre las características de los pacientes, las intervenciones y los resultados a corto y largo plazo. Se hizo una descripción de los pacientes considerados no aptos para ser sometidos a una cirugía de Mohs tras valoración prequirúrgica. RESULTADOS: Tres mil once pacientes fueron incluidos en el registro Regesmohs entre julio de 2013 y octubre de 2016. En 85 pacientes no se realizó cirugía de Mohs porque se consideraron candidatos inadecuados. Sesenta y siete pacientes presentaban CCB. Las razones para ser considerado paciente no apto fueron: contraindicaciones médicas (27,1%, n = 23), tumores de bajo riesgo (18,8%, n = 16) y tumores gigantes e invasión ósea (15,3%, n = 13). Solo un paciente (1,2%) reveló compromiso de ganglios linfáticos y ningún paciente metástasis visceral. De los 85 pacientes considerados no aptos 29 (34,1%) fueron sometidos a cirugía convencional, 24 (28,3%) a radioterapia, 4 (4,7%) a inhibidores de la vía de Hedgehog (solo indicado para el CCB) y 2 (2,4%) a tratamiento paliativo. No hubo datos de seguimiento de 14 pacientes (16,5%). CONCLUSIÓN: Las comorbilidades médicas fueron la razón más habitual para retener la cirugía de Mohs. Retener un tratamiento en función de una propagación a lugares distantes no es algo habitual. La mayoría de los pacientes considerados no aptos recibieron tratamientos más sencillos: cirugía convencional o radioterapia, siendo los inhibidores de la vía de Hedgehog una opción novedosa

BACKGROUND: Regesmohs registry is a nationwide registry including patients evaluated for Mohs surgery in 17 Spanish centres since July 2013. Given that Mohs surgery is the therapy with best results for high risk basal cell carcinoma (BCC) and other skin tumours, we wanted to describe the reasons that lead to some patients being excluded from this therapy and the alternative treatments that they received. These data may be useful to avoid excluding patients for Mohs surgery use, to estimate the healthcare demand of these patients and the demand for Hedgehog inhibitors therapy in this group. OBJECTIVE: To describe patients excluded for Mohs surgery after pre-surgical assessment, and the treatments that they received. METHODS: Regesmohs includes all consecutive patients assessed for Mohs surgery in the participating centres, collecting data on patient characteristics, intervention, and short and long-term results. Patients excluded for Mohs surgery after pre-surgical evaluation were described. RESULTS: 3011 patients were included in Regesmohs from July 2013 to October 2016. In 85, Mohs surgery was not performed as they were considered inadequate candidates. 67 had BCC. Reasons for exclusion were: medical contraindication (27.1%, n = 23) low-risk tumour in (18.8%, n = 16) and giant tumour and bone invasion (15.3%, n = 13). Only 1 patient (1.2%) showed lymph node involvement and no patients had visceral metastases. Of the 85 excluded patients, 29 (34.1%) were treated with conventional surgery, 24 (28.3%) with radiotherapy, 4 (4.7%) with inhibitors of the Hedgehog pathway (only indicated for BCC), and 2 (2.4%) received palliative care. We had no follow-up data on 14 patients (16.5%). CONCLUSION: Medical comorbidities were the most common reason for withholding Mohs surgery. Withholding therapy on the basis of distant extension is uncommon. Most excluded patients received simpler therapies: conventional surgery or radiotherapy, with hedgehog inhibitors being a new option

Description of patients excluded for Mohs surgery after pre-surgical evaluation: data from the Regesmohs Spanish registry. / Descripción de pacientes no aptos para ser sometidos a cirugía de Mohs tras valoración prequirúrgica: datos del registro español Regesmohs.

BACKGROUND: Regesmohs registry is a nationwide registry including patients evaluated for Mohs surgery in 17 Spanish centres since July 2013. Given that Mohs surgery is the therapy with best results for high risk basal cell carcinoma (BCC) and other skin tumours, we wanted to describe the reasons that lead to some patients being excluded from this therapy and the alternative treatments that they received. These data may be useful to avoid excluding patients for Mohs surgery use, to estimate the healthcare demand of these patients and the demand for Hedgehog inhibitors therapy in this group. OBJECTIVE: To describe patients excluded for Mohs surgery after pre-surgical assessment, and the treatments that they received. METHODS: Regesmohs includes all consecutive patients assessed for Mohs surgery in the participating centres, collecting data on patient characteristics, intervention, and short and long-term results. Patients excluded for Mohs surgery after pre-surgical evaluation were described. RESULTS: 3011 patients were included in Regesmohs from July 2013 to October 2016. In 85, Mohs surgery was not performed as they were considered inadequate candidates. 67 had BCC. Reasons for exclusion were: medical contraindication (27.1%, n=23) low-risk tumour in (18.8%, n=16) and giant tumour and bone invasion (15.3%, n=13). Only 1 patient (1.2%) showed lymph node involvement and no patients had visceral metastases. Of the 85 excluded patients, 29 (34.1%) were treated with conventional surgery, 24 (28.3%) with radiotherapy, 4 (4.7%) with inhibitors of the Hedgehog pathway (only indicated for BCC), and 2 (2.4%) received palliative care. We had no follow-up data on 14 patients (16.5%). CONCLUSION: Medical comorbidities were the most common reason for withholding Mohs surgery. Withholding therapy on the basis of distant extension is uncommon. Most excluded patients received simpler therapies: conventional surgery or radiotherapy, with hedgehog inhibitors being a new option.

Descripción de las intervenciones quirúrgicas recogidas en el registro español de cirugía de Mohs (REGESMOHS) (2013-2015) / Characterization of Surgical Procedures in the Spanish Mohs Surgery Registry (REGESMOHS) for 2013-2015

Introducción: El Registro Español de Cirugía de Mohs recoge los datos de aplicación y resultados de esta técnica en España. Se describen los datos de las intervenciones realizadas desde el inicio del Registro en julio de 2013 a enero de 2016. Se analizan los datos de las cirugías tanto perioperatorios como intraoperatorios. Material y métodos: Estudio de cohortes prospectivo en el que participan 18 centros. Se recogen los datos de las intervenciones quirúrgicas como tipo de anestesia, técnica quirúrgica, ingreso hospitalario, número de estadios, manejo de factores de riesgo preoperatorios, tratamientos complementarios, tratamientos previos, tipo de tumor, tiempo empleado en la cirugía y complicaciones. Resultados: Se analizan 1.796 intervenciones quirúrgicas. El tumor intervenido con más frecuencia es el carcinoma basocelular (85,96%), seguido del carcinoma epidermoide (6,18%), lentigo maligno (2,81%) y dermatofibrosarcoma protuberans (1,97%). El 66,9% de los tumores eran primarios, el 19,2% recurrentes y el 13,9% persistentes. El tratamiento previo más frecuente fue quirúrgico. La cirugía de Mohs se realizó con más frecuencia bajo anestesia local (86,7%) y de forma ambulatoria (71,8%). En el 89,5% de los casos se utilizó la técnica de Mohs en congelación. El número de etapas requerido para alcanzar márgenes libres de tumor fue una en 56,45% de los pacientes, 2 en 32,1%, 3 en 7,1%, 4 en 2,7% y 5 o más en 1,8%. El propio dermatólogo reconstruyó el defecto en el 98% de los pacientes y la técnica reconstructiva más utilizada fue el colgajo (47,2%). Solo el 1,62% de los pacientes presentó alguna complicación intraoperatoria y la mediana de la duración de la cirugía fue 75 (p25:60-p75:100). Conclusión: Las características de los pacientes y tumores tratados son similares a las descritas en estudios de las mismas características en otras áreas geográficas. Existe un porcentaje mayor de lentigo maligno y dermatofibrosarcoma protuberans. La reconstrucción la realiza el dermatólogo con más frecuencia que en otras series. El tiempo de utilización de quirófano no es mucho mayor que para otras técnicas y la tasa de complicaciones intraoperatorias es muy reducida (AU)

Introduction: The Spanish Mohs Surgery Registry is used to collect data on the use and outcomes of Mohs micrographic surgery (MMS) in Spain. The aim of this study was to describe perioperative and intraoperative data recorded for MMS procedures performed between July 2013 (when the registry started) and January 2016. Material and methods: Prospective cohort study of data from 18 hospitals. The data collected included type of anesthesia, surgical technique, hospital admission, number of Mohs stages, management of preoperative risk factors, additional treatments, previous treatments, type of tumor, operating time, and complications. Results: Data were available for 1796 operations. The most common tumor treated by MMS was basal cell carcinoma (85.96%), followed by squamous cell carcinoma (6.18%), lentigo maligna (2.81%), and dermatofibrosarcoma protuberans (1.97%). Primary tumors accounted for 66.9% of all tumors operated on; 19.2% of tumors were recurrent and 13.9% were persistent. The most common previous treatment was surgical. MMS was mostly performed under local anesthesia (86.7% of cases) and as an outpatient procedure (71.8%). The frozen section technique was used in 89.5% of cases. One stage was needed to achieve tumor-free margins in 56.45% of patients; 2 stages were required in 32.1% of patients, 3 in 7.1%%, 4 in 2.7%, and 5 or more in 1.8%. The defect was reconstructed by the dermatologist in 98% of patients and the most common technique was flap closure (47.2%). Intraoperative complications were recorded for just 1.62% of patients and the median (interquartile range) duration of surgery was 75 (60-100) minutes. Conclusion: The characteristics of the patients and tumors treated by MMS are similar to those reported for similar studies in other geographic areas. Lentigo maligna and dermatofibrosarcoma protuberans accounted for a higher proportion of cases in our series, and repair of the surgical defect by a dermatologist was also more common. Operating times in MMS are not much longer than those reported for other procedures and the rate of intraoperative complications is very low (AU)

Characterization of Surgical Procedures in the Spanish Mohs Surgery Registry (REGESMOHS) for 2013-2015. / Descripción de las intervenciones quirúrgicas recogidas en el registro español de cirugía de Mohs (REGESMOHS) (2013-2015).

INTRODUCTION: The Spanish Mohs Surgery Registry is used to collect data on the use and outcomes of Mohs micrographic surgery (MMS) in Spain. The aim of this study was to describe perioperative and intraoperative data recorded for MMS procedures performed between July 2013 (when the registry started) and January 2016. MATERIAL AND METHODS: Prospective cohort study of data from 18 hospitals. The data collected included type of anesthesia, surgical technique, hospital admission, number of Mohs stages, management of preoperative risk factors, additional treatments, previous treatments, type of tumor, operating time, and complications. RESULTS: Data were available for 1796 operations. The most common tumor treated by MMS was basal cell carcinoma (85.96%), followed by squamous cell carcinoma (6.18%), lentigo maligna (2.81%), and dermatofibrosarcoma protuberans (1.97%). Primary tumors accounted for 66.9% of all tumors operated on; 19.2% of tumors were recurrent and 13.9% were persistent. The most common previous treatment was surgical. MMS was mostly performed under local anesthesia (86.7% of cases) and as an outpatient procedure (71.8%). The frozen section technique was used in 89.5% of cases. One stage was needed to achieve tumor-free margins in 56.45% of patients; 2 stages were required in 32.1% of patients, 3 in 7.1%%, 4 in 2.7%, and 5 or more in 1.8%. The defect was reconstructed by the dermatologist in 98% of patients and the most common technique was flap closure (47.2%). Intraoperative complications were recorded for just 1.62% of patients and the median (interquartile range) duration of surgery was 75 (60-100) minutes. CONCLUSION: The characteristics of the patients and tumors treated by MMS are similar to those reported for similar studies in other geographic areas. Lentigo maligna and dermatofibrosarcoma protuberans accounted for a higher proportion of cases in our series, and repair of the surgical defect by a dermatologist was also more common. Operating times in MMS are not much longer than those reported for other procedures and the rate of intraoperative complications is very low.

Descripción de los pacientes intervenidos mediante cirugía de Mohs en España. Datos basales del registro español de cirugía de Mohs (REGESMOHS) / Description of Patients Undergoing Mohs Surgery in Spain: Initial Report on Data From the Spanish Registry of Mohs Surgery (REGESMOHS)

INTRODUCCIÓN: En julio de 2013 se inició la recogida de datos del registro español de cirugía micrográfica de Mohs, que describe la aplicación y los resultados de esta técnica en España. En este artículo se describen las características del paciente y de los tumores tratados. MATERIAL Y MÉTODOS: Se trata de un estudio de cohortes prospectivo en el que participan centros en los que se practica al menos una intervención semanal de cirugía micrográfica de Mohs. En cada centro se incluyen todos los pacientes que son valorados para realizar cirugía de Mohs, excepto los declarados judicialmente incapaces. En este artículo describimos las características de los pacientes y los tumores incluidos en la cohorte. RESULTADOS: El número de pacientes incluidos desde julio de 2013 hasta octubre de 2014 es de 655. La mayoría de los tumores cutáneos intervenidos correspondieron a carcinoma basocelular, siendo el infiltrante el subtipo histológico más frecuente. La mayoría de las cirugías se practicaron en tumores localizados en la cara y el cuero cabelludo, siendo la localización más frecuente la nariz. Casi el 40% de los tumores operados son recurrentes o persistentes, y el tamaño tumoral prequirúrgico es similar en nuestro medio al descrito en otros estudios australianos o europeos. Hasta el 45,5% de los pacientes había recibido algún tratamiento quirúrgico previo. CONCLUSIÓN: Los datos observados son similares a los de otras series publicadas, y son relevantes para poder valorar la aplicabilidad en nuestro contexto de estudios realizados en otros medios

INTRODUCTION: The Spanish registry of Mohs micrographic surgery started collecting data in July 2013. The aim of the registry is to report on the use of this technique in Spain and the outcomes achieved. In the present article, we describe the characteristics of patients and the tumors treated. MATERIAL AND METHODS: This is a prospective cohort study of patients treated with Mohs micrographic surgery. The participating centers are hospitals where at least one intervention of this type is performed each week. All patients considered for Mohs micrographic surgery in participating centers are included in the registry except those who have been declared legally incompetent. RESULTS: Between July 2013 and October 2014, data from 655 patients were included in the registry. The most common tumor involved was basal cell carcinoma, and the most common histological subtype was infiltrative basal cell carcinoma. Most of the tumors treated were located on the face or scalp, and the most common site was the nose. Almost 40% of the tumors treated were recurrent or persistent, and preoperative tumor size was similar to that reported in other European studies and in Australia. In total, 45.5% of patients had received previous surgical treatment. CONCLUSION: The findings are similar to those reported in other studies, and the data collected are useful for assessing whether the results of studies carried out elsewhere are applicable in Spain

Description of Patients Undergoing Mohs Surgery in Spain: Initial Report on Data From the Spanish Registry of Mohs Surgery (REGESMOHS).

INTRODUCTION: The Spanish registry of Mohs micrographic surgery started collecting data in July 2013. The aim of the registry is to report on the use of this technique in Spain and the outcomes achieved. In the present article, we describe the characteristics of patients and the tumors treated. MATERIAL AND METHODS: This is a prospective cohort study of patients treated with Mohs micrographic surgery. The participating centers are hospitals where at least one intervention of this type is performed each week. All patients considered for Mohs micrographic surgery in participating centers are included in the registry except those who have been declared legally incompetent. RESULTS: Between July 2013 and October 2014, data from 655 patients were included in the registry. The most common tumor involved was basal cell carcinoma, and the most common histological subtype was infiltrative basal cell carcinoma. Most of the tumors treated were located on the face or scalp, and the most common site was the nose. Almost 40% of the tumors treated were recurrent or persistent, and preoperative tumor size was similar to that reported in other European studies and in Australia. In total, 45.5% of patients had received previous surgical treatment. CONCLUSION: The findings are similar to those reported in other studies, and the data collected are useful for assessing whether the results of studies carried out elsewhere are applicable in Spain.

Carcinoma sebáceo extraocular: presentación de 2 casos / Extraocular Sebaceous Carcinoma: A Report of 2 Cases

El carcinoma sebáceo es un tumor anexial raro y agresivo. Su etiología es desconocida. El 75% de estos tumores son de localización periocular y el 25% extraocular. Es uno de los marcadores cutáneos diagnósticos del síndrome de Muir-Torre, una enfermedad genética asociada a neoplasias viscerales. Por lo tanto, los pacientes con carcinoma sebáceo deben ser cuidadosamente valorados, realizándose una historia oncológica personal y familiar adecuada, una exploración física cuidadosa, pruebas complementarias necesarias y un seguimiento estrecho. Se presentan 2 casos de carcinomas sebáceos extraoculares y se realiza una revisión de la literatura, destacando la asociación entre el carcinoma sebáceo y el síndrome de Muir-Torre (AU)

Sebaceous carcinoma is a rare, aggressive adnexal tumor of unknown etiology; 75% of these tumors arise in the periocular region while 25% arise at extraocular sites. This cutaneous tumor is a diagnostic sign of Muir-Torre syndrome, a disorder associated with visceral malignancies and gene abnormalities. Patients with sebaceous carcinoma should therefore be carefully evaluated; a detailed personal and family history of cancer, a thorough physical examination, additional tests where appropriate, and close monitoring will all be required. We report 2 cases of extraocular sebaceous carcinoma and review the literature, focusing on the association between sebaceous carcinoma and Muir-Torre syndrome (AU)

Extraocular sebaceous carcinoma: a report of 2 cases.

Sebaceous carcinoma is a rare, aggressive adnexal tumor of unknown etiology; 75% of these tumors arise in the periocular region while 25% arise at extraocular sites. This cutaneous tumor is a diagnostic sign of Muir-Torre syndrome, a disorder associated with visceral malignancies and gene abnormalities. Patients with sebaceous carcinoma should therefore be carefully evaluated; a detailed personal and family history of cancer, a thorough physical examination, additional tests where appropriate, and close monitoring will all be required. We report 2 cases of extraocular sebaceous carcinoma and review the literature, focusing on the association between sebaceous carcinoma and Muir-Torre syndrome.

Dermatosis inflamatorias vulvares / Vulvar Inflammatory Dermatoses

La patología cutánea vulvar constituye un motivo frecuente de consulta en el momento actual. La vulva, al igual que el resto de la piel, puede verse afectada por múltiples enfermedades de diferentes etiologías, pero sus especiales condiciones anatómicas y fisiológicas hacen que tenga algunas peculiaridades que pueden suponer una dificultad añadida en su manejo. El estudio de la patología vulvar está emergiendo como una nueva área en la Dermatología. En este artículo, tras valorar la características de la vulva normal, sistematizaremos y realizaremos una breve revisión de las dermatosis inflamatorias vulvares, grupo heterogéneo de enfermedades en las que es clave un abordaje amplio y multidisciplinar (AU)

Vulvar skin disease is a common reason for consultation. The vulva, like the rest of the skin, can be affected by numerous diseases of various etiologies, but its particular anatomic and physiologic characteristics create additional diagnostic and therapeutic difficulties. The study of vulvar disease is emerging as a new branch of dermatology. In this article, we examine the characteristics of the normal vulva, and perform a brief, structured review of vulvar inflammatory dermatoses, which comprise a heterogeneous group of diseases in which a broad, multidisciplinary approach is essential (AU)

Extraocular Sebaceous Carcinoma: A Report of 2 Cases. / Carcinoma sebáceo extraocular: presentación de 2 casos.

Sebaceous carcinoma is a rare, aggressive adnexal tumor of unknown etiology; 75% of these tumors arise in the periocular region while 25% arise at extraocular sites. This cutaneous tumor is a diagnostic sign of Muir-Torre syndrome, a disorder associated with visceral malignancies and gene abnormalities. Patients with sebaceous carcinoma should therefore be carefully evaluated; a detailed personal and family history of cancer, a thorough physical examination, additional tests where appropriate, and close monitoring will all be required. We report 2 cases of extraocular sebaceous carcinoma and review the literature, focusing on the association between sebaceous carcinoma and Muir-Torre syndrome.

[Vulvar inflammatory dermatoses]. / Dermatosis inflamatorias vulvares.

Vulvar skin disease is a common reason for consultation. The vulva, like the rest of the skin, can be affected by numerous diseases of various etiologies, but its particular anatomic and physiologic characteristics create additional diagnostic and therapeutic difficulties. The study of vulvar disease is emerging as a new branch of dermatology. In this article, we examine the characteristics of the normal vulva, and perform a brief, structured review of vulvar inflammatory dermatoses, which comprise a heterogeneous group of diseases in which a broad, multidisciplinary approach is essential.

Allergic contact hobby dermatitis from turpentine.

INTRODUCTION: Turpentine is an oleoresin obtained from various species of pine. It contains a volatile oil (oil of turpentine) which is responsible for its properties and this is the form generally used. Opportunity for contact with turpentine is widespread. It is universally used as a solvent to dissolve and thin lacquers, varnishes and paints. It is also an ingredient in many liniments and cold remedies. Turpentine is regarded as both a local irritant and a sensitizer. Cases of allergic contact dermatitis in painters, mechanics, shoe repairers and home decorators have been reported. CASE REPORT: We report a case of a non-professional painter who developed a contact allergic dermatitis due to his exposure to turpentine while doing oil-painting as a hobby. DISCUSSION: Dermatitis is one of the biggest dangers of working with art materials and occupational contact dermatitis is often detected on the hands of the painters. Solvents are indispensable and turpentine is the most important and the traditional one used in oil-painting. Contact allergy to oil of turpentine was reported to have become rare in Europe but over the last few years, increased rates of turpentine sensitization have been reported.

Allergic contact hobby dermatitis from turpentine

Introduction: Turpentine is an oleoresin obtained from various species of pine. It contains a volatile oil (oil of turpentine) which is responsible for its properties and this is the form generally used. Opportunity for contact with turpentine is widespread. It is universally used as a solvent to dissolve and thin lacquers, varnishes and paints. It is also an ingredient in many liniments and cold remedies. Turpentine is regarded as both a local irritant and a sensitizer. Cases of allergic contact dermatitis in painters, mechanics, shoe repairers and home decorators have been reported. Case report: We report a case of a non-professional painter who developed a contact allergic dermatitis due to his exposure to turpentine while doing oil-painting as a hobby. Discussion: Dermatitis is one of the biggest dangers of working with art materials and occupational contact dermatitis is often detected on the hands of the painters. Solvents are indispensable and turpentine is the most important and the traditional one used in oil-painting. Contact allergy to oil of turpentine was reported to have become rare in Europe but over the last few years, increased rates of turpentine sensitization have been reported

No disponible

Aplasia cutánea congénita / Aplasia cutis congenita

La aplasia cutánea congénita (ACC) es una rara alteración caracterizada por la ausencia congénita de epidermis, dermis y, en ocasiones, de los tejidos subyacentes. La forma más frecuente afecta al vértex, y se puede presentar de forma aislada o asociada a otras malformaciones. Fue descrita por primera vez por Cordon, en 1767, y desde entonces se han documentado más de 500 casos. La frecuencia se ha estimado en torno a 3/10.000 recién nacidos. No hay predilección por la raza ni el sexo. La etiopatogenia no está clara. La manifestación clínica habitual es una lesión oval o circular, solitaria, sin pelo, bien delimitada, de 0,5-2 cm, y localizada en el vértex. Al nacer, las lesiones pueden estar cicatrizadas o presentarse como erosiones o incluso úlceras profundas, con riesgo de afectar a las meninges o la duramadre. La mejor opción terapéutica dependerá del tamaño del defecto

Aplasia cutis congenita (ACC) is a rare condition characterized by the congenital absence of epidermis, dermis and, in some cases, subcutaneous tissues. ACC occurs as an isolated defect (normally localized agenesis of the scalp) or with other associated anomalies. It was first described by Cordon in 1767, and more than 500 cases have been reported since, with an estimated incidence of 3 cases per 10,000 births. There is no racial or sexual predilection. The pathogenesis is not clear. The lesion is usually solitary, circular or oval, hairless and welldemarcated, and it ranges in size from 0.5-2 cm. At birth, the lesions may have already healed with scarring or may appear as superficial erosions or deep ulcerations, occasionally invo lving the dura or the meninges. The best therapeutic option depends on the size of the defect

Liquen «aureus» / Lichen aureus

El liquen aureus es una entidad rara, englobada dentro de un grupo de enfermedades denominadas dermatosis purpúricas pigmentarias. Todas ellas son, básicamente, capilaritis de origen desconocido, caracterizadas histológicamente por un infiltrado perivascular de linfocitos T, extravasación de eritrocitos y depósitos de hemosiderina. Aparece predominantemente en niños y adultos jóvenes de cualquier raza, con más frecuencia en hombres que en mujeres. La biopsia cutánea confirma el diagnóstico. Las lesiones cutáneas suelen ser asintomáticas, aunque en algunos casos puede aparecer un prurito intenso. Tienden a la cronicidad, y se ha descrito, sobre todo en niños, la resolución espontánea al cabo de varios años. La respuesta al tratamiento suele ser bastante limitada. Los corticoides tópicos potentes rara vez son eficaces; en algunos casos, se ha intentado el tratamiento con fototerapia (PUVA), pimecrolimus o pentoxifilina, con resultados variables

Lichen aureus is one of the subtypes of a rare group of diseases referred to as pigmented purpuric dermatoses. They are essentially a capillaritis of unknown origin. Histologically, they are characterized by a perivascular T-cell lymphocytic infiltrate centered on the superficial small blood vessels of the skin. Extravasation of red blood cells with marked hemosiderin deposition in macrophages is also found. It occurs predominantly in children and young adults of any race, more frequently in men than in women. A skin biopsy helps to confirm the diagnosis. The eruption is usually asymptomatic, but pruritus may be a prominent feature in some cases. The lesions usually persist unchanged for many years. Complete resolution rarely occurs, being more frequent in children than in adults. The response to treatment is usually quite limited. Potent topical steroids are rarely effective. Ultraviolet A phototherapy (PUVA), pimecrolimus or pentoxifylline have been reported to be beneficial in some cases

Mastocitosis cutánea / Cutaneous mastocytosis

Definimos mastocitosis como un conjunto de trastornos clínicos, producidos todos ellos por una proliferación de mastocitos y una acumulación en varios órganos, entre los cuales el más común es la piel. La mastocitosis cutánea fue descrita por primera vez por Nettleship y Tay, en 1869, quienes asumieron que la acumulación de mastocitos quedaba limitada únicamente a la piel. Y no es hasta 1949 cuando Ellis describió por primera vez una forma sistémica de mastocitosis. Aproximadamente, un 80% de los pacientes con mastocitosis presenta sólo una afectación cutánea, mientras que el 20% restante tiene una mastocitosis sistémica, y en más de la mitad de éstos aparecen también lesiones cutáneas. El diagnóstico se basa fundamentalmente en los hallazgos clínicos e histológicos. En general, el pronóstico en la edad pediátrica es bueno, con tendencia a la resolución espontánea antes de la pubertad. Aun así, un 15-30% de los niños en quienes la enfermedad persiste durante la edad adulta desarrollará una afectación sistémica

Mastocytosis is a rare disorder, characterized by an abnormal increase and accumulation of mast cells in one or more organ systems, most commonly the skin. Cutaneous mastocytosis was first described by Nettleship and Tay in 1869. It was assumed that the pathological accumulation of mast cells was limited to skin until 1949, when Ellis described a systemic form of mastocytosis for the first time. In approximately 80% of patients with mastocytosis, only the skin is involved. The remaining patients have systemic mastocytosis. More than a half of the patients with systemic mastocytosis have skin lesions as well. The diagnosis is mainly based on the clinical features and the histopathological examination of lesions. The prognosis in childhood is generally good, with a tendency for spontaneous resolution prior to puberty. However, 15% to 30% of the children whose disease persists into adulthood will develop systemic involvement

Hemiatrofia facial progresiva. Síndrome de Parry-Romberg / Progressive facial hemiatrophy: Parry-Romberg syndrome

Presentamos un caso de síndrome de Parry-Romberg en una niña de 11 años con una hemiatrofia facial de la región izquierda, sin otra sintomatología asociada. Se trata de una entidad poco frecuente y de origen desconocido que se caracteriza por una lenta y progresiva atrofia, generalmente unilateral, de las estructuras del territorio del nervio trigémino. Esta atrofia conduce a una asimetría facial que puede acompañarse de otras alteraciones neurológicas, oculares o cutáneas. Es más frecuente en mujeres y el diagnóstico se basa en unas manifestaciones clínicas características. el diagnóstico diferencial se debe llevar a cabo fundamentalmente con al esclerodermia lineal frontoparietal o en coup de sabre, pero en ocasiones puede llegar a ser imposible, dada la coexistencia de ambas, e incluso llevar a plantearse el hecho de que este síndrome sea una variante grave de esclerodermia lineal y que ambos procesos formen parte del mismo espectro clínico. No hay un tratamiento específico, salvo la cirugía plástica con la finalidad de mejorar el aspecto físico

We report a case of Parry-Romberg syndrome in an 11-year-old girl with a left facial hemiatrophy, with no other associated symptoms. This is an uncommon disorder of unknown origin, characterized by a slowly progressive atrophy, generally unilateral, of the skin and soft tissues in the region of the trigeminal nerve. These changes lead to facial asymetry that can be accompanied by neurological, ocular and/or cutaneous abnormalities. The syndrome is more common in females and the diagnosis is based on the clinical findings. The most important differential diagnosis is linear scleroderma en coup de sabre, although the distinction can be difficult because of the possible coexistence of the two entities. Some authors have conclude that this syndrome could be regarded as a variant of linear scleroderma and consider that they both pertain to the same clinicopathological spectrum. the only specific treatment is plastic surgery to correct the deformity and improve cosmetic disfigurement

[Mycobacterium marinum infection. Case report and review of the literature]. / Infección por Mycobacterium marinum. Presentación de un nuevo caso y revisión de la literatura.

Atypical mycobacterial infections are increasingly important in immunosuppressed patients as well as in healthy hosts. The atypical mycobacterium that most commonly affects the skin is Mycobacterium marinum. The infection should be suspected upon the presence of ulcers, nodules or chronic plaques and a history of contact with fresh or salt water. Optimal therapy is yet to be established. We report a case of Mycobacterium marinum infection in a patient receiving immunosuppressive therapy that responded favourably to treatment with doxicycline. We review the different antibiotic regimens prescribed in the past years for the treatment of Mycobacterium marinum infection.