Mácula telangiectásica como forma de presentación de un tumor glómico solitario / Solitary Glomus Tumor Presenting as a Telangiectatic Macule

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Papulosis bowenoide lineal / Linear Bowenoid Papulosis

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Pitiriasis versicolor atrófica / Atrophying Pityriasis Versicolor

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Nevus azul subungueal / Subungual Blue Nevus

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Síndrome de Muir-Torre / Muir-Torre syndrome

El síndrome de Muir-Torre es una genodermatosis de herencia autonómica dominante. Se asocian múltiples tumores viscerales de bajo grado, normalmentecarcinomas colorrectales, con tumores sebáceos de la piel. Representa la expresión fenotípica del cáncer de colon familiar no asociado a poliposis.El síndrome de Muir-Torre podría ser el resultado de una mutación en los genes responsables de la reparación del ADN e inestabilidad de microsatélites.Describimos 2 casos de síndrome de Muir-Torre y mostramos el análisis inmunohistoquímico de las neoplasias sebáceas (AU)

The Muir-Torre syndrome is an autosomal dominant inherited genodermatosis associating multiple low-grade visceral tumors, namely colorectal carcinomas,with sebaceous tumors of the skin. It is a phenotypic subset of hereditary nonpolyposis colorectal cancer. The defect is thought tobe the resultof a mutation in mismatch repair genes and associated with microsatelite instability. We report two cases of Muir-Torre syndrome and show immunohistochemicalanalysis of the sebaceous neoplasm (AU)

Psoriasis tras herpes zóster: respuesta isotópica de Wolf / Psoriasis at the site of healed Herpes Zoster: Wolf’s isotopic response

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[A brain tissue bank in a neuropathology laboratory. Basic methodology]. / Banco de tejidos Neurológicos en el Laboratorio de Neuropatología. Organización básica.

The Meixoeiro Hospital Brain Bank (BB) was established at the end of 2002. A BB is a tissue collection and storage system, established under the best conditions to carry out prospective morphological, biochemical or molecular studies. The BB should ideally be supported by a donor program, although samples may also be obtained from autopsy material from patients with neurodegenerative diseases. Recruitment of control cases from brains without neurological diseases is basic. The main goal of a BB is to provide brain tissue for research. Each case requires accurate clinical data, a definite diagnosis and optimal conditions of tissue preservation. The use of protocols to standardize the handling and processing of tissues, data recruitment and neuropathological diagnosis is fundamental to assure the quality and homogeneity of samples. Close collaboration between neuropathologists, neurologists and other specialists is essential in all the process. Although important advances in the tissue banking field have been achieved, the number of donors in Spain still remains low. Stronger institutional support as well as public awareness through better diffusion of the information is necessary to increase the number of donors and improve BB development.

Banco de Tejidos Neurológicos en el Laboratorio de Neuropatología. Organización básica / A Brain Bank in a Neuropathology Laboratory. Basic methodology

El Banco de Tejidos Neurológicos (BTN) del Hospital Meixoeiro se implantó a finales del año 2002. Un BTN es un sistema prospectivo de almacenamiento y conservación de tejidos en condiciones óptimas que permitan la realización de cualquier estudio morfológico, bioquímico o molecular. Su funcionamiento debe estar basado en un programa de donación, aunque las muestras pueden proceder tanto de donantes como de autopsias clínicas de pacientes con enfermedades neurodegenerativas. Es importante la obtención de cerebros de personas sin enfermedad neurológica para casos control. El objetivo principal de un BTN es el de proporcionar tejido nervioso para la investigación. Los casos deben tener una buena información clínica, un diagnóstico definitivo y unas condiciones de conservación idóneas. Para asegurar la calidad y homogeneidad de las muestras hay que estandarizar, mediante protocolos, los métodos de obtención, extracción y procesamiento del tejido, la recogida de la información y el diagnóstico neuropatológico. La colaboración multidisciplinar entre neuropatólogos, neurólogos y otros especialistas es básica en todo el proceso. Aunque se están realizando grandes avances en el campo de los Bancos Tisulares, el número de donaciones en España es aún muy bajo. Para estimular las donaciones y potenciar el desarrollo de los BTN, hay que conseguir un mayor apoyo institucional y una mayor concienciación de la sociedad mejorando la información (AU)

[Intestinal metaplasia at the esophagogastric junction. Prevelence and association in patients undergoing endoscopy]. / Metaplasia intestinal en la unión esofagogástrica. Prevalencia y asociaciones en una población sometida a endoscopia.

AIM: Short segments of intestinal metaplasia (IM) at the esophagogastric junction is an unclear entity. Several studies have reported wide variations in its prevalence and in the factors associated with its development. Recently, this entity has been divided into esophageal IM and cardiac IM, as two different lesions in etiopathogenesis and prognosis. We studied the prevalence of these conditions and their association with gastroesophageal reflux disease (GERD) and Helicobacter pylori infection. METHODS: In 161 patients, biopsies were obtained from the distal esophagus (2), just below the Z line (3), and in the gastric antrum (4). IM was diagnosed on the basis of staining of goblet cells with Alcian blue and was classified as esophageal if ILZ < IEG or cardiac ILZ = IEG. H. pylori was determined by rapid urease (CLO-test) and histology. Diagnosis of GERD was based on typical symptoms, endoscopy, and histology. In 54 patients with IM (73%) esophageal manometry and 24-hour pH-metry was also performed. RESULTS: IM was detected in 74 patients (46%); IM was esophageal in 33 patients (20.5%) and cardiac in 41 patients (25.4%). Patients with IM were significantly older than those without (p = 0.007) and took proton pump inhibitors more frequently (p = 0.004). No correlation was found between reflux symptoms, esophageal lesions or histological changes with either type of IM. No differences between esophageal or cardiac IM were detected by esophageal pH-metry. H. pylori infection was unrelated to cardiac IM, but these patients had a lower frequency of endoscopic and histological changes in the distal esophagus. CONCLUSIONS: Intestinal metaplasia is a common finding in patients sent for gastroscopy and is probably an acquired lesion that increases in prevalence with age. We found no associations between esophageal IM and GERD, evaluated by typical symptoms, endoscopic and histological changes and pH-metry. H. pylori infection showed no relation to cardiac IM.

Lipomatosis simétrica múltiple: Aportaciónde un caso y revisión de la literatura / Multiple symmetrical lipomatosis. report of a case and review of the literature

La lipomatosis simétrica múltiple es una enfermedad que se caracteriza por presentar múltiples y simétricas masas de tejido adiposo no encapsulado, indoloras y localizadas frecuentemente en cuello, hombros y otras partes del tronco. Habitualmente se asocia a alcoholismo y trastornos metabólicos, y puede cursar con afectación mediastínica y neuropatía. Presentamos el caso de un paciente de 63 años de edad, con importante hábito enólico. Se describen las características clínico-morfológicas, los hallazgos histopatológicos de una biopsia de grasa subcutánea y músculo esquelético, además de otras pruebas complementarias y los posibles tratamientos electivos. Se incluye, además, una amplia revisión de la literatura sobre esta patología (AU)

Lipoid proteinosis. A biochemical and ultrastructural investigation of two new cases.

Lipoid proteinosis is a rare autosomal recessive disease characterized by cutaneous and visceral lesions, in which large amounts of amorphous material are constantly found in stroma. Morphological and biochemical studies indicate abnormal collagen production, but little attention has been paid to the lipid component of lesions. Microscopic and ultrastructural studies of skin, with special emphasis on fibroblasts, vessels, nerve endings and eccrine sweat glands, were conducted in two patients with lipoid proteinosis. Biochemical studies were undertaken in cultured fibroblasts. Evidence of lysosomal storage in epithelial cells of eccrine sweat glands and in dermal histiocytes, very similar to that found in some metabolic disorders, particularly Farber disease, was found in both cases. Our findings suggest that two alterations might coexist in lipoid proteinosis, one characterized by impaired normal collagen production and the other related to a metabolic defect which may lead to accumulation of ceramide or more complex lipids.