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Restrictive dermopathy (RD) is a rare and lethal laminopathy caused by mutations in LMNA or ZMPSTE24. This series reports 3 patients with RD and reviews the literature of the 113 previously reported cases, including highlights of the unique constellation of clinical findings in RD, as well as histologic, radiographic, and genetic features. Early recognition of these characteristic features is vital to establish a prompt diagnosis and provide adequate family counseling for this terminal condition.

Background: There is variation in the outcomes reported in clinical studies of basal cell carcinoma. This can prevent effective meta-analyses from answering important clinical questions. Objective: To identify a recommended minimum set of core outcomes for basal cell carcinoma clinical trials. Methods: Patient and professional Delphi process to cull a long list, culminating in a consensus meeting. To be provisionally accepted, outcomes needed to be deemed important (score, 7-9, with 9 being the maximum) by 70% of each stakeholder group. Results: Two hundred thirty-five candidate outcomes identified via a systematic literature review and survey of key stakeholders were reduced to 74 that were rated by 100 health care professionals and patients in 2 Delphi rounds. Twenty-seven outcomes were provisionally accepted. The final core set of 5 agreed-upon outcomes after the consensus meeting included complete response; persistent or serious adverse events; recurrence-free survival; quality of life; and patient satisfaction, including cosmetic outcome. Limitations: English-speaking patients and professionals rated outcomes extracted from English language studies. Conclusion: A core outcome set for basal cell carcinoma has been developed. The use of relevant measures may improve the utility of clinical research and the quality of therapeutic guidance available to clinicians.

Background Facial aging is a concern for many patients. Wrinkles, loss of volume, and discoloration are common physical manifestations of aging skin. Genetic heritage, prior ultraviolet light exposure, and Fitzpatrick skin type may be associated with the rate and type of facial aging. Although many clinical trials assess the correlates of skin aging, there is heterogeneity in the outcomes assessed, which limits the quality of evaluation and comparison of treatment modalities. To address the inconsistency in outcomes, in this project we will develop a core set of outcomes that are to be evaluated in all clinical trials relevant to facial aging. Methods/design A long list of measureable outcomes will be created from four sources: (1) systematic medical literature review, (2) patient interviews, (3) other published sources, and (4) stakeholder involvement. Two rounds of Delphi processes with homogeneous groups of physicians and patients will be performed to prioritize and condense the list. At a consensus meeting attended by physicians, patients, and stakeholders, outcomes will be further condensed on the basis of participant scores. By the end of the meeting, members will vote and decide on a final recommended set of core outcomes. Subsequent to this, specific measures will be selected or created to assess these outcomes. Discussion The aim of this study is to develop a core outcome set and relevant measures for clinical trials relevant to facial aging. We hope to improve the reliability and consistency of outcome reporting of skin aging, thereby enabling improved evaluation of treatment efficacy and patient satisfaction. Trial registration Core Outcome Measures in Effectiveness Trials (COMET) Initiative, accessible at http://www.comet-initiative.org/studies/details/737. Core Outcomes Set Initiative, (CSG-COUSIN) accessible at https://www.uniklinikum-dresden.de/de/das-klinikum/universitaetscentren/zegv/cousin/meet-the-teams/project-groups/core-outcome-set-for-the-appearance-of-facial-aging. Protocol version date is 28 July 2016. Electronic supplementary material The online version of this article (doi:10.1186/s13063-017-2104-3) contains supplementary material, which is available to authorized users.

Factitious disorder imposed on self is characterized by self-induction. Dermatitis artefacta, the cutaneous subtype of factitious disorder imposed on self, can have a variety of atypical presentations. A 36-year-old woman with an extensive past medical history presented with painful nodules on her abdomen, thighs, and arms. Histologic evaluation identified panniculitis with foreign body material seen under polarization. Chart review from previous hospital visits established a history of factitious disorder imposed on self and upon subsequent search of the hospital room, syringes with an unknown substance were found. Factitial panniculitis should be considered in cases with atypical lesions or locations that do not conform to the presentation of organic causes of panniculitis. Management should include a multidisciplinary approach that prioritizes patient safety and establishes a therapeutic patient-provider relationship.

Factitious disorder imposed on self is characterized by self-induction. Dermatitis artefacta, the cutaneous subtype of factitious disorder imposed on self, can have a variety of atypical presentations. A 36-year-old woman with an extensive past medical history presented with painful nodules on her abdomen, thighs, and arms. Histologic evaluation identified panniculitis with foreign body material seen under polarization. Chart review from previous hospital visits established a history of factitious disorder imposed on self and upon subsequent search of the hospital room, syringes with an unknown substance were found. Factitial panniculitis should be considered in cases with atypical lesions or locations that do not conform to the presentation of organic causes of panniculitis. Management should include a multidisciplinary approach that prioritizes patient safety and establishes a therapeutic patient-provider relationship.

Background Compared with other imaging modalities, ultrasound is relatively deeply penetrating and can be used to evaluate deep dermal and subcutaneous structures. Objective Image skin thickness of the face and neck using high-frequency diagnostic ultrasound devices. Materials and methods Skin overlying 20 different predesignated face and neck anatomic sites in 32 individuals was imaged using 2 commercially available high-frequency diagnostic ultrasound devices, a dedicated imaging device and a diagnostic device bundled with a therapeutic device. At each site, the subcutaneous and combined epidermal and dermal layer thicknesses were assessed by blinded expert raters. Results Similar skin thickness measurements were obtained. Notably, subcutaneous fat depth was measured to be 0.2 cm at the forehead; 0.5 cm at the mental eminence; and 0.6 cm at the submental, supraglenoid, and temporal regions. The combined epidermal and dermal thickness was approximately 0.1 cm at the zygomatic process, suborbital area, inferior malar region, gonion, supraglenoid area, and nasolabial-buccal, and nasolabial fold regions. Conclusion This is the first study using high-resolution superficial diagnostic ultrasound to map skin thickness of the face and neck at standard anatomic locations. Ultrasound is an inexpensive, noninvasive, and convenient means to monitor dermatologic conditions and guide their treatment.

Objective Efforts to improve patient safety are challenged by the lack of universally agreed upon terms. The International Classification for Patient Safety (ICPS) was developed by the World Health Organization for this purpose. This study aimed to test the applicability of the ICPS to a surgical population. Design A web-based safety debriefing was sent to clinicians involved in surgical care of abdominal organ transplant patients. A multidisciplinary team of patient safety experts, surgeons and researchers used the data to develop a system of classification based on the ICPS. Disagreements were reconciled via consensus, and a codebook was developed for future use by researchers. Results A total of 320 debriefing responses were used for the initial review and codebook development. In total, the 320 debriefing responses contained 227 patient safety incidents (range: 0–7 per debriefing) and 156 contributing factors/hazards (0–5 per response). The most common severity classification was ‘reportable circumstance,’ followed by ‘near miss.’ The most common incident types were ‘resources/organizational management,’ followed by ‘medical device/equipment.’ Several aspects of surgical care were encompassed by more than one classification, including operating room scheduling, delays in care, trainee-related incidents, interruptions and handoffs. Conclusions This study demonstrates that a framework for patient safety can be applied to facilitate the organization and analysis of surgical safety data. Several unique aspects of surgical care require consideration, and by using a standardized framework for describing concepts, research findings can be compared and disseminated across surgical specialties. The codebook is intended for use as a framework for other specialties and institutions.

Background Squamous cell carcinoma (SCC) is a common skin cancer that poses a risk of metastasis. Clinical investigations into SCC treatment are common, but the outcomes reported are highly variable, omitted, or clinically irrelevant. The outcome heterogeneity and reporting bias of these studies leave clinicians unable to accurately compare studies. Core outcome sets (COSs) are an agreed minimum set of outcomes recommended to be measured and reported in all clinical trials of a given condition or disease. Although COSs are under development for several dermatologic conditions, work has yet to be done to identify core outcomes specific for SCC. Methods/design Outcome extraction for COS generation will occur via four methods: (1) systematic literature review; (2) patient interviews; (3) other published sources; and (4) input from stakeholders in medicine, pharmacy, and other relevant industries. The list of outcomes will be revaluated by the Measuring PRiority Outcome Variables via Excellence in Dermatologic surgery (IMPROVED) Steering Committee. Delphi processes will be performed separately by expert clinicians and patients to condense the list of outcomes generated. A consensus meeting with relevant stakeholders will be conducted after the Delphi exercise to further select outcomes, taking into account participant scores. At the end of the meeting, members will vote and decide on a final recommended set of core outcomes. The Core Outcome Measures in Effectiveness Trials (COMET) organization and the Cochrane Skin Group - Core Outcome Set Initiative (CSG-COUSIN) will serve as advisers throughout the COS generation process. Discussion Comparison of clinical trials via systematic reviews and meta-analyses is facilitated when investigators study outcomes that are relevant and similar. The aim of this project is to develop a COS to guide use for future clinical trials. Electronic supplementary material The online version of this article (doi:10.1186/s13063-017-2069-2) contains supplementary material, which is available to authorized users.

Importance Patients undergoing skin surgery under local anesthesia can experience anxiety. Adjuvant intraoperative anxiety reduction methods may help. Objectives To assess whether hand-holding or holding a stress ball reduces patient anxiety during excisional surgery of head or neck skin cancer with the patient under local anesthesia and to measure pain and patient satisfaction. Design, Setting, and Participants In this nonblinded, single-center randomized clinical trial, performed from January 24 through April 26, 2017, at a dermatology outpatient service in an urban, academic medical center, a consecutive sample of 135 adults who required excisional removal of nonmelanoma skin cancer of the head or neck was randomized and studied. Interventions Participants were randomized 1:1:1 to 3 groups: hand-holding, stress ball, or control (treatment as usual). Participants in the hand-holding group had a female researcher hold one of their hands during administration of anesthesia and extirpation, and those in the stress ball group held a round compressible ball and squeezed it as desired during the same period. Main Outcomes and Measures The primary outcome was anxiety, measured by a visual analog scale (VAS), 6-item State Trait Anxiety Inventory (STAI), and physiologic measures (blood pressure and heart rate). Secondary outcomes were pain during the procedure and overall participant satisfaction. In addition, participants were asked how many hours they spent researching the procedure before surgery. Results A total of 135 participants, 45 per study arm, were enrolled (mean [SD] age, 65.5 [13.9] years; 84 [62.2%] male; 134 [99.3%] white). There were no withdrawals or dropouts. Anxiety decreased over time in all groups, but no significant differences were found in the 3 anxiety measures across the 3 groups (VAS anxiety score before: control group, 3.11; hand-holding group, 3.04; stress ball group, 3.09 [P > .99]; VAS anxiety score during: control group, 1.89; hand-holding group, 2.31; stress ball group, 2.47 [P = .55]; STAI score: control group, 8.91; hand-holding group, 8.93; stress ball group, 8.76 [P = .96]). The 3 groups also did not significantly differ in postprocedure pain scores (control group, 0.78; hand-holding group, 0.64; stress ball group, 0.67;P = .85). Almost all participants (134 [99.2%]) were very satisfied. Participants who had done research had higher preoperative VAS anxiety scores (researched, 3.84; did not research, 2.62;P = .04). Conclusions and Relevance Hand-holding and squeezing a stress ball do not appear to provide incremental anxiety reduction in patients during excisional skin cancer surgery. It is possible that some subgroups may respond better or that patients may respond better when able to select and tailor their preferred anxiety reduction method. Trial Registration ClinicalTrials.gov identifier:NCT02816996

Background Video recorded and in-person observations are methods of quality assessment and monitoring that have been employed in high risk industries. In the medical field, observations have been used to evaluate the quality and safety of various clinical processes. This review summarises studies utilising video recorded or in-person observations for assessing clinician performance in medicine and surgery. Methods A search of MEDLINE (PubMed) was conducted using a combination of medical subject headings (MeSH) terms. Articles were included if they described the use of in-person or video recorded observations to assess clinician practices in three categories: (1) teamwork and communication between clinicians; (2) errors and weaknesses in practice; and (3) compliance and adherence to interventions or guidelines. Results The initial search criteria returned 3215 studies, 223 of which were identified for full text review. A total of 69 studies were included in the final set of literature. Observations were most commonly used in data dense and high risk environments, such as the emergency department or operating room. The most common use was for assessing teamwork and communication factors. Conclusions Observations are useful for the improvement of healthcare delivery through the identification of clinician lapses and weaknesses that affect quality and safety. Limitations of observations include the Hawthorne effect and the necessity of trained observers to capture and analyse the notes or videos. The comprehensive, subtle and sensitive information observations provided can supplement traditional quality assessment methods and inform targeted interventions to improve patient safety and the quality of care.