[Bazex syndrome: a rare paraneoplastic disease]. / Síndrome de Bazex: una enfermedad paraneoplásica infrecuente.

Bazex syndrome is a paraneoplastic disorder most commonly linked to squamous cell carcinomas of the upper aerodigestive tract, followed by lung cancer and other malignancies. It manifests through three stages of skin involvement that mirror the tumor's progression. Remarkably, skin lesions precede tumor symptoms or diagnosis in two-thirds of cases, underscoring the crucial role of suspecting this condition as it can promptly reveal an underlying neoplasm. Treatment primarily focuses on addressing the root neoplasm, with recurrent skin lesions potentially indicating tumor relapse. In this context, we present a clinical case involving a male patient whose manifestation of this syndrome facilitated the timely diagnosis of lung adenocarcinoma. This case underscores the significance of understanding this uncommon syndrome and its link to cancer, enabling early and accurate oncological diagnosis.

El síndrome de Bazex es una enfermedad paraneoplásica que se asocia con mayor frecuencia a carcinomas de células escamosas del tracto aerodigestivo superior, seguido en frecuencia por el cáncer de pulmón y otras neoplasias. Afecta a la piel en tres etapas que tienen un comportamiento paralelo al crecimiento del tumor. En dos tercios de los casos, las lesiones cutáneas preceden a los síntomas o al diagnóstico del tumor. De ahí la importancia de la sospecha de esta entidad, que puede desenmascarar a la neoplasia asociada en una etapa temprana. Su tratamiento consiste en tratar la neoplasia subyacente. La recurrencia de las lesiones cutáneas puede revelar la recaída del tumor. Comunicamos el caso clínico de un paciente de sexo masculino en el cual el hallazgo de este síndrome permitió realizar el diagnóstico de un adenocarcinoma de pulmón, lo cual destaca la importancia de conocer a esta rara enfermedad y su asociación con cáncer, para poder realizar el diagnóstico oncológico de forma temprana y oportuna.

Calcinosis escrotal: ¿una entidad idiopática? / Scrotal Calcinosis: an Idiopathic Entity?

La calcinosis escrotal es una enfermedad benigna e infrecuente que se presenta en adultos de mediana edad, con múltiples nódulos asintomáticos a nivel de la piel del escroto. Algunos autores vinculan la aparición de estas lesiones a la calcificación secundaria de quistes epidérmicos o ecrinos. Cuando no se encuentra relacionada con dichas entidades ni con alteraciones del metabolismo fosfocálcico, el cuadro se considera idiopático. El tratamiento de elección es quirúrgico, en caso de impacto en la calidad de vida o relevancia estética para el paciente. (AU)

Scrotal calcinosis is a rare, benign disease that presents in middle-aged adults with multiple asymptomatic nodules on the skin of the scrotum. Some authors link the appearance of these lesions to the secondary calcification of epidermal or eccrine cysts. When it is not related to these entities or to alterations in phosphocalcic metabolism, the condition is considered idiopathic. The treatment of choice is surgical, in case of impact on the quality of life or aesthetic relevance for the patient. (AU)

Hemangioma segmentario de la barba / Segmental Beard Hemangioma

Los hemangiomas infantiles son el resultado de la proliferación de células del endotelio vascular y representan los tumores benignos más frecuentes en la infancia, con una incidencia estimada del 4-10% en bebés caucásicos. Se clasifican según el número, la profundidad y la distribución. Dentro de esta última clasificación se encuentran aquellos denominados segmentarios, que se caracterizan por su distribución extensa en áreas de prolongaciones mesodérmicas embrionarias. Se comunica el caso de una paciente evaluada al mes y medio de vida, con un hemangioma extenso del área mandibular y cuello anterior (hemangioma segmentario de la barba). Se describe la importancia de los estudios complementarios para evaluar el compromiso de órganos subyacentes, para detectar síndromes asociados y definir el tratamiento sobre la base de estos resultados. (AU)

Infantile hemangiomas arise from the proliferation of vascular endothelial cells and represent the most common benign tumors in infancy, with an estimated incidence of 4-10% in Caucasian infants. They vary according to their number, depth, and distribution. Within the latter classification are the so-called segmental ones, which feature an extensive distribution in areas of embryonic mesodermal extensions. We report the case of a patient evaluated at one and a half months of life with an extensive hemangioma of the mandibular area and anterior neck (segmental hemangioma of the beard). We describe the importance of complementary studies for evaluating the involvement of underlying organs, detecting associated syndromes, and defining the treatment based on these findings. (AU)

Corticoides sistémicos en el tratamiento de la radiodermitis grave: una serie de casos / Systemic corticoids in the treatment of severe radiodermitis: a series of cases

Introducción: la radiodermitis es uno de los efectos secundarios más frecuentes de la radioterapia y afecta aproximadamente al 95% de los pacientes que la reciben. La radiodermitis aguda se presenta dentro de los 90 días posteriores al inicio del tratamiento, tiene un profundo impacto en la calidad de vida de los pacientes y puede ser la causa de la interrupción prematura de la radioterapia. Su tratamiento es complejo y el papel de los corticoides sistémicos en él aún no ha sido evaluado. Materiales y métodos: estudio descriptivo de 6 pacientes mayores de 18 años con radiodermitis grave, tratados con corticoides sistémicos al no responder a la terapia tópica inicial. Hubo un seguimiento de 6 meses, entre el 1 de junio de 2019 y el 30 de mayo de 2020, en el Servicio de Dermatología de un hospital de alta complejidad. Resultados: se indicó tratamiento con corticoides sistémicos en dosis de meprednisona 40 mg/día o equivalentes, durante 5 días, con resolución completa del cuadro en un período máximo de 15 días. Discusión: en la bibliografía no hemos encontrado trabajos científicos que comuniquen o evalúen la utilidad de los corticoides sistémicos en la radiodermitis grave. Proponemos, entonces, demostrar su utilidad en esta patología. Conclusión: el objetivo de este trabajo es comunicar nuestra experiencia en pacientes con radiodermitis aguda grave, con gran repercusión en el estado general, que evolucionaron con una rápida resolución del cuadro y un adecuado manejo sintomático, mediante el uso de corticoides sistémicos. (AU)

Introduction: radiodermitis is one of the most frequent side effects of radiotherapy and affects approximately 95% of the patients who receive it. Acute radiodermitis occurs within 90 days after the start of treatment, has a profound impact on the quality of life of patients and may be the cause of premature discontinuation of radiotherapy. Its treatment is complex and the role of systemic corticosteroids in it has not yet been evaluated. Materials and methods: descriptive study of 6 patients older than 18 years with severe radiodermatitis, treated with systemic corticosteroids when they did not respond to initial topical therapy. With a 6-month follow-up, between June 1, 2019 and May 30, 2020 at the Dermatology Service of a high complexity hospital. Results: treatment with systemic corticosteroids was indicated at a dose of meprednisone 40 mg/day or equivalent, for 5 days, with complete resolution of the symptoms in a maximum period of 15 days. Discussion: in the literature, we have not found scientific papers that report or evaluate the usefulness of systemic corticosteroids in severe radiodermatitis. We propose to demonstrate their usefulness in this pathology. Conclusion: the objective of this work is to communicate our experience in patients with severe acute radiodermatitis, with great repercussions on the general state, who evolved with rapid resolution of the symptoms and adequate symptomatic management, with the use of systemic corticosteroids. (AU)

ECHO project in atopic dermatitis in Argentina: An innovative strategy to reach underserved areas with up to date knowledge, first year of experience

Abstract Background: The ECHO® (Extension for Community Healthcare Outcomes) project is a model of distance medical education developed in the United States to support health professionals in the management of patients with complex diseases. Since 2019, it has been implemented in atopic dermatitis (AD) in Argentina. The program consists of the periodic presentation of clinical cases by videoconference, virtual classes, and a permanently available open chat between professionals in charge of patients with AD and a group of experts. Objective: The objective of this study was to analyze the impact of the ECHO Project AD on the medical knowledge and medical skills of Argentinian health professionals when treating patients with AD. Methods: A survey was carried out among the participants in order to evaluate the impact of the program on the care of patients with AD. Results: ECHO Project AD revealed a significant improvement in the management of patients with AD. The program contributed to the interpretation and use of severity scores, use of phototherapy, and management and prescription of both classic and innovative topical and systemic treatments. Study limitations: The reduced number of participants and the short period of time. The answers of the survey may be biased by the enthusiasm of the participants. Conclusions: The ECHO project is an educational tool that enhances the medical skills of doctors and institutions, in which a climate of a partnership comes first and the participants look forward to learning from experiences, successes, and mistakes from one another, producing a scientific hub in constant evolution.

ECHO project in atopic dermatitis in Argentina: An innovative strategy to reach underserved areas with up to date knowledge, first year of experience.

BACKGROUND: The ECHO® (Extension for Community Healthcare Outcomes) project is a model of distance medical education developed in the United States to support health professionals in the management of patients with complex diseases. Since 2019, it has been implemented in atopic dermatitis (AD) in Argentina. The program consists of the periodic presentation of clinical cases by videoconference, virtual classes, and a permanently available open chat between professionals in charge of patients with AD and a group of experts. OBJECTIVE: The objective of this study was to analyze the impact of the ECHO Project AD on the medical knowledge and medical skills of Argentinian health professionals when treating patients with AD. METHODS: A survey was carried out among the participants in order to evaluate the impact of the program on the care of patients with AD. RESULTS: ECHO Project AD revealed a significant improvement in the management of patients with AD. The program contributed to the interpretation and use of severity scores, use of phototherapy, and management and prescription of both classic and innovative topical and systemic treatments. STUDY LIMITATIONS: The reduced number of participants and the short period of time. The answers of the survey may be biased by the enthusiasm of the participants. CONCLUSIONS: The ECHO project is an educational tool that enhances the medical skills of doctors and institutions, in which a climate of a partnership comes first and the participants look forward to learning from experiences, successes, and mistakes from one another, producing a scientific hub in constant evolution.

Fenómeno infrecuente la co-localización de alopecia frontal fibrosante y vitiligo en un varón: informe de un caso / Infrequent phenomenon the co-localization of fibrosing frontal alopecia and vitiligo in a male: case repor

La alopecia frontal fibrosante es una alopecia cicatricial que se caracteriza por la recesión de la línea de implantación frontotemporal que afecta principalmente a mujeres caucásicas en edad posmenopáusica y rara vez a hombres. Actualmente los mecanismos específicos de desarrollo continúan en estudio; sin embargo hay varias hipótesis sobre la asociación de la alopecia frontal fibrosante con otros trastornos autoinmunitarios. Se comunica el caso de un paciente masculino de 58 años con alopecia frontal fibrosante en áreas comprometidas por vitiligo. (AU)

Frontal fibrosing alopecia is a cicatricial alopecic characterized by progressive regression of the frontotemporal hairline. It usually affects postmenopausal caucasian women, and rarely men. Currently the specific mechanisms of development remain unknown, however there are several hypotheses about the association of frontal fibrosing alopecia with other autoimmune disorders. The case of a 58-year-old male patient with frontal fibrosing alopecia in areas affected by vitiligo. (AU)

Validación de un cuestionario en español sobre la usabilidad de la telemedicina / Validation of a Spanish questionnaire on telemedicine usability

Resumen La telemedicina es la prestación de servicios de la salud mediante la utilización de tecnologías de la información y la comunicación. Su implementación se realiza a través de sistemas cuya usabilidad es variable. En 2016, Parmant y col., desarrollaron y validaron un cuestionario en inglés que mide su usabilidad y permite evaluar los factores que influyen en la misma. En 2019, tradujimos, adaptamos transculturalmente y publicamos este cuestionario en español. Con el objetivo de transformar el mismo en una herramienta confiable y accesible, cuya utilidad es indiscutible en el contexto actual, llevamos a cabo un trabajo de investigación que permitió su validación y simplificación.

Abstract Telemedicine is the provision of services by health professionals through the use of information and communication technologies. Its implementation is usually carried out through systems of variable usability. In 2016, Parmanto et al, developed and validated a questionnaire in English that measures its usability and allows to evaluate all factors that influence it. In 2019, we translated, adapted cross-culturally, and published this questionnaire in Spanish. With the aim of transforming it into a reliable and accessible tool, the utility of which is indisputable in the current context, we carried out a research work that allowed its validation and simplification.

[Validation of a Spanish questionnaire on telemedicine usability]. / Validación de un cuestionario en español sobre la usabilidad de la telemedicina.

Telemedicine is the provision of services by health professionals through the use of information and communication technologies. Its implementation is usually carried out through systems of va riable usability. In 2016, Parmanto et al, developed and validated a questionnaire in English that measures its usability and allows to evaluate all factors that influence it. In 2019, we translated, adapted cross-culturally, and published this questionnaire in Spanish. With the aim of transforming it into a reliable and accessible tool, the utility of which is indisputable in the current context, we carried out a research work that allowed its validation and simplification.

La telemedicina es la prestación de servicios de la salud mediante la utilización de tecnologías de la información y la comunicación. Su implementación se realiza a través de sistemas cuya usabilidad es variable. En 2016, Parmant y col., desarrollaron y validaron un cuestionario en inglés que mide su usabilidad y permite evaluar los factores que influyen en la misma. En 2019, tradujimos, adaptamos transculturalmente y publicamos este cuestionario en español. Con el objetivo de transformar el mismo en una herramienta confiable y accesible, cuya utilidad es indiscutible en el contexto actual, llevamos a cabo un trabajo de investigación que permitió su validación y simplificación.

[Spanish translation and transcultural adaptation of a questionnaire on telemedicine usability]. / Traducción al español y adaptación transcultural de un cuestionario sobre la usabilidad de la telemedicina.

Telemedicine is the provision of services by health professionals, through the use of information and communication technologies. Its implementation helps to shorten distances, facilitate access to the health system, improve the effectiveness of care and reduce costs. In 2016, Bambang Parmanto et al, developed and validated a questionnaire in English that measures its usability and allows to evaluate all the factors that influence it. Since similar tools do not exist in the Spanish language to date, a research work was carried out for transcultural translation and adaptation that included seven steps. The questionnaire obtained after this process provides a valuable tool to objectify information that is vital to contribute to the cycle of continuous improvement of the care process of these characteristics, in which patients, health professionals and funders are involved.

La telemedicina es la prestación de servicios por profesionales de la salud, mediante la utilización de tecnologías de la información y la comunicación. Su implementación contribuye a acortar distancias, facilitar el acceso al sistema de salud, mejorar la efectividad de los cuidados y reducir costos. En 2016, Bambang Parmanto y col., desarrollaron y validaron un cuestionario en inglés que mide su usabilidad y permite evaluar todos los factores que influyen en la misma. Dado que no existen hasta la fecha herramientas similares en el idioma español, se llevó a cabo un trabajo de investigación para la traducción y adaptación transcultural que incluyó siete pasos. El cuestionario obtenido luego de este proceso brinda una herramienta valiosa que permite objetivar información que resulta vital para contribuir al ciclo de mejora continua del proceso asistencial de estas características, en el que están involucrados los pacientes, los profesionales de la salud y los financiadores.

Traducción al español y adaptación transcultural de un cuestionario sobre la usabilidad de la telemedicina / Spanish translation and transcultural adaptation of a questionnaire on telemedicine usability

La telemedicina es la prestación de servicios por profesionales de la salud, mediante la utilización de tecnologías de la información y la comunicación. Su implementación contribuye a acortar distancias, facilitar el acceso al sistema de salud, mejorar la efectividad de los cuidados y reducir costos. En 2016, Bambang Parmanto y col., desarrollaron y validaron un cuestionario en inglés que mide su usabilidad y permite evaluar todos los factores que influyen en la misma. Dado que no existen hasta la fecha herramientas similares en el idioma español, se llevó a cabo un trabajo de investigación para la traducción y adaptación transcultural que incluyó siete pasos. El cuestionario obtenido luego de este proceso brinda una herramienta valiosa que permite objetivar información que resulta vital para contribuir al ciclo de mejora continua del proceso asistencial de estas características, en el que están involucrados los pacientes, los profesionales de la salud y los financiadores.

Telemedicine is the provision of services by health professionals, through the use of information and communication technologies. Its implementation helps to shorten distances, facilitate access to the health system, improve the effectiveness of care and reduce costs. In 2016, Bambang Parmanto et al, developed and validated a questionnaire in English that measures its usability and allows to evaluate all the factors that influence it. Since similar tools do not exist in the Spanish language to date, a research work was carried out for transcultural translation and adaptation that included seven steps. The questionnaire obtained after this process provides a valuable tool to objectify information that is vital to contribute to the cycle of continuous improvement of the care process of these characteristics, in which patients, health professionals and funders are involved.

Real-life approach and treatment of chronic urticaria in Argentina: A National Survey.

The management of chronic urticaria (CU) has been controversial. Recently updated international guidelines propose evidence-based diagnosis and treatment. Nevertheless, dermatologists have heterogeneous approaches to managing CU. To estimate the percentage of dermatologists who have an optimal approach to CU according to the international guidelines, and to explore the variables associated with optimal management a cross-sectional study using a 17-question survey was delivered by email to dermatologists from Argentina. Optimal first, second, and third line treatment were considered when dermatologists chose a nonsedative antihistamine; increased the dosage of nonsedative antihistamines, and added omalizumab, respectively. Logistic regression was used to identify variables associated with optimal management. A total of 165 questionnaires were available for analysis. An optimal first, second, and third-line treatment approach was identified in 50%, 35%, and 15% of the dermatologists, respectively. The dermatologists' age being above 55 years old (OR: 0.12, 95% CI: 0.01-0.99, p .005) and having more than 5 years of expertise (OR: 0.4, 95% CI: 0.19-0.81, p .001) were significantly associated with a suboptimal approach in second-line treatment. We could not find variables associated with an optimal first or third-line treatment. The real-life management of CU in Argentina is partly suboptimal according to the international guidelines.

WhatsApp: A Real-Time Tool to Reduce the Knowledge Gap and Share the Best Clinical Practices in Psoriasis.

BACKGROUND: Psoriasis is a chronic inflammatory disease that affects around 100 million people worldwide. The burden of disease is high, but more recent therapies show promising results. Clinicians need, however, more training in the use of such therapies. INTRODUCTION: Project ECHO® (Extension for Community Healthcare Outcomes) is structured around the promise of delivering medical education at a distance, empowering clinicians who operate in remote areas. The use of instant messaging services, such as WhatsApp® Messenger, has the potential to improve on the existing framework and bridge the existing gap of knowledge. This article reports on a study concerning the implementation of a WhatsApp discussion group in Project ECHO Psoriasis in Argentina. MATERIALS AND METHODS: One hundred thirty-two dermatologists in Argentina were invited to participate in the WhatsApp discussion group. After 1 year of participation, a follow-up questionnaire was used to assess the effectiveness of the project. RESULTS: Eighty dermatologists participated. All questions placed in the discussion were answered by a psoriasis specialist, 79% of which were answered within the first 5 min. Clinicians report significant improvement in diagnosis, comorbidities, and treatment with both conventional and biological therapies. DISCUSSION: Preliminary results are promising. This new cost-effective solution builds on the existing Project ECHO Psoriasis in Argentina and shows potential in bridging the gap of knowledge, promoting better clinical decisions through empowerment of medical doctors operating in remote locations. CONCLUSIONS: Further research is needed to increase generalization of the results. Moreover, it would be interesting to match the data from the discussion group with follow-up questionnaires.

[Implementation of a telementoring model of medical education in psoriasis]. / Implementación de una modalidad de educación médica a distancia en psoriasis.

The ECHO® (Extension for Community Healthcare Outcomes) project is a model of distance medical education. Its mission is to expand knowledge and evaluate the results of this action, both in the training of human resources in healthcare and in the accomplishment of the best medical practices in the community target. It is developed through case presentation videoconferencing, between experts in chronic and complex diseases and physicians, with the aim of reducing the healthcare asymmetries between large urban centers and peripherals areas. We have implemented this telementoring for dermatologists and residents who treat patients with psoriasis. After 10 sessions, a survey was conducted to evaluate the educational attainment of the participants. A significant improvement was found in their abilities to determine the severity of psoriasis, screening for arthritis, assessment of the patient before beginning systemic treatment and appropriate follow-up under different systemic therapies. ECHO replication model helped improve the skills of the participants in the management of this disease, and reduced professional isolation.

Implementación de una modalidad de educación médica a distancia en psoriasis / Implementation of a telementoring model of medical education in psoriasis

El proyecto ECHO® (Extension for Community Healthcare Outcomes) es un modelo de educación médica a distancia. Tiene por misión expandir conocimiento y evaluar los resultados de esta acción, tanto en la formación del recurso humano en salud como en el cumplimiento de las mejores prácticas médicas en la población alcanzada. Se desarrolla a través de la presentación de casos clínicos por videoconferencias entre expertos en enfermedades crónicas y complejas y profesionales que manejan dichas enfermedades, con el objetivo de disminuir las asimetrías de atención entre los grandes centros urbanos y los periféricos. Implementamos este formato de telementoring para pacientes con psoriasis orientado a dermatólogos y residentes. Luego de 10 sesiones de videoconferencia, se realizó una encuesta para evaluar los logros educativos de los participantes. Se observó una mejoría significativa en sus habilidades para determinar el grado de compromiso de la psoriasis, detectar artritis, evaluar al paciente antes de iniciar tratamiento sistémico y el seguimiento apropiado bajo las diferentes terapias sistémicas indicadas. La replicación del modelo ECHO® contribuyó a mejorar la capacidad de los participantes en el manejo de esta enfermedad y disminuir el aislamiento profesional.

The ECHO® (Extension for Community Healthcare Outcomes) project is a model of distance medical education. Its mission is to expand knowledge and evaluate the results of this action, both in the training of human resources in healthcare and in the accomplishment of the best medical practices in the community target. It is developed through case presentation videoconferencing, between experts in chronic and complex diseases and physicians, with the aim of reducing the healthcare asymmetries between large urban centers and peripherals areas. We have implemented this telementoring for dermatologists and residents who treat patients with psoriasis. After 10 sessions, a survey was conducted to evaluate the educational attainment of the participants. A significant improvement was found in their abilities to determine the severity of psoriasis, screening for arthritis, assessment of the patient before beginning systemic treatment and appropriate follow-up under different systemic therapies. ECHO replication model helped improve the skills of the participants in the management of this disease, and reduced professional isolation.

[Nevoid basal cell carcinoma syndrome with corpus callosum agenesis, PTCH1 mutation and absence of basal cell carcinoma]. / Síndrome de carcinoma basocelular nevoide con agenesia de cuerpo calloso, mutación en PTCH1 y ausencia de carcinoma basocelular.

Nevoid Basal Cell Carcinoma Syndrome (NBCCS) or Gorlin-Goltz syndrome is a rare autosomal dominant disorder, mainly due to PTCH1 gene mutations, that comprises a broad spectrum of clinical manifestations. The presence of multiple basal cell carcinomas (BCCs) is a cardinal sign in NBCCS, therefore cases in which BCCs are absent entails a delay in the diagnosis.We present a 14 years old boy with a clinical diagnosis of NBCCS by the presence of odontogenic cysts, hypertelorism, macrocephaly, and corpus callosum agenesia, but with absence of skin lesions. His 43 years old mother has NBCCS diagnosis and no history of BCCs. For a deeper study, PTCH1 mutation screening from peripheral blood samples were performed by both bidirectional sequencing and multiplex ligation dependent probe amplification (MLPA) techniques. The proband and his mother carry 25 pb duplication in exon 10 (c.1375dupl25bp) that causes a reading frameshift with a premature stop codon. Bioinformatics analysis predicted that this mutation results in a truncated protein shorter than normal. Our results suggest that complete clinical and genealogical studies accompanied by genetic analysis are essential in the early detection of the NBCCS cases such the one presented here.