Childhood hypopigmented mycosis fungoides: a commonly delayed diagnosis

Primary cutaneous lymphomas (PCLs) are exceedingly rare in children and adolescents, with mycosis fungoides (MF) being the most frequent PCL diagnosed in childhood. There are numerous unusual clinical variants of MF, including the hypopigmented type form (HMF). HMF is exceptional overall, but comparatively common among children. We present an 8-year-old boy with a 3-year history of progressive, generalised, scaly, hypopigmented round patches and few erythematous papules. He was first diagnosed with pityriasis alba (PA), and moisturisers were prescribed with no improvement. Skin biopsy showed typical features of MF, and the patient was successfully treated with narrowband ultraviolet B. HMF may simulate atopic dermatitis, PA, pityriasis lichenoides, tinea versicolour, vitiligo, postinflammatory hypopigmentation or leprosy. Therefore, persistent and unusual hypopigmented lesions should be biopsied to rule out this rare variant of MF

Paraneoplastic pemphigus with clinical features of lichen planus associated with low-grade B cell lymphoma

BACKGROUND: Neoplasia-induced lichen planus is described as a cell-mediated reaction to unknown epithelial antigens. Paraneoplastic pemphigus (PNP), characterized by the presence of a specific array of autoantibodies, probably represents a different form of presentation of the same autoimmune syndrome where the mucocutaneous expression depends on the dominant pathologic mechanism. METHODS: The authors report a case of PNP with predominant lichen planus-like lesions and review the relevant literature. We observed a 74-year-old female with vesico-bullous, erosive, target-shaped and flat papular lichenoid lesions on the lower legs, palms and soles, evolving for 3 weeks. Histopathology revealed a lichenoid dermatitis. Direct immunofluorescence showed C3 deposition around keratinocytes and epidermal IgG intranuclear deposition. Indirect immunofluorescence revealed circulating IgG with intercellular staining on rat bladder substrate. Immunoblotting demonstrated bands of 130, 190, 210 and 250 kDa antigens. A pararenal B cell lymphoma was found. RESULTS: Oral corticotherapy with 40 mg prednisolone daily was initiated with a good cutaneous response. Four months later, cyclophosphamide (50 mg/day) was introduced because of a discrete enlargement of the pararenal mass. The patient died on the seventh month of follow up as a result of respiratory insufficiency. CONCLUSION: PNP has different forms of presentation and the lack of a consensus about diagnostic criteria may contribute to underdiagnosed cases. Advances on the knowledge of the sensitivity and specificity of diagnostic criteria have allowed a better accuracy of diagnosis

Unilateral progressive osseous heteroplasia

A 50-year-old male patient presented with firm subcutaneous nodules and plaques with a gritty texture, unilaterally affecting the left side of the trunk and the left limbs. These lesions had had a progressive course since early childhood and caused functional impairment. There was no family history of similar disorders. No phospho-calcium metabolism abnormalities were observed. Biopsies of the affected areas demonstrated osteoma cutis. Analysis of DNA showed no mutation of the GNAS gene. The clinical features were consistent with progressive osseous heteroplasia, atypically presented in a unilateral form, probably revealing a mosaic distribution

La estancia en bibliotecas extranjeras como instrumento de formación bibliotecaria: la experiencia de la Universidad de Navarra (2003-2010)

En el entorno actual de continuos cambios tecnológicos y de profundas modificaciones en la docencia e investigación, es necesaria una actualización constante. La Biblioteca de la Universidad de Navarra (en adelante UN) inició un programa de estancias de profesionales en bibliotecas extranjeras del Reino Unido y de los Estados Unidos en el año 2003, que continúa en vigor. En el artículo se exponen los resultados de la encuesta llevada a cabo entre los participantes del programa y se describen las principales características del programa, tanto desde el punto de vista de su puesta en práctica, como de su financiación. Asimismo, se señalan las ventajas de esas estancias para el desarrollo profesional de los bibliotecarios y se detallan las principales mejoras aplicadas en la Biblioteca de la UN. La adecuada respuesta de las bibliotecas receptoras y la continuidad y regularidad del programa son decisivos para obtener el mayor provecho de las estancias.-------------------------------------------The changing technological environment and the new learning and research framework require constant updating. The University of Navarra (UN) Library established a professional exchange program in 2003 (still on-going). The results of a survey completed by the librarians who participated in this placement program are presented in this article, and the program’s characteristics and means of funding are explained. Moreover, the advantages for the librarians’ professional development are pointed out and the main improvements applied within UN’s Library are detailed. A suitable response from the host libraries together with the the program’s continuity and regularity are crucial for extracting greater advantage of the placements

Difusión de la investigación en redes sociales: ¿Cómo darle mayor visibilidad?

Se ofrecen pautas en la utilización de las redes sociales como Research.Gate, Academia. edu, etc. como plataformas para la difusión de la investigación académica. Utilizando además, el repositorio Dadun, de la Universidad de Navarra. Presentación realizada durante la mesa redonda, durante la Semana del Acceso Abierto 2014 (OAW2014) del Servicio de Bibliotecas de la Universidad de Navarra.La presentación también está disponible a través de Slideshare: http://www.slideshare.net/unavbiblioteca/oa2014-mesaredonda-201

Pityriasis lichenoides et varioliformis acute: case report and review of the literature

We report a case of a 63-year-old man hospitalized for a polymorphous generalized eruption consisting of maculopapules with peripheral scaling, vesicopustules, and ulceronecrotic and crusted lesions measuring 5-20 mm, localized on his trunk and extremities, particularly exuberant in the flexural area. Histopathology showed necrotic keratinocytes with exocytosis of red blood cells and lymphocytes and a dermal perivascular and periadnexal inflammatory infiltrate, composed of CD8+/CD4-/CD30- T cells, indicating the clinical diagnosis of pityriasis lichenoides et varioliformis acuta. He was treated with erythromycin and methylprednisolone reduced gradually over 5 months, with a slow but complete response; the patient was without lesions after 2 years of follow-up. The authors want to remind of this rare entity which may present difficulties in diagnosis and therap