This study summarizes current research and understanding of the pathogenesis of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) and provides an update on the treatment of these conditions in children. The association of specific human leukocyte antigen subtypes with SJS and TEN occurring in certain racial groups to specific drugs has led to recommendations on pretreatment testing. Several pathways have been postulated to lead to keratinocyte apoptosis in SJS and TEN. These include Fas-Fas ligand interaction, cytotoxic T-cell and natural killer-cell damage via perforin/granzyme B/granulysin and tumor necrosis factor-alpha. The use of intravenous immunoglobulins and systemic corticosteroids in TEN is still controversial, and more trials are needed to prove the efficacy of these agents. Newer agents such as cyclosporin, infliximab and plasmapheresis have shown promise in the treatment of SJS and TEN. As the pathogenesis of SJS and TEN is further unraveled, the emergence of newer therapeutic agents with more specific mechanisms of action may lead to improved survival in this oftentimes devastating disease.
1 January 2010 in Journal of the American Academy of Dermatology
Background: Smartphone apps could support patients and caregivers in disease self-management. However, as patients’ experiences and needs might not always align with clinical judgments, the eliciting and engaging of perspectives of all stakeholders in the smartphone app design process is of paramount importance.Objective: The aims of this study are to better understand the needs of and challenges facing caregivers and health care professionals (HCPs) who care for children with atopic dermatitis (AD) and to explore the desirable features and content of a smartphone app that would support AD self-management.Methods: This study adopted a qualitative participatory co-design methodology involving 3 focus group discussions: workshop one focused on caregivers; workshop two engaged with HCPs; and in the last workshop, caregivers and digital health experts were asked to design the wireframe prototype. The participants completed a sociodemographic questionnaire, a technology acceptance questionnaire, and a workshop evaluation form.Results: Twelve caregivers participated in the first workshop, and 10 HCPs participated in the second workshop. Eight caregivers and 4 digital health experts attended the third workshop. Three superordinate themes that reflected caregivers’ and HCPs’ challenges and needs were identified: empowerment by education, confusion over treatment, and emotional impact. Workshop participants also raised a series of suggestions on the features and contents of the AD self-management app, which informed the last co-design workshop, and described their needs and challenges. In the last workshop, the participants developed a wireframe prototype of the app following the identified requirements and recommendations.Conclusions: The co-design approach was found to be a successful way of engaging with the participants, as it allowed them to express their creativity and helped us to articulate the root of the clinical problems. The co-design workshop was successful in creating and generating new ideas and solutions for smartphone app development.
1 November 2009 in Journal of the American Academy of Dermatology