Linfoma cutáneo de células B de la zonamarginal y sarcoidosis: presentación simultánea / Cutaneous marginal zone B-cell lymphoma and sarcoidosis: simultaneous presentation

El síndrome sarcoidosis-linfoma es la asociación en un mismo paciente de sarcoidosis y un linfoma. Describimos un paciente de 32 años que acude por tres lesiones nodulares cuya biopsia es diagnóstica de linfoma cutáneo de células B de la zona marginal. Durante el estadiaje se detectaron adenopatías mediastínicas cuya biopsia mostró granulomas no necrotizantes compatibles con sarcoidosis. Este sería el primer caso descrito que asocia linfoma cutáneo B de la zona marginal con sarcoidosis de forma simultánea (AU)

The sarcoidosis-lymphoma syndrome is the coexistence of sarcoidosis and malignant lymphoproliferative disease in the same patient. We describe a 32-year-old man who presented with three cutaneous nodular lesions. Skin biopsies revealed marginal zone cutaneous B-cell lymphoma. Radiologic Studies showed hiliar lymphadenopathy that histologically revealed non-caseating granulomas consistent with sarcoidosis. This report is the first case of simultaneous ocurrente in the same patient of primary cutaneous marginal zone B cell lymphoma and sarcoidosis (AU)

[Initial evaluation, diagnosis, staging, treatment, and follow-up of patients with primary cutaneous malignant melanoma. Consensus statement of the Network of Catalan and Balearic Melanoma Centers]. / Valoración inicial, diagnóstico, estadificación, tratamiento y seguimiento de los pacientes con melanoma maligno primario de la piel. Documento de consenso de la "Xarxa de Centres de Melanoma de Catalunya i Balears"

The consensus statement on the management of primary cutaneous melanoma that we present here was based on selection, discussion, review, and comparison of recent literature (including national and international guidelines). The protocols for the diagnosis, treatment, and follow-up used in the hospital centers throughout Catalonia and the Balearic Isles belonging to the Network of Catalan and Balearic Melanoma Centers were also considered. The main objective of this statement was to present the overall management of melanoma patients typically used in our region at the present time. As such, the statement was not designed to be an obligatory protocol for health professionals caring for this group of patients, and neither can it nor should it be used for this purpose. Professionals reading the statement should not therefore consider it binding on their practice, and in no case can this text be used to guarantee or seek responsibility for a given medical opinion. The group of dermatologists who have signed this statement was created 3 years ago with the aim of making our authorities aware of the importance of this complex tumor, which, in comparison with other types of cancer, we believe does not receive sufficient attention in Spain. In addition, the regular meetings of the group have produced interesting proposals for collaboration in various epidemiological, clinical, and basic applied research projects on the subject of malignant melanoma in our society.

Valoración inicial, diagnóstico, estadificación, tratamiento y seguimiento de los pacientes con melanoma maligno primario de la piel. Documento de consenso de la 'Xarxa de Centres de Melanoma de Catalunya i Balears' / Initial evaluation, diagnosis, staging, treatment, and follow-up of patients with primary cutaneous malignant melanoma. Consensus statement of the network of Catalan and Balearic Melanoma Centers

El documento de consenso respecto al manejo del melanoma primario de la piel, que detallamos a continuación, nace de la puesta en común, aceptación, revisión y confrontación con la literatura reciente (incluyendo guías clínicas nacionales e internacionales), así como de los protocolos de diagnóstico, seguimiento y tratamiento consensuados en los diferentes centros hospitalarios de toda Cataluña y Balerares pertenencientes a la Xarxa de Centres de Melanoma de Catalunya i Balears. El objetivo principal de este documento es exponer de forma conjunta el manejo habitual del paciente con melanoma que actualmente se realiza en nuestro medio. Sin embargo, este documento no pretende, ni puede, por lo que tampoco debiera ser usado como un protocolo de obligado cumplimiento por los profesionales que atendemos a este grupo de enfermos. En este sentido, cabe mencionar que la consulta de este documento por parte del profesional no es vinculante para su acción, y en ningún caso este texto podrá ser utilizado para garantizar o buscar responsabilidades del juicio médico concreto. El grupo de dermatólogos que firman dicho documento se formó hace ahora tres años, con la intención de dar a conocer a nuestras autoridades la importancia de este complejo tumor, que en nuestro país creemos que se encuentra erróneamente infravalorada con respecto a otros tipo de cáncer. Además, fruto de las reuniones periódicas del grupo, han surgido también interesantes propuestas de colaboración en distintos proyectos de investigación epidemiológica, clínica y básica aplicada en torno al melanoma maligno en nuestra sociedad (AU)

The consensus statement on the management of primary cutaneous melanoma that we present here was based on selection, discussion, review, and comparison of recent literature (including national and international guidelines). The protocols for the diagnosis, treatment, and follow-up used in the hospital centers throughout Catalonia and the Balearic Isles belonging to the Network of Catalan and Balearic Melanoma Centers were also considered. The main objective of this statement was to present the overall management of melanoma patients typically used in our region at the present time. As such, the statement was not designed to be an obligatory protocol for health professionals caring for this group of patients, and neither can it nor should it be used for this purpose. Professionals reading the statement should not therefore consider it binding on their practice, and in no case can this text be used to guarantee or seek responsibility for a given medical opinion. The group of dermatologists who have signed this statement was created 3 years ago with the aim of making our authorities aware of the importance of this complex tumor, which, in comparison with other types of cancer, we believe does not receive sufficient attention in Spain. In addition, the regular meetings of the group have produced interesting proposals for collaboration in various epidemiological, clinical, and basic applied research projects on the subject of malignant melanoma in our society (AU)

Ulceraciones orales por alendronato / Mouth Sores Caused by Alendronate

No disponible

No disponible

[Asian tiger mosquito bites: perception of the affected population after Aedes albopictus became established in Spain]. / Picaduras por mosquito tigre. Percepción de la población afectada tras el establecimiento de Aedes albopictus en España.

INTRODUCTION: The presence of Aedes albopictus was detected in Spain in 2004 and it has now become fully established, causing significant discomfort among the population in the affected areas. OBJECTIVES: The aim of this study was to investigate the impact of the arrival of A albopictus and its subsequent establishment on the population a year after being detected in Spain. MATERIAL AND METHODS: A survey questioned 309 users of the Valldoreix-Sant Cugat Healthcare Center about their knowledge of Aedes albopictus, the characteristics of bites by this insect, and their attitude to prevention and treatment. RESULTS: Ninety one percent of respondents knew about the tiger mosquito. Sixty-one percent (66 % of women and 53 % of men) had reported suffering bites attributed to this insect. The most common type of bite was a small swelling (78 %) and the most common site was the legs (93 %). Children had a greater number of lesions and a more generalized distribution. Blistering lesions were more frequent in women. Itching was very intense (65 %), particularly in women (71 %) and children (76 %). The majority of patients (80 %) did not seek attention from their health care services and 36 % consulted their pharmacist. Fifty percent (61 % of women and 47 % of men) treated their bites, mainly with topical corticosteroids (56 %) and antihistaminics (26 %). Forty-six percent of respondents mainly children reported use of insect repellents. CONCLUSION: The arrival of the Asian tiger mosquito has had a major impact on the population, with a lower quality of life and a deterioration in skin health, due to the numerous and irritating bites.

Picaduras por mosquito tigre. Percepción de la población afectada tras el establecimiento de Aedes albopictus en España / Asian tiger mosquito bites: perception of the affected population after aedes albopictus became established in Spain

Introducción. Aedes albopictus se identificó en España en el año 2004 y en la actualidad está plenamente establecido, causando un intenso malestar entre la población de las áreas afectadas. Objetivos. Estudiar el impacto de la llegada y el asentamiento de Aedes albopictus sobre la población un año después de su identificación en nuestra comunidad. Material y métodos. Encuesta a 309 usuarios del CAP Vall d’oreix-Sant Cugat acerca de los conocimientos sobre este mosquito, características de sus picaduras y actitud en cuanto a prevención y tratamiento. Resultados. El 91 % de los encuestados conocían el mosquito tigre. El 61 % referían haber sufrido picaduras (66% mujeres y 53% hombres), que atribuían a este insecto. El tipo de picadura más frecuente fue el habón (78 %) y la localización, las extremidades inferiores (93 %). Los niños mostraban mayor número de lesiones y una distribución más generalizada. Las lesiones ampollosas fueron más frecuentes en mujeres. El prurito fue muy intenso (65 %) sobre todo en mujeres (71 %) y niños (76 %). La mayoría de los pacientes (80 %) no consultó a los servicios médicos y un 36 % lo hizo al farmacéutico. Un 50 % realizó tratamiento (61% mujeres y 47% hombres), principalmente con corticoides tópicos (56 %) y antihistamínicos (26 %). Un 46 % de los encuestados afirmaba usar repelentes, sobre todo niños. Conclusión. La llegada del mosquito tigre ha tenido un gran impacto sobre la población, mermando la calidad de vida y empeorando su salud dermatológica debido a sus numerosas y molestas picaduras (AU)

Introduction. The presence of Aedes albopictus was detected in Spain in 2004 and it has now become fully established, causing significant discomfort among the population in the affected areas. Objectives. The aim of this study was to investigate the impact of the arrival of A albopictus and its subsequent establishment on the population a year after being detected in Spain. Material and methods: A survey questioned 309 users of the Vall d’oreix-Sant Cugat Healthcare Center about their knowledge of Aedes albopictus, the characteristics of bites by this insect, and their attitude to prevention and treatment. Results. Ninety one percent of respondents knew about the tiger mosquito. Sixty-one percent (66% of women and 53% of men) had reported suffering bites attributed to this insect. The most common type of bite was a smalls welling (78%) and the most common site was the legs (93%). Children had a greater number of lesions and amore generalized distribution. Blistering lesions were more frequent in women. Itching was very intense (65%), particularly in women (71%) and children (76%). The majority of patients (80%) did not seek attention from their health care services and 36 % consulted their pharmacist. Fifty percent (61% of women and 47% of men) treated their bites, mainly with topical corticosteroids (56%) and antihistaminics (26%). Forty-six percent of respondents—mainly children—reported use of insect repellents. Conclusion. The arrival of the Asian tiger mosquito has had a major impact on the population, with a lower quality of life and a deterioration in skin health, due to the numerous and irritating bites (AU)

Dermatofibrosarcoma protuberans congénito: descripción de un caso / Congenital dermatofibrosarcoma protuberans: a case report

El dermatofibrosarcoma protuberans es un tumor fibrohistiocitario de bajo grado poco común y que suele aparecer en adultos entre los 20 y 50 años de edad. Inicialmente se presenta como una mácula o placa violácea de aspecto vascular sobre la que aparecen posteriormente lesiones nodulares en la superficie. El diagnóstico es histológico y se trata de un tumor de células fusiformes que se disponen en fascículos cortos adoptando un patrón arremolinado característico. Es un tumor con una elevada tasa de recurrencias locales, aunque son raras las metástasis. El tratamiento es quirúrgico con amplios márgenes. Este tumor excepcionalmente afecta a niños menores de 16 años y todavía más raros son los casos congénitos, con sólo 27 casos descritos en la literatura. Se describe el caso de un niño de 10 años de edad con un dermatofibrosarcoma protuberans presente desde el nacimiento y sin signos de recurrencia en la actualidad

Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern. The local recurrence rate is high but metastases are rare. The treatment is surgical resection with wide margins. The tumor rarely affects children under 16 years of age and it is even less common at birth--only 27 congenital cases have been described in the literature. We describe the case of a 10-year-old boy with dermatofibrosarcoma protuberans present since birth and currently without signs of recurrence

[Congenital dermatofibrosarcoma protuberans: a case report]. / Dermatofibrosarcoma protuberans congénito: descripción de un caso.

Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern. The local recurrence rate is high but metastases are rare. The treatment is surgical resection with wide margins. The tumor rarely affects children under 16 years of age and it is even less common at birth--only 27 congenital cases have been described in the literature. We describe the case of a 10-year-old boy with dermatofibrosarcoma protuberans present since birth and currently without signs of recurrence.

Atrophic dermatofibroma accompanied by aneurysmatic characteristics.

A 40-year-old man presented a painful haemorrhagic plaque on his chest in the same location where a nodular lesion had been presented for many years. After 2 months, the plaque was replaced by a depressed lesion. The lesion diagnosed as an anetoderma was excised and the biopsy showed an atrophic dermatofibroma accompanied by aneurysmatic characteristics.

Sézary syndrome and related variants of classic cutaneous T-cell lymphoma. A descriptive and prognostic clinicopathologic study of 29 cases.

Large series of patients with Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma (CTCL), have been reported infrequently because of its low incidence. Here we recorded several clinical, histopathological and immunophenotypical features of 29 cases of leukemic CTCL patients from four Dermatology Departments of Catalonia, Spain, and analyzed their prognostic value. Clinical data included sex, age, delay of SS diagnosis, previous diagnosis of lymphoma, B-symptoms, type of skin lesions, peripheral adenopathy, histologic evaluation of lymph node biopsy, visceral involvement, percentage of circulating Sézary cells, serum LDH and beta-2-microglobulin levels, first treatment and response, disease-free interval, further therapies and survival. Histopathological data examined were epidermotropism, depth and thickness of the infiltrate, cell size, adnexal involvement, presence of granuloma, eosinophils and plasma cells, mitotic rate. The percentage of CD45Ro, CD43, CD20, CD30 and CD8 positive dermal cells were also recorded. Survival showed a mean actuarial risk of 57% at 3 years and 38% at 5 years, with a median survival of 48 months. Analysis of actuarial survival demonstrated as following as features linked with a bad prognosis: fast evolution of the disease (from symptoms onset up to diagnosis) (p = 0.0274) raised levels of serum lactate dehydrogenase (p = 0.0379) and beta-2-microglobulin (p = 0.0151), the latter being the most important prognostic factor. In conclusion although SS had been traditionally considered as a low-grade lymphoma, the present study agrees with the recent classification rating SS as an aggressive type of CTCL with a poor prognosis. Our results show that some simple clinical and blood test data can be useful as prognostic indicators in this disease.

[Eosinophilic ulcer of the oral mucosa: 11 cases]. / Ulcère éosinophilique de la muqueuse buccale: 11 cas.

BACKGROUND: The eosinophilic ulcer is a lesion of the oral mucosa, that has been infrequently described in the literature. This is a benign and self-limiting lesion of unknown origin. CASE REPORTS: In this article, eleven new cases of eosinophilic ulcer of the oral mucosa are presented. The clinical, histologic and evolutive features are reviewed. DISCUSSION: Recurrent trauma is clearly involved in the pathogenesis of this entity.

Genotypic analysis of cutaneous T-cell lymphoma: a comparative study of Southern blot analysis with polymerase chain reaction amplification of the T-cell receptor-gamma gene.

The diagnosis of early cutaneous T-cell lymphoma (CTCL) is a difficult point in dermatology. Recently, Southern blot analysis (SBA) and polymerase chain reaction (PCR) have been used to detect clonality in initial lesions in which clinical and histological findings are unspecific. Forty-one samples from 25 patients with CTCL were investigated for the presence of T-cell receptor-gamma gene rearrangement using a nested PCR technique and analysed by polyacrylamide gel electrophoresis (PAGE). Conventional SBA was also performed on 28 samples from 20 of these patients. In addition, 20 samples corresponding to patients with large plaque parapsoriasis (LPP), cutaneous B-cell lymphoma (CBCL) and eczema were analysed by PCR in the same way as were the CTCL specimens. Most of the CTCL specimens (81%) showed clonality on PCR analysis. Among patients with mycosis fungoides, 71% of initial patch lesions and 100% of plaques and tumours showed clonal disease. Clonality could be detected in three of four histologically negative post-treatment lesions. Clonal rearrangement was detected in one of three patients with LPP and in three of 10 patients with CBCL. None of the samples corresponding to patients with eczema showed positive results. SBA was significantly less sensitive than PCR in detecting clonality in CTCL patients (42% among early disease and 60% among advanced cases). The results indicate that this PCR/PAGE technique is a reliable and useful method for the detection of clonality in early skin lesions of CTCL patients and probably in the identification of silent extracutaneous involvement.

Changes in T-cell phenotype and adhesion molecules expression in psoriatic lesions after low-dose cyclosporin therapy.

Cyclosporin is a very effective treatment for severe psoriasis, but its exact mechanism of action in this disease is not completely understood. It has been hypothesized that the drug could act through the inhibition of the expression of certain cell adhesion molecules on the keratinocytes prior to the reduction in the number of epidermal inflammatory cells. Several studies have focused on ICAM-1 changes on keratinocytes and endothelial cells after cyclosporin treatment in psoriatic patients but their results have been somewhat contradictory. We examined changes in T-cell markers and adhesion molecules among keratinocytes, endothelial and inflammatory cells after low-dose cyclosporin treatment for severe psoriasis. We performed a histological and immunohistochemical study on psoriatic skin among 10 patients (7 males and 3 females; mean age 37 years) treated with low-dose (2.5 mg/kg/day) cyclosporin, prior to therapy, after 1 month, and after 3 months of treatment. The mean PASI (Psoriasis Area and Severity Index) before treatment was 23 +/- 4, 13 +/- 7 after the first month of therapy, and 8 +/- 2 at the end of the third month of therapy. Pretherapy samples showed a moderate to severe inflammatory infiltrate mainly due to T-lymphocytes expressing a T-cell memory (UCHL-1) and helper/inducer (CD4) phenotype. Most of these cells also expressed HLA-DR and LFA-1 and ICAM-1 antigens. After the treatment, an overall reduction in the degree of epidermal hyperplasia was seen (p = 0.01). The severity of the infiltrate was clearly reduced (p = 0.05), but no significant changes in the phenotype profile were observed. Although slightly reduced, endothelial ICAM-1 expression persisted after cyclosporin therapy. Keratinocyte ICAM-1 expression was uniformly and significantly reduced after 1 month and 3 months of therapy (p = 0.01). These results support the hypothesis that cyclosporin interferes with the expression of keratinocyte adhesion molecules in patients with psoriasis. Servitje O, Bordas X, Serón D, Vidaller A, Moreno A, Curcó N, Sais G, Peyrí J. Changes in T-cell phenotype and adhesion molecules expression in psoriatic lesions after low-dose cyclosporin therapy.

Mycobacterium kansasii infection limited to the skin in a patient with AIDS.

We describe a case of cutaneous Mycobacterium kansasii infection in a 56-year-old man with acquired immunodeficiency syndrome, who received treatment with trimethoprim-sulphamethoxazole for Pneumocystis carinii pneumonia. Resolution of the cutaneous lesion was observed without specific treatment.

Salivary gland lymphoplasmacytoid lymphoma with nodular cutaneous amyloid deposition and lambda chain paraproteinaemia.

We report the case of an 83-year-old woman who developed a lymphoplasmacytoid lymphoma of the salivary glands with extension to the skin of the cheeks and laterocervical area. The most remarkable feature was the massive amyloid deposition that gave the cutaneous lesions a distinctive clinical appearance, similar to that observed in cases of nodular cutaneous amyloidosis. An IgM-lambda paraprotein was also detected in the serum.