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Summary: A 17‐year‐old male presented with a large sebaceous naevus (SN) comprising part of his right face and scalp and a speckled lentiginous naevus (SLN) on his left trunk, hip, neck and scalp with a checkerboard pattern. His right oral hemimucosa showed extensive papillomatous lesions, which were contiguous with the upper‐lip SN lesions. He also showed extracutaneous manifestations including cardiac, musculoskeletal and ocular alterations. Internally, he had developed two primary rhabdomyosarcomas. DNA samples of the SN, SLN, oral papillomatous hyperplasia and both rhabdomyosarcomas were analysed by Sanger sequencing. An HRAS c.37G>C mutation was detected in all of them. Skin and blood DNA were wild‐type. Phacomatosis pigmentokeratotica (PPK) is characterized by the association of an SN with a papular naevus spilus and extracutaneous manifestations. Until recently, the aetiopathogenetic hypothesis of didymosis was accepted. However, in 2013 Groesser et al. proved the existence of an activating HRAS mutation as the cause of this syndrome. A higher incidence of cancer has been observed in germline RASopathies. Furthermore, up to 30% of human cancers show dysregulation of the Ras–Raf–MEK–ERK pathways. In our patient, an HRAS mosaic mutation explains not only the cutaneous but also the extracutaneous manifestations. To our knowledge this is the first described case of PPK in which the existence of an HRAS mosaic mutation is the confirmed cause of rhabdomyosarcoma. Furthermore, the HRAS c.37G>C mutation has never been related to any type of rhabdomyosarcoma. Mosaicisms could be underdiagnosed causes of childhood tumours. As dermatologists we stand in a privileged position of being able to detect these alterations. What's already known about this topic? Phacomatosis pigmentokeratotica (PPK) is a neurocutaneous congenital disorder that has classically been included among the epidermal naevus syndromes.Recently it has been recognized as a mosaic RASopathy, caused by a postzygotic mutation in the Ras–Raf–MEK–ERK pathways.So far, pathogenic mutations have been described in 11 cases. An HRAS mutation occurred in eight of them, a BRAF mutation in two and a KRAS mutation in another. What does this study add? We report a new case of PPK with florid cutaneous, extracutaneous and neoplastic manifestations.To our knowledge this is the first report of an HRAS mutation causing rhabdomyosarcoma in PPK.Furthermore, to our knowledge an HRAS c.37G>C substitution has never been related to rhabdomyosarcoma, either in this syndrome or in isolated cases. Linked Comment:Siegel et al. Br J Dermatol 2018; 179:1031–1032. [ABSTRACT FROM AUTHOR]

In this paper we present SimulNet, our contribution to the improvement of high-quality learning at home using new technologies. SimulNet provides students with a teleteaching environment where the 'learning-by-doing' paradigm is possible. Unlike other distance teaching systems whose aim is to achieve a virtual classroom, SimulNet provides a virtual laboratory to put theoretical knowledge into practice. Because SimulNet is a 100% pure Java system, our labware can be mn on any computer and operating system. Our approach is based on the simulation of the actual laboratory tools which are delivered through the Internet (Java applets) or by CD-ROM technology (Java applications). Although SimulNet can be used in a remote access way, Java allows us to provide always the highest level of interactivity, which is an essential feature in any distance education system. In addition, SimulNet also provides a set of communication and tutoring tools for learners and instructors, providing a full cooperative learning atmosphere. We believe distance education should not mean to study alone and, therefore, we made an extra effort to provide an environment where students and teachers feel as is they were in a virtual lecture room. Index Terms--CD-ROM, labware, learning by doing, simulation, WWW.

E-learning is one of the killer applications in the new Information Society. In recent years, many educational systems have been developed, targeted to specific learning environments. One specific area of concern is practical training. This paper presents the latest update on the WWW-based teaching laboratory SimulNet. Particularly, we describe the functionality of the SimulNet whiteboard and authoring tools. Among others, the whiteboard features allow any kind of data sharing on the Web, on-line synchronous communication among learners and instructors and the possibility of following virtual presentations with an embedded Web-based slide projector. The SimulNet authoring environment includes a multimedia editor tool and both static and dynamic content structure generator. Also, current efforts in the learning technology standardization process were taken into account. We set the basis to make SimulNet compliant with the leading standard recommendations. Index Terms--Authoring tools, cooperative learning, learning technologies, ODL, standardization, WWW.

This paper presents the current trends in Internet-based training by experimental work. We show how to apply the 'learning by doing' paradigm in Internet-based distance learning, both for academic educational environments and life long training systems, taking into account available computer and network resources. First, the different phases in the learning process are introduced. The aim of this introduction is to show to the readers the importance of the learning by doing paradigm, which is not implemented in many Internet-based educational environments. Then, we identify the most important trends in this field which can be classified into two main groups. The first one consists of accessing to the real equipment through an Internet interface. The other one is based on simulation, very often, Java-based simulation. Both approaches are discussed, including brief descriptions of current available systems that implement them. Finally, these approaches are compared from different points of view. We point out the most significant variables to bear in mind and, as the readers may find a tradeoff between some of them, we also provide a graphical guide to help them in their choice. Index Terms--Internet-based education, Internet-based training, JAVA, learning-by-doing, WWW.

An adult male was described, who developed a tumour on his left arm, which was diagnosed as a primary cutaneous extramedullary plasmacytoma after histopathological, electron microscopic and immunocytochemical studies. Further studies ruled out the presence of multiple myeloma, extramedullary plasmacytoma of another site, or paraproteinemia/paraproteinuria. During a relapse of the tumour 2 1/2 years after initial treatment, a new immunocytochemical study demonstrated that the tumour cells expressed monoclonal λ IgG at the intracytoplasmic level, an unusual finding. [ABSTRACT FROM AUTHOR]

The article announces the availability of the fourth edition of Institute of Electrical & Electronics Engineers Inc. (IEEE)/RITA, an electronic journal issued by the Spanish Chapter of the IEEE Education Society through the Chapter's Technical Committee of Accreditation and Evaluation (CTAE). It also outlines the papers published within the journal's Volume 3, Number 1, May 2008 issue.