Puesta al día en hemangiomas de la infancia / Update in infantile haemangioma

Los hemangiomas de la infancia (HI) son los tumores de partes blandas más frecuentes de la infancia. Se presentan en un 5 por ciento- 10 por ciento de la población pediátrica. El comportamiento de estos tumores es especial, ya que, a diferencia de otras neoplasias benignas, los HI tienden a regresar e involucionar espontáneamente en un 90 por ciento. Algunos pacientes con HI pueden sufrir complicaciones tales como ulceración y hemorragia. Estas complicaciones han llevado a la búsqueda de alternativas terapéuticas, donde, por mucho tiempo fueron los esteroides la primera opción de tratamiento. Esto hasta el año 2008, cuando se descubre accidentalmente el propranolol administrado por vía oral, como alternativa terapéutica para los HI severos, convirtiéndose hasta el día de hoy, en el gold standard de tratamiento. Dado la diversidad en su presentación, y las diferentes alternativas de manejo existentes, es que esta revisión pretende abordar la patogenia, clínica y enfrentamiento de estos tumores de la infancia.

Hemangiomas of infancy (HI) are the most common soft tissue tumors of childhood. They occur in up to percent-10 percent of the pediatric population and they have a special behaviour because, unlike other benign neoplasms, HI tend to regress spontaneously in up to 90 percent of the cases. Complications such as ulceration and hemorrhage can occur. These complications have led to search for new therapeutic options. Steroids were the first choice of treatment for a long time, until 2008, when oral propranolol was accidentally discovered as a potential treatment for large HI4. Today, propranolol is the gold standard for treatment. Given their variable clinical presentation and different therapeutic options available nowadays, this paper to review the pathogenesis, clinical presentation and approach of these tumors of infancy.

Efecto de la dieta en psoriasis moderada a severa, en pacientes con sobrepeso y obesos con tratamiento tópico y sistémico con metotrexato / Effect of diet on moderate or severe psoriasis in patients with obese and overweight under treatment with topical and systemic methotrexate

INTRODUCTION: Psoriasis is a chronic inflammatory disease with significant impact on quality of life. There are environmental factors that can change the course of the disease. OBJECTIVE: To investigate whether moderate weight loss, improves response to methotrexate in obese or overweight patients who suffer from the disease. METHODOLOGY: A controlled pilot project of 8 weeks was performed in 15 patients with moderate to severe psoriasis under treatment with Methotrexate and topical therapy. The intervention corresponded to a nutritional assessment, a strict diet and physical activity suggestion. Patients were evaluated using PASI, at 0, 4 and 8 weeks. RESULTS: After 8 weeks, mean PASI decreased by 13% and a decrease in weight, BMI and waist circumference was achieved, but without statistical significance. CONCLUSION: Further studies are required to demonstrate the importance of weight loss in obese or overweight psoriatic patients under methotrexate treatment.

INTRODUCCIÓN: La Psoriasis es una enfermedad inflamatoria crónica con importante impacto en la calidad de vida.Existen factores ambientalesque pueden modificar el curso de la enfermedad. OBJETIVO: Investigar si una pérdida moderada de peso, aumenta la respuesta a Metotrexato en pacientes obesos o con sobrepeso que padecen la enfermedad. METODOLOGÍA: Proyecto Piloto, controlado, de 8 semanas en 15 pacientes con psoriasis moderada a severa,en tratamiento con Metotrexato y terapia tópica, atendidos en Santiago de Chile. La intervención correspondió a una evaluación nutricional, una dieta estricta y sugerencia de actividad física. Los pacientes fueron evaluadas con PASI, a las 0, 4 y 8 semanas. RESULTADOS: Después de 8 semanas, se logró una disminución del PASI promedio en un 13% y una disminución del peso, IMC y circunferencia abdominal, pero sin significancia estadística. CONCLUSIÓN: Se requieren realizar estudios con un mayor número de pacientes para demostrar la importancia de una disminución de peso en el tratamiento con metotrexato en pacientes psoriáticos obesos o con sobrepeso.

[AA amyloidosis with cutaneous manifestations. Report of one case]. / Manifestaciones cutáneas de amiloidosis sistémica como clave diagnóstica. Caso clínico.

BACKGROUND: Systemic amyloidosis is a rare disease that can affect any organ. Its clinical manifestations are varied and nonspecific. The skin involvement of this disease is common and can be easily recognized on physical examination. We report a 57-year-old male presenting with a two years history of malaise, dyspnea and myalgias. On physical examination, ungueal dystrophy, orange pigmentation of eyelids with periocular petechiae and mild macroglossia were observed. Incisional biopsies of the eyelids, cheeks and hands were obtained. The pathological study demonstrated amyloid deposits. Since protein electrophoresis was normal, the diagnosis of AA amyloidosis was postulated.

Manifestaciones cutáneas de amiloidosis sistémica como clave diagnóstica: Caso clínico / AA amyloidosis with cutaneous manifestations: Report of one case

Background: Systemic amyloidosis is a rare disease that can affect any organ. Its clinical manifestations are varied and nonspecific. The skin involvement of this disease is common and can be easily recognized on physical examination. We report a 57-year-old male presenting with a two years history of malaise, dyspnea and myalgias. On physical examination, ungueal dystrophy, orange pigmentation of eyelids with periocular petechiae and mild macroglossia were observed. Incisional biopsies of the eyelids, cheeks and hands were obtained. The pathological study demonstrated amyloid deposits. Since protein electrophoresis was normal, the diagnosis of AA amyloidosis was postulated.

Pilomatrixoma: una presentación clínica inusual / Pilomatrixoma: unusual clinical presentation

El pilomatrixoma, una neoplasia benigna poco frecuente, se origina de la matriz del folículo piloso. Es más frecuente durante la infancia, especialmente entre los 5 y 15 años de edad. La forma múltiple de pilomatrixoma es rara y su presencia se asocia a síndromes sistémicos, entre los cuales destaca la distrofa miotónica. El diagnóstico de pilomatrixoma es fundamentalmente clínico. Estudios complementarios como la ecografía pueden ser útiles para confrmar su diagnóstico. La resección quirúrgica de la lesión corresponde el tratamiento curativo, con baja tasa de recurrencia. Se presenta una paciente de sexo femenino, de 17 años de edad, por su pilomatrixoma de características clínicas inusuales en edad pediátrica: variante anetodérmica, forma múltiple y crecimiento rápido.

Pilomatrixoma is a rare, benign neoplasm that is derived from hair matrix cells. It is more frequent during childhood, especially between the ages of 5 and 15 years. Pilomatrixoma is usually a hard, well circumscribed, solitary lesion, and can be located on any part of the body, except palms and soles, with a predilection for maxilofacial region. Multiple pilomatrixomas are rare and they have been associated with various systemic syndromes, of which myotonic dystrophy has been the most described. The diagnosis of pilomatrixoma is fundamentally clinical. Complementary studies such as ultrasound can be useful to confrm the diagnosis. Excisional surgery is the standard curative treatment. We present a 17-year-old female patient with an extremely uncommon clinical presentation of pilomatrixoma: multiple lesions, anetodermic, and rapid growth.

[Pilomatrixoma: unusual clinical presentation]. / Pilomatrixoma: una presentación clínica inusual.

Pilomatrixoma is a rare, benign neoplasm that is derived from hair matrix cells. It is more frequent during childhood, especially between the ages of 5 and 15 years. Pilomatrixoma is usually a hard, well circumscribed, solitary lesion, and can be located on any part of the body, except palms and soles, with a predilection for maxillofacial region. Multiple pilomatrixomas are rare and they have been associated with various systemic syndromes, of which myotonic dystrophy has been the most described. The diagnosis of pilomatrixoma is fundamentally clinical. Complementary studies such as ultrasound can be useful to confirm the diagnosis. Excisional surgery is the standard curative treatment. We present a 17-year-old female patient with an extremely uncommon clinical presentation of pilomatrixoma: multiple lesions, anetodermic, and rapid growth.