Dermatitis granulomatosa postherpética en un paciente tratado con nivolumab / Postherpetic Granulomatous Dermatitis in a Man Treated With Nivolumab

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Granuloma elastolítico anular de células gigantes en área no fotoexpuesta con respuesta a dapsona / Annular Elastolytic Giant Cell Granuloma in Sun-Protected Sites Responds to Dapsone

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Angioleiomioma cutáneo / Cutaneous angioleiomyoma

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Aportación de la tomografia por emisión de fotón Simple/tomografía computadorizada a la biopsia selectiva del ganglio centinela en pacientes diagnosticados de melanoma de cabeza y cuello / Role of SPECT-CT in sentinel lymph node biopsy in patients diagnosed with head and neck melanoma

Objetivo. Valorar la aportación de la SPECT-TC a la biopsia selectiva del ganglio centinela (GC) en la correcta localización anatómica de este, en pacientes diagnosticados de melanoma cutáneo de cabeza y cuello. Material y métodos. Estudio retrospectivo entre febrero de 2010 y junio de 2013 que incluyó 22 pacientes consecutivos diagnosticados de melanoma cutáneo de cabeza y cuello (9 mujeres y 13 hombres), con una edad media de 55 años y criterios de inclusión de biopsia selectiva del GC. A todos ellos se les realizó una linfogammagrafía preoperatoria tras la inyección pericicatricial de nanocoloides de albúmina marcados con 99mTc, y posteriormente se obtuvieron imágenes planares sectoriales del lugar de inyección y de cuerpo completo, así como SPECT-TC. Resultados. La tasa de detección del GC fue del 91% (20 de 22 pacientes) para la linfogammagrafía planar y del 95,4% (21 de 22 pacientes) para SPECT-TC. En 14 de 22 pacientes la SPECT-TC mostró información relevante sobre la localización del GC modificando la vía de abordaje quirúrgica, siendo el impacto clínico de un 63,6%. En un 9,1% de los pacientes el GC fue positivo para metástasis de melanoma. Conclusión. La SPECT-TC proporciona información anatómica relevante sobre la localización del GC y detecta un mayor número de ganglios linfáticos que la linfogammagrafía. Se recomienda el uso rutinario de SPECT-TC en la linfogammagrafía del melanoma de cabeza y cuello para optimizar la localización y el número de GC en esta área (AU)

Objective. Assess the role of SPECT-CT in sentinel lymph node (SLN) biopsy in the accurate anatomical location of the SNL in patients with cutaneous head and neck melanoma. Material and methods. A retrospective study was conducted from February 2010 to June 2013 on 22 consecutive patients with a diagnosis of cutaneous head and neck melanoma (9 female, 13 male), with a mean age of 55 years old and who met the inclusion criteria for SLN biopsy. Patients underwent preoperative scanning after peri-scar injection of 99mTc-labeled-nanocolloid. Planar images of the injection-site, whole-body, and SPECT-CT scanning were acquired. Results. Detection rate of SLN reached up to 91% (20/22 patients) by planar lymphoscintigraphy and 95.4% (21/22 patients) by SPECT-CT. SPECT-CT provided an accurate location of SLN in 14/22 patients, enabling to improve the surgical approach (clinical impact: 63.6%). SLN was positive for metastatic cells in 9.1% patients. Conclusion. SPECT-CT provides detailed anatomical SLN location and allows detecting a higher number of SLN than planar lymphoscintigraphy. Routine use of SPECT-CT is recommended in order to optimise the SLN detection and location in patients with head and neck melanoma (AU)

Role of SPECT-CT in sentinel lymph node biopsy in patients diagnosed with head and neck melanoma.

OBJECTIVE: Assess the role of SPECT-CT in sentinel lymph node (SLN) biopsy in the accurate anatomical location of the SNL in patients with cutaneous head and neck melanoma. MATERIAL AND METHODS: A retrospective study was conducted from February 2010 to June 2013 on 22 consecutive patients with a diagnosis of cutaneous head and neck melanoma (9 female, 13 male), with a mean age of 55 years old and who met the inclusion criteria for SLN biopsy. Patients underwent preoperative scanning after peri-scar injection of (99m)Tc-labeled-nanocolloid. Planar images of the injection-site, whole-body, and SPECT-CT scanning were acquired. RESULTS: Detection rate of SLN reached up to 91% (20/22 patients) by planar lymphoscintigraphy and 95.4% (21/22 patients) by SPECT-CT. SPECT-CT provided an accurate location of SLN in 14/22 patients, enabling to improve the surgical approach (clinical impact: 63.6%). SLN was positive for metastatic cells in 9.1% patients. CONCLUSION: SPECT-CT provides detailed anatomical SLN location and allows detecting a higher number of SLN than planar lymphoscintigraphy. Routine use of SPECT-CT is recommended in order to optimise the SLN detection and location in patients with head and neck melanoma.

Nodular regenerative hyperplasia of the liver and cutaneous T-cell lymphoma: an unreported association.

Nodular regenerative hyperplasia of the liver -a type of noncirrhotic portal hypertension- is a rare condition of unknown etiopathogenesis that has been associated with multiple disorders, including diverse types of hematologic disease. We report the case of a 36-year-old female patient diagnosed with cutaneous T-cell lymphoma of the mycosis fungoides variety, staged as T2N0M0B0, where a transjugular liver biopsy demonstrated the presence of nodular regenerative hyperplasia with a hepatic venous pressure gradient of 15 mm Hg. The study was triggered by the incidental radiologic finding of hepatomegaly with indirect evidence of portal hypertension. We are not aware of any previous reports on the association of nodular regenerative hyperplasia with mycosis fungoides in the medical literature.

Urticaria vasculitis: estudio retrospectivo de 15 casos / Urticarial Vasculitis: A Retrospective Study of 15 Cases

Introducción: La urticaria vasculitis (UV) es un subtipo de vasculitis caracterizada clínicamente por lesiones de tipo urticarial e histológicamente por vasculitis necrotizante. Objetivo: El objetivo de este trabajo es el estudio de los pacientes con urticaria vasculitis que han sido atendidos en el Servicio de Dermatología del Hospital Universitario Virgen del Rocío (Sevilla, España) para determinar las características de presentación clínicas e histológicas, así como el despistaje de hipocomplementemia, en estrecha relación con enfermedad sistémica asociada. Material y métodos: Se han extraído de la base de datos de nuestro servicio los casos de urticaria vasculitis confirmados histológicamente durante un período de 10 años. Resultados: Quince pacientes fueron diagnosticados de urticaria vasculitis (9 mujeres y 6 hombres, con una mediana de edad de 51 años). En 14 pacientes (93%) las lesiones persistían más de 24 horas. En 9 pacientes (60%) las lesiones se resolvían con púrpura e hiperpigmentación residual. Siete pacientes (47%) presentaron niveles disminuidos de complemento en sangre, 12 pacientes (80%) asociaron síntomas extracutáneos y 8 pacientes (53%) asociaban enfermedad sistémica, siendo la entidad más frecuente el lupus eritematoso sistémico. Conclusiones: La urticaria vasculitis constituye a menudo una entidad infradiagnosticada, con una variable respuesta al tratamiento y en la que la existencia de niveles de complemento disminuidos y de síntomas extracutáneos puede ser indicativa de enfermedad sistémica asociada (AU)

Introduction: Urticarial vasculitis is a subtype of vasculitis characterized clinically by urticarial lesions and histologically by necrotizing vasculitis. Objective: To study the clinical and histologic features of urticarial vasculitis in patients seen in the dermatology department of Hospital Universitario Virgen de Rocío in Seville, Spain, and to examine the association between hypocomplementemia and systemic disease. Material and methods: We performed a chart review of histologically confirmed cases of urticarial vasculitis in the database of our department covering a period of 10 years. Results: Fifteen patients (9 women and 6 men with a median age of 51 years) were included. In 14 patients (93%), the lesions persisted for more than 24 hours, and in 9 cases (60%) the lesions resolved leaving residual purpura or hyperpigmentation. Seven patients (47%) had low complement levels in the blood, 12 (80%) had extracutaneous symptoms, and 8 (53%) had associated systemic disease, the most common of which was systemic lupus erythematosus. Conclusions: Urticarial vasculitis may be underdiagnosed. Response to treatment is variable, and hypocomplementemia and extracutaneous symptoms may indicate the presence of associated systemic disease (AU)

Estudio de inestabilidad de microsatélites y genético de los pacientes con síndrome de Muir-Torre / Microsatellite and Genetic Instability in Patients With Muir-Torre Syndrome

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Urticarial vasculitis: a retrospective study of 15 cases.

INTRODUCTION: Urticarial vasculitis is a subtype of vasculitis characterized clinically by urticarial lesions and histologically by necrotizing vasculitis. OBJECTIVE: To study the clinical and histologic features of urticarial vasculitis in patients seen in the dermatology department of Hospital Universitario Virgen de Rocío in Seville, Spain, and to examine the association between hypocomplementemia and systemic disease. MATERIAL AND METHODS: We performed a chart review of histologically confirmed cases of urticarial vasculitis in the database of our department covering a period of 10 years. RESULTS: Fifteen patients (9 women and 6 men with a median age of 51 years) were included. In 14 patients (93%), the lesions persisted for more than 24hours, and in 9 cases (60%) the lesions resolved leaving residual purpura or hyperpigmentation. Seven patients (47%) had low complement levels in the blood, 12 (80%) had extracutaneous symptoms, and 8 (53%) had associated systemic disease, the most common of which was systemic lupus erythematosus. CONCLUSIONS: Urticarial vasculitis may be underdiagnosed. Response to treatment is variable, and hypocomplementemia and extracutaneous symptoms may indicate the presence of associated systemic disease.

Xantogranuloma juvenil diseminado del adulto asociado a linfoma folicular, respuesta completa a quimioterapia y rituximab. Revisión de la literatura / Disseminated Juvenile Xanthogranuloma Associated With Follicular Lymphoma in an Adult: Successful Treatment With Chemotherapy and Rituximab. A Review of the Literature

El xantogranuloma juvenil es una histiocitosis de células no Langerhans que acontece en la edad infantil; sin embargo, se han descrito varios casos en el adulto, algunos de ellos en relación con hemopatías malignas. Presentamos el caso de una mujer de 61 años de edad con lesiones diseminadas de xantogranuloma juvenil que a los 4 años de seguimiento desarrolló un linfoma de tipo folicular. Tras 6 meses de tratamiento con quimioterapia y rituximab se consiguió remisión del linfoma y la involución de las lesiones cutáneas. Destacamos este caso por tratarse de una entidad poco frecuente y de difícil diagnóstico en el adulto, así como por ser el primer caso asociado con linfoma folicular y que ha presentado una excelente respuesta con quimioterapia y rituximab. Además, dada su posible asociación con enfermedades hematológicas, el xantogranuloma juvenil podría representar una manifestación de una neoplasia oculta (AU)

Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis that typically affects children, but several cases have been reported in adults, some in connection with hematologic malignancies. We present the case of a 61-year-old woman with multiple xanthogranulomas who developed a follicular lymphoma after 4 years of follow-up. After 6 months of treatment with chemotherapy and rituximab, the cutaneous lesions disappeared and the patient achieved remission from lymphoma. We highlight this case because xanthogranuloma is a rare disorder that is difficult to diagnose in adults and also because this is the first report of an association between xanthogranuloma and follicular lymphoma. Excellent response was achieved with chemotherapy and rituximab. Finally, given the possible association between xanthogranulomas and hematologic diseases, these lesions may be a cutaneous manifestation of an occult malignancy (AU)

Disseminated juvenile xanthogranuloma associated with follicular lymphoma in an adult: successful treatment with chemotherapy and rituximab. A review of the literature.

Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis that typically affects children, but several cases have been reported in adults, some in connection with hematologic malignancies. We present the case of a 61-year-old woman with multiple xanthogranulomas who developed a follicular lymphoma after 4 years of follow-up. After 6 months of treatment with chemotherapy and rituximab, the cutaneous lesions disappeared and the patient achieved remission from lymphoma. We highlight this case because xanthogranuloma is a rare disorder that is difficult to diagnose in adults and also because this is the first report of an association between xanthogranuloma and follicular lymphoma. Excellent response was achieved with chemotherapy and rituximab. Finally, given the possible association between xanthogranulomas and hematologic diseases, these lesions may be a cutaneous manifestation of an occult malignancy.

Elastosis perforante serpiginosa / Elastosis perforans serpinginosa

La Elastosis perforante serpiginosa es una rara dermatosis crónica caracterizada por eliminación de fibras elásticas alteradas, a través de la epidermis. A menudo aparece asociada con distintos trastornos del tejido conectivo y puede desarrollarse después de un tratamiento con penicilamina. Sin embargo, también pueden presentarse de forma idiopática. No se conoce el mecanismo por el que se modifican las fibras elásticas y, lamentablemente, no hay ningún tratamiento para esta enfermedad que haya demostrado evidencia. Describimos un caso de elastosis idiopática perforante serpiginosa en placa, tratada de forma satisfactoria con láser de CO2 (AU)

Elastosis perforans serpiginosa is a rare chronic dermatosis characterized by extrusion of altered elastic fibers through the epidermis. It often occurs in association with a variety of connective tissue disorders, and may develop following penicillamine therapy; however, it may also present without c morbidities. The mechanism by which the elastic fibers are altered is not known and, unfortunately, there are no evidence-based treatments for this disease. We describe one patient with idiopathic elastosis perforans serpiginosa in patch pattern, which was successfully treated with CO2laser (AU)