Granulomas en dermatopatología: principales entidades. Parte I / Granulomas in Dermatopathology: Principal Diagnoses - Part 1

En esta serie de 2 artículos realizamos una revisión de las principales entidades dermatopatológicas que cursan con granulomas. Esta primera parte se ha centrado en la aclaración de los conceptos, la presentación de los tipos de granulomas y de las células gigantes, así como en entidades muy diversas de origen no infeccioso. Algunas de ellas de origen metabólico, como la necrobiosis lipoídica: otras relacionadas con linfomas, como la micosis fungoides granulomatosa, y otras tan extendidas que casi resultan un problema cotidiano en las consultas de dermatología, como la rosácea (AU)

This series of 2 articles on dermatopathologic diagnoses reviews conditions in which granulomas form. Part 1 clarifies concepts, discusses the presentation of different types of granulomas and giant cells, and considers a large variety of noninfectious diseases. Some granulomatous diseases have a metabolic origin, as in necrobiosis lipoidica. Others, such as granulomatous mycosis fungoides, are related to lymphomas. Still others, such as rosacea, are so common that dermatologists see them nearly daily in clinical practice (AU)

Granulomas en dermatopatología: principales entidades. Parte II / Granulomas in Dermatopathology: Principal Diagnoses — Part 2

Esta es la segunda parte de una serie dedicada a la patología granulomatosa en la biopsia cutánea. Mientras que en la primera parte hablamos, entre otras, de algunas condiciones metabólicas y tumorales, esta segunda parte abordará fundamentalmente patología infecciosa de diversos tipos, junto con otras condiciones relativamente frecuentes en las consultas de dermatología (AU)

Part 2 of this series on granulomatous diseases focuses on skin biopsy findings. Whereas the first part treated noninfectious conditions (metabolic disorders and tumors, among other conditions), this part mainly deals with various types of infectious disease along with other conditions seen fairly often by clinical dermatologists (AU)

Granulomas in Dermatopathology: Principal Diagnoses - Part 1. / Granulomas en dermatopatología: principales entidades. Parte I.

This series of 2 articles on dermatopathologic diagnoses reviews conditions in which granulomas form. Part 1 clarifies concepts, discusses the presentation of different types of granulomas and giant cells, and considers a large variety of noninfectious diseases. Some granulomatous diseases have a metabolic origin, as in necrobiosis lipoidica. Others, such as granulomatous mycosis fungoides, are related to lymphomas. Still others, such as rosacea, are so common that dermatologists see them nearly daily in clinical practice.

Granulomas in Dermatopathology: Principal Diagnoses - Part 2. / Granulomas en dermatopatología: principales entidades. Parte II.

Part 2 of this series on granulomatous diseases focuses on skin biopsy findings. Whereas the first part treated noninfectious conditions (metabolic disorders and tumors, among other conditions), this part mainly deals with various types of infectious disease along with other conditions seen fairly often by clinical dermatologists.

Queratoconjuntivitis por Vittaforma corneae: una enfermedad emergente entre los viajeros que regresan del sudeste asiático / Vittaforma Corneae keratoconjunctivitis: An emerging pathology among travelers returning from Southeast Asia

La queratoconjuntivitis por Microsporidium es un cuadro infrecuente. Se ha asociado a brotes epidémicos en Asia relacionados con la exposición a agua o tierra contaminada. Habitualmente estos cuadros son autolimitados y leves, pero pueden tener evoluciones prolongadas. Presentamos el caso de una paciente de 29 años que comenzó con dolor, enrojecimiento, visión borrosa en su ojo derecho tras su vuelta de un viaje a Singapur y que no mejoró tras un tratamiento convencional frente a conjuntivitis. Fue diagnosticada de queratoconjuntivitis por Microsporidium mediante PCR y tinción con PAS del epitelio corneal. El tratamiento inicial fue desbridamiento epitelial, albendazol oral y voriconazol, levofloxacino y propamidina, pero la enfermedad conjuntival y corneal no cedió hasta que si introdujeron corticoides tópicos 5 meses después para tratar la limbitis. Debemos tener la sospecha de queratitis por Microsporidium en casos de queratitis unilateral y conjuntivitis, sobre todo en pacientes que vuelvan de Asia

Microsporidium keratoconjunctivitis is an very rare disease. It is related to outbreaks in Asia due to exposure to contaminated water or soil. Microsporidium keratoconjunctivitis is a a self-limited disease, but it could have long term courses. We present the case of a 29 year old woman who started with pain, redness and blurred vision after a holiday in Singapore and did not respond to conjunctivitis treatment. PCR sequencing and PAS staining of corneal epithelial biopsy identified Vittaforma corneae as the causative organism. Treatment was initiated with corneal debridement, oral albendazol, and intensive topical voriconazole, levofloxacin and propamidine, but the conjunctival and corneal disease was only resolved 5 months later with the introduction of topical steroids to treat her severe limbitis. Suspicion of Microsporidium keratoconjunctivitis should be raised amongst ophthalmologists in unilateral keratitis with mild conjunctivitis in travelers from Asia

Vittaforma Corneae keratoconjunctivitis: An emerging pathology among travelers returning from Southeast Asia. / Queratoconjuntivitis por Vittaforma corneae: una enfermedad emergente entre los viajeros que regresan del sudeste asiático.

Microsporidium keratoconjunctivitis is an very rare disease. It is related to outbreaks in Asia due to exposure to contaminated water or soil. Microsporidium keratoconjunctivitis is a a self-limited disease, but it could have long term courses. We present the case of a 29 year old woman who started with pain, redness and blurred vision after a holiday in Singapore and did not respond to conjunctivitis treatment. PCR sequencing and PAS staining of corneal epithelial biopsy identified Vittaforma corneae as the causative organism. Treatment was initiated with corneal debridement, oral albendazol, and intensive topical voriconazole, levofloxacin and propamidine, but the conjunctival and corneal disease was only resolved 5 months later with the introduction of topical steroids to treat her severe limbitis. Suspicion of Microsporidium keratoconjunctivitis should be raised amongst ophthalmologists in unilateral keratitis with mild conjunctivitis in travelers from Asia.

Dermatitis granulomatosa intersticial y tocilizumab: ¿es este un tratamiento de utilidad para esta dermatosis? / Interstitial Granulomatous Dermatitis and Tocilizumab: Is This Treatment Useful for This Skin Condition?

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Nódulo en la mama tras radioterapia / A Nodule on the Breast after Radiation Therapy

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Early outcome of a 31-gene expression profile test in 86 AJCC stage IB-II melanoma patients. A prospective multicentre cohort study.

BACKGROUND: The clinical and pathological features of primary melanoma are not sufficiently sensitive to accurately predict which patients are at a greater risk of relapse. Recently, a 31-gene expression profile (DecisionDx-Melanoma) test has shown promising results. OBJECTIVES: To evaluate the early prognostic performance of a genetic signature in a multicentre prospectively evaluated cohort. METHODS: Inclusion of patients with AJCC stages IB and II conducted between April 2015 and December 2016. All patients were followed up prospectively to assess their risk of relapse. Prognostic performance of this test was evaluated individually and later combined with the AJCC staging system. Prognostic accuracy of disease-free survival was determined using Kaplan-Meier curves and Cox regression analysis. Results of the gene expression profile test were designated as Class 1 (low risk) and Class 2 (high risk). RESULTS: Median follow-up time was 26 months (IQR 22-30). The gene expression profile test was performed with 86 patients; seven had developed metastasis (8.1%) and all of them were in the Class 2 group, representing 21.2% of this group. Gene expression profile was an independent prognostic factor for relapse as indicated by multivariate Cox regression analysis, adjusted for AJCC stages and age. CONCLUSIONS: This prospective multicentre cohort study, performed in a Spanish Caucasian cohort, shows that this 31-gene expression profile test could correctly identify patients at early AJCC stages who are at greater risk of relapse. We believe that gene expression profile in combination with the AJCC staging system could well improve the detection of patients who need intensive surveillance and optimize follow-up strategies.

Sífilis secundaria simulando liquen plano en el paciente con infección por VIH / Secondary Syphilis Mimicking Lichen Planus in a Patient With HIV Coinfection

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Malformaciones arteriovenosas digitales adquiridas. Una anomalía vascular infrecuente tratada con láser / Acquired Digital Arteriovenous Malformations: Laser Treatment Of An Uncommon Vascular Abnormality

Las malformaciones arteriovenosas digitales adquiridas (MAVDA) son lesiones vasculares infrecuentes, habitualmente localizadas en zonas acrales, principalmente en los dedos de las manos. Su tratamiento a menudo requiere la extirpación completa de la lesión, requiriendo reconstrucciones complejas como colgajos o injertos. Sin embargo, en los últimos años se han comunicado algunos casos tratados satisfactoriamente con láser. Nosotros aportamos una serie de 6 pacientes con MAVDA, 5 de ellos tratados con láser con buena respuesta, y otro paciente tratado quirúrgicamente. En este trabajo hacemos una revisión exhaustiva de este tipo de lesiones y concluimos que la terapia láser es una alternativa terapéutica segura y efectiva para estas malformaciones, siempre y cuando los pacientes sean diagnosticados correctamente y se encuentren en estadio i de Schöbinger (AU)

Acquired digital arteriovenous malformations (ADAVM) are uncommon acral vascular lesions most often affecting the finger. Treatment usually requires complete excision of the lesion with complex advancement flap or skin graft reconstruction. However, in recent years, some authors have reported satisfactory outcomes with laser treatment. We describe a series of 6 patients with ADAVM, 5 of whom received laser treatment and obtained a good response. The other patient was treated surgically. After an in-depth review of this type of lesion, we conclude that laser treatment is a safe and effective alternative for these malformations, provided the patients are appropriately diagnosed and have Schöbinger stage i lesions (AU)

Acquired digital arteriovenous malformations: laser treatment of an uncommon vascular abnormality.

Acquired digital arteriovenous malformations (ADAVM) are uncommon acral vascular lesions most often affecting the finger. Treatment usually requires complete excision of the lesion with complex advancement flap or skin graft reconstruction. However, in recent years, some authors have reported satisfactory outcomes with laser treatment. We describe a series of 6 patients with ADAVM, 5 of whom received laser treatment and obtained a good response. The other patient was treated surgically. After an in-depth review of this type of lesion, we conclude that laser treatment is a safe and effective alternative for these malformations, provided the patients are appropriately diagnosed and have Schöbinger stage i lesions.

Papulosis linfomatoide: hallazgos clínico-patológicos en 18 pacientes / Lymphomatoid papulosis: Clinical and pathological findings in 18 patients

Introducción: La papulosis linfomatoide (PL) es una dermatosis que se engloba dentro de los procesos linfoproliferativos CD30 positivos de la piel. Se ha descrito su asociación a linfoma de Hogking (LH), así como su progresión a micosis fungoide (MF) y linfoma cutáneo anaplásico de célula grande (LCACG). Objetivos: Investigar los hallazgos clínicos, histológicos y la respuesta al tratamiento en un grupo de pacientes con PL. Material y métodos: Se llevó a cabo un estudio retrospectivo, descriptivo y observacional. Se seleccionaron 18 pacientes con diagnóstico histológico confirmado de PL y con un adecuado seguimiento clínico. Se recopilaron los hallazgos histológicos de las biopsias de piel, la forma de presentación, la evolución y la respuesta a los tratamientos utilizados. Resultados: Se reclutaron un total de 18 pacientes, 10 varones y 8 mujeres. La mayoría de las biopsias, 14 de 18 (78%) mostraban un infiltrado linfocitario en cuña, CD30 positivo, CD3 positivo y CD56 negativo. El tipo histológico más frecuente fue el tipo A, presente en un 83% de las biopsias de los pacientes. La forma clínica de presentación más frecuente fue en forma de pápulas en el tronco (83%). Un 62% de los pacientes sufrió un único brote autorresolutivo. La media de seguimiento fue de 7 años, durante los cuales un 12% de los pacientes desarrolló una micosis fungoide, sin encontrarse otras asociaciones. Discusión: Existen pocas series de pacientes con PL publicadas en los últimos años; sin embargo, globalmente los hallazgos descritos en ellas coinciden con las de nuestro grupo de pacientes. Conclusiones: La PL es un cuadro linfoproliferativo típicamente CD30 positivo que habitualmente tiene un curso benigno con buena respuesta a los tratamientos utilizados (AU)

Background: Lymphomatoid papulosis (LyP) is a CD30+ lymphoproliferative skin disease that has been described in association with Hodgkin lymphoma. It has also been reported to progress to mycosis fungoides or cutaneous anaplastic large-cell lymphoma. Objective: To study the clinical and histologic features of LyP and response to treatment in a patient series. Materials and methods: For this retrospective, descriptive, observational study of patients with histologically confirmed LyP and sufficient follow-up data on record, we extracted histologic findings on skin biopsy, clinical presentation, clinical course, and response to treatments. Results: Eighteen patients (10 male, 8 female) were identified. Most biopsies (14/18, 78%) showed a wedge-shaped lymphocytic infiltrate with CD30+, CD3+, and CD56− cells. A type A histologic pattern was present in the biopsies of 83% of the patients. The most common presentation (83%) consisted of papules on the trunk; for 62% LyP resolved after a single episode. Twelve percent of the patients developed mycosis fungoides (mean follow-up, 7 years); no other associations were noted. Discussion: Although few series of patients with LyP have been published in recent years, the findings reported generally coincide with our observations. Conclusion: LyP is typically a CD30+ lymphoproliferative disorder that usually runs a benign course and responds well to treatment (AU)

[Lymphomatoid papulosis: clinical and pathological findings in 18 patients]. / Papulosis linfomatoide: hallazgos clínico-patológicos en 18 pacientes.

BACKGROUND: Lymphomatoid papulosis (LyP) is a CD30(+) lymphoproliferative skin disease that has been described in association with Hodgkin lymphoma. It has also been reported to progress to mycosis fungoides or cutaneous anaplastic large-cell lymphoma. OBJECTIVE: To study the clinical and histologic features of LyP and response to treatment in a patient series. MATERIALS AND METHODS: For this retrospective, descriptive, observational study of patients with histologically confirmed LyP and sufficient follow-up data on record, we extracted histologic findings on skin biopsy, clinical presentation, clinical course, and response to treatments. RESULTS: Eighteen patients (10 male, 8 female) were identified. Most biopsies (14/18, 78%) showed a wedge-shaped lymphocytic infiltrate with CD30(+), CD3(+), and CD56(-) cells. A type A histologic pattern was present in the biopsies of 83% of the patients. The most common presentation (83%) consisted of papules on the trunk; for 62% LyP resolved after a single episode. Twelve percent of the patients developed mycosis fungoides (mean follow-up, 7 years); no other associations were noted. DISCUSSION: Although few series of patients with LyP have been published in recent years, the findings reported generally coincide with our observations. CONCLUSION: LyP is typically a CD30(+) lymphoproliferative disorder that usually runs a benign course and responds well to treatment.

Ameloblastoma desmoplásico / Desmoplastic ameloblastoma

Presentamos un caso de ameloblastoma desmoplásico en una mujer de 32 años que afectaa la región media del maxilar superior. Se describen las características específicas, tantohistológicas como clínicas, de esta variante de ameloblastoma, y se insiste en su imagenradiológica similar a la de una lesión fibroósea benigna. Se indica la misma conducta terapéuticaque para el resto de los ameloblastomas intraóseos sólidos(AU)

We report a case of desmoplastic ameloblastoma in a 32 year-old female patient affectingthe region half of the upper jaw. We describe the specific characteristics, both histologicaland clinical of this variant of ameloblastoma, insisting its radiological image similar to thatof a benign fibroosseous lession. We indicate the same therapeutic conduct for the rest ofthe solid intraosseous ameloblastomas(AU)