Unilateral clubbing-like digital thickening as a clinical manifestation of low-flow vascular malformations: a series of 13 cases.

BACKGROUND: Digital clubbing is a well-known clinical sign characterized by thickening of the distal phalanges of the fingers and toes. Unilateral clubbing occurs less frequently. A previous report showed for the first time two cases of unilateral clubbing as a clinical manifestation of lower limb venous malformation. The objective of the present study is to describe a series of 13 patients with a low-flow vascular malformation where a clubbing-like unilateral digital thickening is also observed. METHODS: All patients were retrospectively included after reviewing clinical photographs from a vascular malformations database. RESULTS: A total of 13 patients with low-flow vascular malformations were included in this study. The mean age at diagnosis was 11 years (range 5-26 years) with a female predominance (nine patients). The most frequent vascular malformation collected was a blue rubber bleb nevus syndrome in four patients, followed by common venous malformations in three patients. All patients characteristically exhibited a clubbing-like digital thickening. Seven patients had foot involvement and six patients hand involvement. CONCLUSIONS: Although the number of cases is limited, our study is the first series of cases where a clubbing-like digital thickening is described in patients with a low-flow vascular malformation. The unilateral presence of clubbing or pseudoclubbing should lead to the suspicion of an underlying vascular malformation.

Estado actual de la atención multidisciplinar para pacientes con artritis psoriásica en España: proyecto NEXUS 2.0 / Current status of multidisciplinary care in psoriatic arthritis in Spain: NEXUS 2.0 project

OBJETIVO: 1) Analizar la implementación de los modelos de atención multidisciplinar en pacientes con artritis psoriásica (APs), y 2) definir estándares de calidad de mínimos y de excelencia. MÉTODOS: Se envió una encuesta a profesionales que ya realizan atención multidisciplinar o están en vías preguntando por: 1) tipo de modelo de abordaje multidisciplinar, y 2) grado, prioridad y facilidad de la implementación de los estándares de calidad de estructura, proceso y resultado. En 6 reuniones regionales se presentaron y discutieron los resultados de la encuesta, tanto a nivel nacional como regional, y se definió la prioridad definitiva de los estándares de calidad. En una reunión de grupo nominal, 11 expertos (reumatólogos y dermatólogos) analizaron los resultados de la encuesta y las reuniones regionales. Con ello definieron qué estándares de calidad son actualmente de mínimos y cuáles de excelencia. RESULTADOS: Los modelos de atención multidisciplinar conjunto y paralelo son los más implementados, y los de los estándares de calidad es muy variable: en los de estructura varía del 22 al 74%, en los de proceso del 17 al 54% y en los de resultado del 2 al 28%. De los 25 estándares de calidad originales, 9 se consideran solo de mínimos, 4 de excelencia y 12 tienen definidos unos criterios para ser de mínimos y otros para la excelencia. CONCLUSIONES: La definición de estándares de calidad de mínimos y de excelencia ayudará en la consecución del objetivo de la atención multidisciplinar para pacientes con APs, que es la mejor asistencia sanitaria posible

OBJECTIVE: 1) To analyze the implementation of multidisciplinary care models in psoriatic arthritis (PsA) patients, 2) To define minimum and excellent standards of care. METHODS: A survey was sent to clinicians who already performed multidisciplinary care or were in the process of undertaking it, asking: 1) Type of multidisciplinary care model implemented; 2) Degree, priority and feasibility of the implementation of quality standards in the structure, process and result for care. In 6 regional meetings the results of the survey were presented and discussed, and the ultimate priority of quality standards for care was defined. At a nominal meeting group, 11 experts (rheumatologists and dermatologists) analyzed the results of the survey and the regional meetings. With this information, they defined which standards of care are currently considered as minimum and which are excellent. RESULTS: The simultaneous and parallel models of multidisciplinary care are those most widely implemented, but the implementation of quality standards is highly variable. In terms of structure it ranges from 22% to 74%, in those related to process from 17% to 54% and in the results from 2% to 28%. Of the 25 original quality standards for care, 9 were considered only minimum, 4 were excellent and 12 defined criteria for minimum level and others for excellence. CONCLUSIONS: The definition of minimum and excellent quality standards for care will help achieve the goal of multidisciplinary care for patients with PAs, which is the best healthcare possible

Current status of multidisciplinary care in psoriatic arthritis in Spain: NEXUS 2.0 project. / Estado actual de la atención multidisciplinar para pacientes con artritis psoriásica en España: proyecto NEXUS 2.0.

OBJECTIVE: 1) To analyze the implementation of multidisciplinary care models in psoriatic arthritis (PsA) patients, 2) To define minimum and excellent standards of care. METHODS: A survey was sent to clinicians who already performed multidisciplinary care or were in the process of undertaking it, asking: 1) Type of multidisciplinary care model implemented; 2) Degree, priority and feasibility of the implementation of quality standards in the structure, process and result for care. In 6 regional meetings the results of the survey were presented and discussed, and the ultimate priority of quality standards for care was defined. At a nominal meeting group, 11 experts (rheumatologists and dermatologists) analyzed the results of the survey and the regional meetings. With this information, they defined which standards of care are currently considered as minimum and which are excellent. RESULTS: The simultaneous and parallel models of multidisciplinary care are those most widely implemented, but the implementation of quality standards is highly variable. In terms of structure it ranges from 22% to 74%, in those related to process from 17% to 54% and in the results from 2% to 28%. Of the 25 original quality standards for care, 9 were considered only minimum, 4 were excellent and 12 defined criteria for minimum level and others for excellence. CONCLUSIONS: The definition of minimum and excellent quality standards for care will help achieve the goal of multidisciplinary care for patients with PAs, which is the best healthcare possible.

Expression patterns of aquaporin 1 in vascular tumours.

BACKGROUND: Aquaporins (AQPs) are a family of water channels expressed in various body tissues. Beyond osmotic balance, AQPs have recently been confirmed to be involved in processes related to cancer (tumour proliferation, angiogenesis, etc.). OBJECTIVES: To analyse the presence of these proteins in the endothelium of several vascular tumours, both benign and malignant, in order to establish whether AQPs may be used as a marker or future therapeutic target. MATERIALS AND METHODS: We studied AQP1 expression in 39 patients with vascular tumours, classified into six groups according to ISSVA classification: haemangiomas, benign vascular tumours different from infantile haemangiomas, angiosarcomas, classic Kaposi's sarcoma (KS), and epidemic KS. RESULTS: AQP1 expression was present in 28 of 39 patients, representing 92.9% benign lesions, whereas no expression was found in 72% of malignant lesions. AQP1 expression was associated with benign lesions with an OR of 34.5 (95% CI: 5-250); p<0.0005, and was most frequently identified with a focal endothelial pattern (38%). A kappa index of 0.823 (95% CI: 0.678-0.971) was determined regarding the patterns of expression overall. CONCLUSION: The expression of AQP1 was greater in benign lesions than malignant lesions and this difference was statistically significant, thus AQP1 expression could serve as a marker for benignity of vascular tumours. In addition, the expression pattern of AQP1 was different according to the type of vascular tumour.

Oral Nadolol for Children with Infantile Hemangiomas and Sleep Disturbances with Oral Propranolol.

BACKGROUND/OBJECTIVE: Oral propranolol has been shown to be safe and effective in infants with infantile hemangioma (IH). Side effects such as sleep disturbances have been associated with propranolol. The aim of this study was to evaluate the efficacy and safety of oral nadolol in a small series of patients whose propranolol therapy was discontinued due to sleep disturbances. METHODS: A retrospective study of patients with IHs who were treated with oral nadolol due to propranolol-related sleep disturbances at a pediatric tertiary care center between July 2008 and March 2013. Clinical response to oral nadolol and disappearance of propranolol-related side effects were analyzed. RESULTS: A total of 97 patients presenting IH received oral propranolol. Nine patients (9.3%) developed sleep disturbances. Oral propranolol was discontinued in seven patients and switched to oral nadolol, with resolution of these side effects in 5 (71%) of the cases. One patient developed sleep disturbances again after four months of oral nadolol. LIMITATIONS: The sample size was too small to draw generalizable conclusions and to draw any statistical inference as to the incidence of sleep disturbances with nadolol therapy. CONCLUSIONS: The use of oral nadolol in the treatment of IH in our series of 7 patients, resolved the propranolol-related sleep disturbances in 5 (71%), while in one patient the symptoms recurred after 4 months of oral nadolol at a dose of 2 mg/kg/day. In most cases, switching beta-blockers did not compromise efficacy, and is recommended when sleep disturbance necessitates discontinuation of beta-blocker therapy of IH.

Matricoma melanocítico: hallazgos clinicopatológicos e inmunohistoquímicos. Presentación de un caso y revisión de la literatura / Clinicopathological and immunohistochemical findings in melanocytic matricoma. A case report and a review of the literature

El matricoma melanocítico es una neoplasia cutánea inusual descrita recientemente. Clínicamente se presenta como una lesión papulo-nodular pigmentada en personas añosas, localizada en zonas con daño solar crónico. Histológicamente se caracteriza por una proliferación celular bifásica constituida por células dendríticas melanocíticas y células epiteliales de hábito basalioide con áreas focales de atipia citológica y figuras mitóticas dispersas entremezcladas con células en sombra. El estudio inmunohistoquímico muestra positividad del componente basalioide para -catenina y focal para citoqueratinas AE1/AE3, mientras que las células dendríticas melanocíticas son positivas para Melan A, HMB45 y S-100. Presentamos los hallazgos clinicopatológicos, inmunohistoquímicos, diagnóstico diferencial y revisión de la literatura de un nuevo caso recientemente diagnosticado en nuestro servicio. En la actualidad existen aproximadamente 12 casos publicados en la literatura (AU)

The matricoma melanoma is a rare, recently described cutaneous neoplasm. It appears in elderly patients as a papulo-nodular pigmented lesion in areas with long standing exposure to the sun. The histopathological characteristics are a biphasic proliferation of both dendritic melanocytes and basaloid epithelial cells with varying nuclear atypia and mitotic activity, and numerous interspersed shadow cells. Immunohistochemical studies for -catenin highlighted the morphology of the basaloid cells and focal expression of cytokeratins AE1/AE3, while studies for Melan A, HMB-45 and S-100 protein confirmed the dendritic melanocyte component. The clinicopathological and immunohistochemical findings and differential diagnosis of a new case are described, together with a review of the literature. Approximately only 12 cases have been previously reported(AU)

Primary cutaneous cryptococcosis of the penis.

Primary cutaneous cryptococcosis is characterized by skin lesions confined to one body region, without evidence of simultaneous dissemination. Skin lesions frequently occur in immunocompromised patients. We report a case of primary cutaneous cryptococcosis in an immunocompetent patient affecting genital area successfully treated with oral itraconazole.