These guidelines for management of alopecia areata have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.These guidelines were commissioned by the British Association of Dermatologists Therapy Guidelines and Audit subcommittee. Members of the committee are N.H.Cox (Chairman), A.
Although there are no effective disease-modifying therapies for mitochondrial diseases, an increasing number of trials are being conducted in this rare disease group. The use of sensitive and valid endpoints is essential to test the effectiveness of potential treatments. There is no consensus on which outcome measures to use in children with mitochondrial disease. The aims of this two-day Delphi-based workshop were to (i) define the protocol for an international, multi-centre natural history study in children with mitochondrial myopathy and (ii) to select appropriate outcome measures for a validation study in children with mitochondrial encephalopathy. We suggest two sets of outcome measures for a natural history study in children with mitochondrial myopathy and for a proposed validation study in children with mitochondrial encephalopathy.
Summary This guideline was designed to provide service providers and users with an evidence‐based set of current best practice guidelines for people and their families and carers, living with epidermolysis bullosa (EB). A systematic literature review relating to the podiatric care of patients with EB was undertaken. Search terms were used, for which the most recent articles relating to podiatric treatment were identified from as early as 1979 to the present day, across seven electronic search engines: MEDLINE, Wiley Online Library, Google Scholar, Athens, ResearchGate, Net and PubFacts.com. The Scottish Intercollegiate Guidelines Network (SIGN) methodology was used. The first guideline draft was analysed and discussed by clinical experts, methodologists and patients and their representatives at four panel meetings. The resulting document went through an external review process by a panel of experts, other healthcare professionals, patient representatives and lay reviewers. The final document will be piloted in three different centres in the U.K. and Australia. Following an EB community international survey the outcomes indicated six main areas that the community indicated as a priority to foot management. These include blistering and wound management, exploring the most suitable footwear and hosiery for EB, management of dystrophic nails, hyperkeratosis (callus), maintaining mobility and fusion of toes (pseudosyndactyly). The evidence here is limited but several interventions currently practised by podiatrists show positive outcomes.
Summary We report the case of a pigeon keeper with CD4 lymphopenia, not associated with human immunodeficiency virus infection, who developed primary cutaneous cryptococcosis at the site of a pigeon peck.
Epidermolysis bullosa (EB) is characterized by skin fragility with blister formation occurring spontaneously or following minor trauma such as gentle pressure or friction. Current physiotherapy practice is based on anecdotal care, clinical expertise and creative problem solving with caregivers and individuals with EB. Evidence based intervention is needed to establish a foundation of knowledge and to guide international practitioners to create and improve standards of care to effectively work with individuals living with EB. This clinical practice guideline (CPG) was created for the purpose of providing evidence based interventions and best clinical practices for the physiotherapy management of individuals with EB. A survey was conducted within the EB community and six outcomes were identified as a priority to address in physiotherapy management, including (1) attaining developmental motor milestones, (2) identifying safe and functional mobility in the natural environment, (3) encouraging ambulation endurance, (4) supporting safe ability to bear weight, (5) improving access to physiotherapy services, and (6) optimizing interaction with the community. A systematic literature review was conducted and articles were critically analyzed by an international panel consisting of thirteen members: healthcare professionals (including physiotherapist, doctors, and occupational therapist), caregivers, and individuals with EB. Recommendations were formulated from evidence and panel consensus. An external panel of twelve were invited to improve the quality and gather feedback on draft manuscript and recommendations. This CPG describes the development of recommendations for physiotherapy management including several best practice interventions. This guideline lays the foundational work for physiotherapist throughout the world to provide high quality services while improving and maintaining functional mobility and independence within the EB community. The CPG outlines limitations in the evidence available and possible future research needed to improve physiotherapy practice.
In our study normally painful electrical stimuli elicited itch in a patient suffering from HES-induced pruritus. This reaction was most pronounced in those skin areas in which spontaneous itch was perceived. As the tested skin sites did not show the expected spreading erythema during neurogenic inflammation induced by depolarization of sensory nerve fibres, there is no evidence for a peripheral sensitization process. In addition, peripheral sensitization would be expected to enhance pain sensitivity and therefore pain ratings would increase, sensations that were clearly not enhanced in our patient. Thus, we conclude that the most probable explanation for the electrically and mechanically induced itch is sensitization of the spinal processing of itch. Ongoing activity of peripheral 'itch-neuron' itch receptors has been proposed to induce and maintain the state of hypersensitivity to pruritic stimuli in the spinal cord. 8 Our results cannot explain the mechanism by which the peripheral itch receptors are activated in the HES-treated patient. However, the identification of central sensitization as a component of this clinical itch condition might have therapeutic implications; in neuropathic pain conditions central sensitization has been successfully treated by gabapentin. 9 It will be of major interest to explore further the therapeutic implications of central sensitization for the treatment of pruritus induced by HES.
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