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Erythromelalgia is a rare disorder characterized by episodic burning pain with redness and warmth of the extremities. Topical and systemic medications are the mainstay of management. We reviewed the published evidence for using procedural interventions to manage erythromelalgia, including their proposed mechanism of action and possible adverse effects, and included information in this review on epidural infusion, sympathetic ganglion block, sympathectomy, pulsed radiofrequency, spinal cord stimulation, dorsal root ganglion stimulation, brain stimulation, transcranial magnetic stimulation, and botulinum toxin injections. Both successful and unsuccessful outcomes have been reported. Although these procedural interventions extend the therapeutic options for erythromelalgia, the evidence for their use is limited. Case reports and small case series comprise most of the evidence. Based on our review, a multidisciplinary approach to management may be needed for patients with erythromelalgia., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: BRAF and MEK inhibitors are standard treatments in histiocytic disorders, such as Erdheim-Chester disease (ECD). Some patients lack MAPK-pathway alterations, making these treatments less effective., Methods: We describe three patients with histiocytic disorders who have novel non-MAPK pathway alterations. These alterations were studied through genomic and in silico analyses when applicable, then treated with off-label medications rationally selected on the basis of genomic alterations., Results: Patient 1 had rapidly progressive ECD involving the CNS. A CSF1R in-frame deletion (p.S560_P566del) was identified, and in silico modeling predicted a gain-of-function mutation. This alteration was targeted with pexidartinib, which led to a clinical complete response (CR) within 2 months, and a partial response (PR) on imaging after 3 months. After 15 months, the disease became resistant to pexidartinib and transformed to histiocytic sarcoma. Patient 2 has skin-only involvement of a xanthogranuloma disorder. A KIF5B-FGFR1 fusion was identified on RNA sequencing and targeted with pemigatinib. At 24 months of follow-up, she remains in a clinical PR. Patient 3 has ECD involving the bone marrow, gastrointestinal tract, and subcutaneous tissues. A MEF2C-FLT3 fusion was identified and targeted with sorafenib. He achieved a clinical CR and radiographic PR within 3 months, which has continued for 30 months., Conclusion: We report three patients with histiocytic disorders harboring novel alterations who had sustained responses to off-label kinase inhibitors specific to their histiocytic disorder. Pathogenic variants outside of the MAPK pathway, including variants of unknown significant, may be targeted with readily available small molecules.

Competing Interests: Conflicts of interest None disclosed.

Objectives: To define the prevalence, distribution, and characteristics of patients with VEXAS who have confirmed vasculitis., Methods: Patients with VEXAS syndrome, verified by positive UBA1 mutation, were included. Chart review was performed to identify., Patient: characteristics and outcomes. Vasculitis diagnosis was based on either histopathology showing vascular inflammation or non-invasive angiography findings. Summary statistics were calculated., Results: Eighty-nine patients met inclusion criteria. All were male with a median age of onset of 66.9 years (IQR 60.1, 72.7). Median (IQR) follow up was 3.8(2.2-5.5) years during which 21 patients (23.6%) had evidence of vasculitis. Vasculitis subtypes included small vessel vasculitis (19.1%), cutaneous medium vessel vasculitis (2.2%), and large vessel vasculitis (2.2%). No patient had more than one vessel size involved. Histopathology in small vessel vasculitis patients was consistent with cutaneous leukocytoclastic vasculitis in the majority, though one patient had leukocytoclastic peritubular capillaritis on renal biopsy. Cranial symptoms (headache, vision changes, or jaw pain) were noted in 18.0%. Two additional patients not experiencing cranial symptoms exhibited large vessel involvement with confirmed carotid thickening on non-invasive angiography; one of these had a positive temporal artery biopsy., Conclusion: VEXAS syndrome manifests as a variable vessel vasculitis in a quarter of patients, with cutaneous small and medium vessel involvement being particularly common. Some patients may have positive ANCA serologies or even renal vasculitis leading to misdiagnosis. Cranial symptoms are common and may mimic giant cell arteritis, though documented large vessel inflammation is rare., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Competing Interests: None disclosed.

Competing Interests: None disclosed.

Abstract: Skin cancer is the most common cancer in the United States, with an estimated 9,500 new diagnoses made each day. Dermoscopy (also called dermatoscopy) is an established clinical approach to improving skin cancer evaluation. However, only 8% to 9% of primary care physicians use it, and no data are available for physician associate/assistant or NP use. This article reports a dermoscopy algorithm that primary care providers can use to increase the detection of skin cancer and reduce unnecessary referrals and biopsies., (Copyright © 2024 American Academy of Physician Associates.)

Competing Interests: Conflicts of interest None disclosed.

Introduction: Patients often first present to their primary care provider for skin lesion concerns, and dermoscopy is a tool that enhances diagnostic acumen of both malignant and benign skin lesions. Physician assistants (PAs) frequently serve as primary care and dermatology providers, but to our knowledge, no current research on dermoscopy expertise with PAs exists. We hypothesize that PA students could be taught dermoscopy based on the triage amalgamated dermoscopic algorithm (TADA) to increase their diagnostic skill, as previously shown with medical students., Methods: Dermoscopy was taught to first-year PA students at all 5 PA programs in the state of Minnesota. The training was 50 minutes in length and focused on the fundamentals of the TADA method. Physician assistant students participated in a pretraining and post-training test, consisting of 30 dermoscopic images., Results: A total of 139/151 (92%) PA students completed both the pretraining and post-training tests. Overall, mean scores for all students increased significantly ( P < .0001) after dermoscopy training was given (18.5 ± 7.1 vs. 23.8 ± 6.7)., Conclusion: Our study demonstrates that after TADA training, PA students improved their ability to assess dermoscopy images of both skin cancer and benign lesions accurately, suggesting that PAs can be trained as novice dermoscopists and provide better dermatologic care to patients. We strongly encourage integration of dermoscopy into didactic education across PA programs. Implementing a dermoscopy curriculum in established PA programs will enable future PAs to provide better clinical care when evaluating skin lesions., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 PA Education Association.)

Background: Delivery of dermatologic care through telemedicine was accelerated by the COVID-19 pandemic. We sought to analyze the teledermatology experience across Mayo Clinic's health care system to identify strengths and limitations of teledermatology., Methods: Electronic health records of dermatology televisits were reviewed from multiple U.S. Mayo Clinic sites from January 2020 through January 2021., Results: A total of 13,181 dermatology televisits were conducted in 6468 unique patients. Patients were primarily female (60.2%), and mean age of all patients was 34.1 years. Synchronous / live video conferencing visits were the most common (40.0%) telecare modality. Synchronous / live audio conferencing and asynchronous / store-and-forward visits comprised 33.0% and 27.0% of appointments. In total, 3944 televisits (29.9%) were successfully concluded via a single appointment. An in-person appointment was needed for 1693 patients (26.2%) after their initial televisit. For patients with a single televisit, synchronous / live video conferencing was the most common virtual modality (58.0% vs 32.2% of patients with multiple visits, p  < 0.001). Patients needing in-person follow-up visits were slightly older than those who did not (mean [SD], 38.8 [22.3] vs 35.0 [23.6] years; p  < 0.001) but without any sex-based difference. Around one-third of patients needed an in-person follow-up visit after their initial asynchronous / store-and-forward visit which was higher when compared with synchronous / live audio and video conferencing., Conclusion: Single dermatology televisits effectively managed nearly one-third of patients who did not require in-person follow-up. An initial synchronous / live video conferencing was more likely to yield a single clinical encounter, whereas asynchronous / store-and-forward visits required more in-person follow-up. Future studies are required that focus on dermatology-specific cost, diagnoses, access, quality of care, and outcomes., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Background: Proper diagnosis in primary care is crucial due to the large number of skin cancer diagnoses each year and its associated growing economic burden. Understanding how primary care providers can be best trained in dermoscopy is instrumental in helping primary care providers differentiate benign and malignant cutaneous lesions so that appropriate action can be taken (eg, biopsy/referral to dermatology or reassurance)., Objective: To assess the success of concise dermoscopy training among primary care providers., Design: A cohort study was conducted in primary care at Mayo Clinic, Rochester, including primary care physicians, nurse practitioners, and physician assistants in internal medicine and family medicine. Diagnostic accuracy was measured on standardized testing through image-based recognition and in clinical practice through observation of referral and biopsy patterns both before and after dermoscopy education, focused on the TADA plus method., Results: Forty-three primary care providers completed the image-based recognition tests, with significant improvement in mean score measured after dermoscopy training workshop (20%). Among the thirteen primary care providers who continued to use dermoscopy in clinical practice, a significant improvement (31%) in mean clinical diagnostic accuracy was observed comparing 1 year of practice data before versus after dermoscopy training., Conclusion: Improvement in diagnostic accuracy with utilization of the TADA plus method translates into clinical practice. Therefore, training primary care providers in dermoscopy may improve the dermatologic care patients receive in primary care, especially in rural or medically underserved areas where access to dermatology is limited., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Bier anemic spots, cyanosis with urticaria-like eruption (BASCULE) syndrome is a recently described entity with episodic urticarial lesions and white anemic halos on a background of erythrocyanosis, commonly affecting the lower extremities. Possible association with autonomic dysfunction remains poorly understood. Existing publications are limited, but the condition is suggested as highly underrecognized., Objective: To further characterize clinical and epidemiologic data for BASCULE syndrome., Methods: We performed an IRB-approved retrospective chart review on patients with BASCULE syndrome evaluated at Mayo Clinic from April 2021 to November 2022., Results: A total of 17 patients were identified (13 female, 4 male). Median age of onset was 12 years (range 9-17). Lower extremities were involved in all patients (17). Most patients were symptomatic with pruritus (8) or burning pain (8); three were asymptomatic. Triggers were standing (11), hot showers or hot environments (7), or no clear trigger (4). Autonomic dysfunction was present in 10 patients. Treatment responses were observed from propranolol (3) and high-dose cetirizine (1)., Conclusion: Novel epidemiologic data from 17 pediatric and young adult patients with BASCULE syndrome further supports an association with autonomic dysfunction and suggests a higher prevalence than previously acknowledged., (© 2023 Wiley Periodicals LLC.)

Vulvar lichen planus (VLP) is a chronic inflammatory disease which adversely affects patients' quality of life. The pathogenesis of VLP is unknown although Th1 immune response has been implicated. We aimed to discover specific tissue-based protein biomarkers in VLP compared to normal vulvar tissue (NVT), vulvar lichen sclerosus (VLS) and oral lichen planus (OLP). We used laser capture microdissection-liquid chromatography- tandem mass spectrometry to assess protein expression in fixed lesional mucosal specimens from patients with VLP (n = 5). We then compared proteomic profiles against those of NVT (n = 4), VLS (n = 5), OLP (n = 6) and normal oral mucosa (n = 5), previously published by our group. IL16, PTPRC, PTPRCAP, TAP1 and ITGB2 and were significantly overexpressed in VLP compared to NVT. Ingenuity pathway analysis identified antigen presentation and integrin signalling pathways. Proteins overexpressed in both VLP versus NVT and OLP versus NOM included IL16, PTPRC, PTPRCAP, TAP1, HLA-DPB1, HLA-B and HLA-DRA. This proteomic analysis revealed several overexpressed proteins in VLP that relate to Th1 autoimmunity, including IL16. Overlapping pathways, including those involving IFNγ and Th1 signalling, were observed between VLP, VLS, and OLP., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Lichen planus (LP) can affect multiple body sites including skin, mucosae, scalp and nails, causing considerable impact on patients' quality of life. Currently, there are no LP patient-reported outcome measures (PROMs) that address all body sites potentially affected by LP. We developed a LP Quality of Life Questionnaire (LPQoL), informed by an expert consortium and patient survey study, to address this gap. The study was approved by our institution's Institutional Review Board. First, a 22-item LPQoL was designed with input from LP experts at our institution. The tool was then optimized by garnering input from patients recently diagnosed with LP, who were asked to complete the LPQoL, as well as the Dermatology Life Quality Index (DLQI) and a feedback form about the LPQoL. Fifty-eight of 150 patients (39% response rate) returned the questionnaire. Mean DLQI score was 4.9 ± 5.6 SD (range 0-25) and mean LPQoL score was 13.6 ± 10.4 SD (range 0-54). LPQoL score was positively correlated with DLQI score (r = 0.79; p < 0.001). Forty-nine out of 56 (88%) and 6/56 (11%) rated the LPQoL as 'very easy' or 'fairly easy' to complete, respectively. Based on participants' feedback, we increased the recall period from one week to one month and added questions on esophageal involvement. With iterative input from LP experts and patients, we developed a LPQoL to address the gap in a multi-site PROM specific to LP. This is a pilot study and there is ongoing validation studies; therefore, this measure should not be used in clinical practice or research until validated., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disease with frequent cutaneous manifestations., Methods: We conducted a retrospective study of all patients with genetically confirmed VEXAS syndrome seen at our institution. Available clinical photographs and skin biopsy slides were reviewed., Results: Cutaneous manifestations developed in 22/25 (88%) patients with VEXAS syndrome. From this group, 10/22 (45%) developed skin involvement before or at the time of other clinical features of VEXAS. Twenty distinct dermatologic presentations of VEXAS from 14 patients were reviewed, and histopathologic patterns were classified as follows: neutrophilic urticarial dermatosis (n = 5, 25%), leukocytoclastic/urticarial vasculitis (n = 4, 20%), urticarial tissue reaction (n = 4, 20%), neutrophilic dermatosis (n = 3, 15%), neutrophilic panniculitis (n = 2, 10%), and nonspecific chronic septal panniculitis (n = 2, 10%). Common systemic findings included macrocytic anemia (96%), fever (88%), thrombocytopenia (76%), weight loss (76%), ocular inflammation (64%), pulmonary infiltrates (56%), deep venous thrombosis or pulmonary embolism (52%), and inflammatory arthritis (52%)., Conclusions: Cutaneous involvement is a common feature of VEXAS syndrome, and histopathologic findings exist on a spectrum of neutrophilic inflammatory dermatoses., (© 2023 the International Society of Dermatology.)

Purpose of Review: Histiocytic disorders, including Erdheim-Chester disease (ECD), Langerhans cell histiocytosis (LCH), and Rosai-Dorfman disease (RDD), are rare neoplasms that may present with a spectrum of neurologic involvement. Diagnostic delay is common due to heterogeneity in presentation and challenging pathology., Recent Findings: Recent advances in the treatment of these diseases targeted towards mutations in the MAP kinase pathway have led to an improved prognosis in these patients with neurologic involvement. It is critical for clinicians to have a high index of suspicion to allow for early targeted treatment and optimize neurologic outcomes. A systematic approach to diagnosis is presented in this article to allow for accurate diagnosis of these rare diseases., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Oral lichen planus (OLP) confers an approximately 1% risk of transformation to oral squamous cell carcinoma (OSCC). Early identification of high-risk OLP would be very helpful for optimal patient management. We aimed to discover specific tissue-based protein biomarkers in patients with OLP who developed OSCC compared to those who did not. We used laser capture microdissection- and nanoLC-tandem mass spectrometry to assess protein expression in fixed lesional mucosal specimens in patients with indolent OLP (no OSCC after at least 5-year follow-up, n = 6), transforming OLP (non-dysplastic epithelium with lichenoid inflammation marginal to OSCC, n = 6) or normal oral mucosa (NOM, n = 5). Transforming OLP protein profile was enriched for actin cytoskeleton, mitochondrial dysfunction and oxidative phosphorylation pathways. CA1, TNNT3, SYNM and MB were overexpressed, and FBLN1 was underexpressed in transforming OLP compared with indolent OLP. Integrin signalling and antigen presentation pathways were enriched in both indolent and transforming OLP compared with NOM. This proteomic study provides potential biomarkers, such as CA1 overexpression, for higher-risk OLP. While further validation studies are needed, we propose that epithelial-mesenchymal transition may be involved in OLP carcinogenesis., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Purpose: To evaluate the clinical presentation, treatment, and outcomes in adult patients with histiocytic disorders with ocular, orbital, optic nerve, or cavernous sinus involvement., Design: Observational, retrospective chart review., Participants: Adult patients (age ≥ 18 years) at Mayo Clinic from January 1, 1996, to July 1, 2021, with histiocytic disorders. Inclusion criteria were (1) histiocytic disorder by biopsy and appropriate clinical phenotype; (2) available medical records; and (3) ocular, orbital, optic nerve, or cavernous sinus involvement., Methods: Retrospective chart review., Main Outcome Measures: Response to therapy, measured in clinical and radiographic impact., Results: Thirty-two patients were identified: 7 with Langerhans cell histiocytosis (LCH); 15 with Erdheim-Chester disease (ECD); 1 with mixed LCH/ECD phenotype; 8 with Rosai-Dorfman disease (RDD); and 1 with mixed RDD/ECD phenotype. Ophthalmologic involvement was part of the initial presentation in 69% of patients (22/32). Eyelid edema (13/32, 41%) and proptosis (12/32, 38%) were the most frequent presentations. Isolated orbital or cavernous sinus involvement was present in 3 of 7 patients with LCH and 1 of 8 patients with RDD. Optic nerve sheath involvement was present in 2 of 7 LCH patients, 14 of 15 ECD patients, and 1 RDD/ECD patient. Diffuse (> 75%) orbital involvement was seen in 12 of 15 ECD patients and 1 of 7 LCH patients. Ocular involvement was seen in 1 of 15 ECD patients, 6 of 8 RDD patients, and 1 of 1 mixed RDD/ECD patient. The cavernous sinuses were involved in 1 of 7 LCH patients, 5 of 15 ECD patients, and both mixed phenotype patients. Visual acuity was affected in 14 patients (14/24, 58%) with a median logarithm of the minimum angle of resolution visual acuity of 0.1 (range, -0.12 to 3). BRAF V600E mutations were found in 75% (3/4) of LCH patients and 91% (10/11) of ECD patients. Patients received a variety of treatment, and response was variable across disease types., Conclusions: Orbital involvement was more commonly seen in LCH and ECD, whereas ocular involvement was more common in RDD. Visual acuity may be impacted from ocular involvement or compression of the optic nerve with diffuse orbital involvement., (Copyright © 2022 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background: Reactive granulomatous dermatitis (RGD) is an umbrella term used to describe interstitial granulomatous dermatitis (IGD), palisaded neutrophilic and granulomatous dermatitis (PNGD), and interstitial granulomatous drug eruption (IGDR)., Objective: The aim of this study was to describe systemic associations of RGD, explore possible associations between histopathologic findings and systemic RGD associations and determine clinical relevance of RGD subtypes., Methods: We retrospectively studied clinical and histopathologic characteristics of patients with RGD from 1990 through 2020., Results: Of 65 patients with RGD (41 women, 24 men; median age at diagnosis, 62 years), 37 had IGD, 26 had PNGD, and 2 had IGDR. Fifty patients (76.9%) had an associated systemic condition; rheumatologic conditions were identified for 34 (52.3%) patients. The associated systemic condition occurred before RGD in approximately 75% of patients. Statistical analyses did not show significant associations between specific subtypes of RGD and systemic diseases or treatment response, and specific histopathologic findings were not predictive of an associated systemic disease., Conclusions: Although most patients with RGD had an associated systemic condition, subtypes of RGD did not correlate with systemic associations, lending support to the use of the umbrella term RGD., (© 2022 European Academy of Dermatology and Venereology.)

Vulvar lichen sclerosus (VLS) confers approximately 3% risk of malignant transformation to vulvar squamous cell carcinoma (VSCC). We used unbiased proteomic methods to identify differentially expressed proteins in tissue of patients with VLS who developed VSCC compared to those who did not. We used laser capture microdissection- and nanoLC-tandem mass spectrometry to assess protein expression in individuals in normal vulvar tissue (NVT, n = 4), indolent VLS (no VSCC after at least 5 years follow-up, n = 5) or transforming VSCC (preceding VSCC, n = 5). Interferon-γ and antigen-presenting pathways are overexpressed in indolent and transforming VLS compared to NVT. There was differential expression of malignancy-related proteins in transforming VLS compared to indolent VLS (CAV1 overexpression, AKAP12 underexpression), particularly in the EIF2 translation pathway, which has been previously implicated in carcinogenesis. Results of this study provide additional molecular evidence supporting the concept that VLS is a risk factor for VSCC and highlights possible future biomarkers and/or therapeutic targets., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Competing Interests: Conflicts of interest None disclosed.

Background: Oral lichen planus confers a 1% risk of transformation to oral squamous cell carcinoma. While prior exome sequencing studies have identified multiple genetic mutations in oral squamous cell carcinoma, mutational analyses of lichen planus-derived OSCC are lacking. We sought to clarify genomic events associated with oral lichen planus transformation., Methods: Using rigorous diagnostic criteria, we retrospectively identified patients with non-transforming oral lichen planus (i.e., known to be non-transforming with 5 years of clinical follow-up; n = 17), transforming oral lichen planus (tissue marginal to oral squamous cell carcinoma, n = 9), or oral squamous cell carcinoma arising in lichen planus (n = 17). Gene mutational profiles derived from whole-exome sequencing on fixed mucosal specimens were compared among the groups., Results: The four most frequently mutated genes in transforming oral lichen planus and oral squamous cell carcinoma (TP53, CELSR1, CASP8, and KMT2D) identified 12/17 (71%) of oral squamous cell carcinomas and 5/9 (56%) of transforming oral lichen planus but were absent in non-transforming oral lichen planus. We identified other known oral squamous cell carcinoma mutations (TRRAP, OBSCN, and LRP2) but also previously unreported mutations (TENM3 and ASH1L) in lichen planus-associated oral squamous cell carcinomas., Conclusions: These findings suggest alterations in DNA damage response and apoptosis pathways underlie lichen planus-related oral squamous cell carcinoma transformation and are supported by mutational signatures indicative of DNA damage. This study characterized patterns of mutational events present in oral lichen planus associated with squamous cell carcinoma and in squamous cell carcinoma associated with oral lichen planus but not in non-transforming oral lichen planus., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Introduction: Histiocytic disorders pose significant diagnostic and management challenges for the clinicians due to diverse clinical manifestations and often non-specific histopathologic findings. Herein, we report the tumor board experience from the first-of-its-kind Histiocytosis Working Group (HWG)., Materials and Methods: The HWG was established in June 2017 and consists of experts from 10 subspecialties that discuss cases in a multidisciplinary format. We present the outcome of tumor board case discussions during the first 2 years since its inception (June 2017-June 2019)., Results: Forty cases with a suspected histiocytic disorder were reviewed at HWG during this time period. Average number of subspecialties involved in HWG case discussion was 5 (range, 2-9). Histiocytosis Working Group tumor board recommendations led to significant changes in the care of 24 (60%) patients. These included change in diagnosis (n = 11, 27%) and change in treatment (n = 13, 33%)., Conclusion: Our report highlights the feasibility of a multidisciplinary tumor board and its impact on outcomes of patients with histiocytic disorders., (© The Author(s) 2022. Published by Oxford University Press.)

Importance: Despite the reassuring emerging evidence on the lack of a causal relationship between sun protection and vitamin D deficiency, there is scarce data on whether multimodal sun protection is associated with reduced bone mineral density (BMD) and/or increased prevalence of osteoporotic bone fractures. This lack of data may lead to worry and decreased sun-protective behaviors on the part of patients., Objective: To investigate the association of sun-protective behaviors with BMD z scores and the prevalence of osteoporotic fractures., Design, Setting, and Participants: This population-based cross-sectional study included data from US adults who participated in the 2017 to 2018 cycle of the National Health and Nutrition Examination Survey (NHANES). Data were analyzed between September and November 2020., Main Outcomes and Measures: Definition of sun-protective behaviors (staying in the shade, wearing long sleeves, and sunscreen use), site-specific and total BMD, and osteoporotic fractures (hip, wrist, and spine) in the NHANES data., Results: Data from 3418 adults 20 years and older (average age, 39.5 [95% CI, 38.6-40.4] years; 1612 [47.2%] men and 1806 [52.9%] women) who completed the NHANES dermatology questionnaire were included in this study. The prevalence of frequent staying in the shade, wearing of long sleeves, and sunscreen use were 31.6% (95% CI, 27.8%-35.7%), 11.8% (95% CI, 10.6%-13.1%), and 26.1% (95% CI, 23.5%-28.8%), respectively. The use of individual sun-protective behaviors was not associated with diminished site-specific and total BMD z scores in the multivariate models (estimate, -0.23 [95% CI, -0.47 to 0.02], P = .18; -0.08 [-0.27 to 0.12], P = .72; and -0.10 [-0.32 to 0.13], P = .15 for frequent staying in the shade, wearing of long sleeves, and sunscreen use, respectively). Moderate to frequent staying in the shade was associated with reduced prevalence of spine fractures in the multivariate model (odds ratio, 0.19 [95% CI, 0.04-0.86], P = 0.02)., Conclusion and Relevance: In this cross-sectional study, routine use of sun-protective behaviors among the US adult population was not associated with decreased BMD or increased risk of osteoporotic fracture. Sun protection may be associated with a modest decrease in the prevalence of osteoporotic fractures, possibly owing to risk-averse behaviors. These reassuring findings add to the growing body of evidence on the safety of sun protection, with no considerable negative association with bone health.

Background: Hospitalized patients with hematologic malignancies are medically complex and commonly affected by dermatologic conditions., Methods: Retrospective chart review from January 1, 2014, to December 31, 2018, at Rochester Methodist Hospital (Rochester, Minnesota, USA). Patients hospitalized on hematology and BMT services receiving dermatology consultation were included., Results: In all, 578 consultations (63% male, median age 61 years) were reviewed. Drug reactions (22%), infection (17%), and malignant neoplasm (10%) accounted for nearly half of diagnoses. Exanthematous drug reaction (10%), graft-versus-host disease (7%), and lymphoma or leukemia cutis (6%) were the commonest individual diagnoses. There were significantly more drug reactions in severe neutropenia (33.2% vs. 15.0%), neutrophilic dermatoses in myeloid neoplasm (5.2% vs. 0.3%), and viral infection in lymphoid neoplasm (8.3% vs. 1.2%). Consultation frequently altered treatment (68%), diagnostic workup (63%), and the primary service's initial diagnostic impression (53%). Biopsies were performed in 52% of consultations and helped secure a diagnosis 73% of the time. A total of 16.4% of consultations did not receive a definitive final diagnosis, and 18.5% were resolved in one visit., Conclusion: This is the largest study to date of hospital dermatology consultation in hematology patients. Biopsies are utilized frequently and are diagnostically useful. The complexity of this patient population is evidenced by the fact that a final diagnosis remains elusive in a number of cases despite the multiple visits required for the vast majority of consultations. Nevertheless, dermatology consultation alters diagnosis and treatment in the majority of patients, highlighting the critical role dermatologists have in the care of these patients., (© 2021 the International Society of Dermatology.)

Introduction: Dermatologic complaints are a common reason for emergency department visits., Methods: Retrospective chart review from 1 January 2015 to 31 December 2019. Patients in the Mayo Clinic Emergency Department receiving dermatology consultation were included., Results: Dermatitis (24.7%, n  = 113), infection (20.4%, n  = 93), and drug reaction (10.3%, n  = 47) accounted for the majority of diagnoses. Emergency department providers often provide no diagnosis (38%) or a differential diagnosis (22%), and dermatology consultation frequently alters diagnosis (46%) and treatment (83%). Patients receiving in-person consultations are admitted more frequently than those receiving teledermatology consultations (40% vs. 16%, p  < 0.001). Primary diagnostic concordance with subsequent dermatology evaluation is high for in-person (94%) and teledermatology (88%) consultations., Discussion: This is the largest study of emergency department dermatology consultations in the United States and the first to compare in-person and teledermatology emergency department consultation utilization in clinical practice. These modalities are utilized in a complementary fashion at our institution, with severe dermatologic diagnoses seen in-person. The valuable role of emergency department dermatologists is highlighted by frequent changes to diagnosis and treatment plans that result from dermatology consultation. Furthermore, our data suggest that teledermatology is an effective modality with the potential to expand access to dermatologic expertise in the emergency department setting.

Background: Few large studies have assessed spironolactone treatment of adult female acne., Objectives: To explore the role of spironolactone in the treatment of adult female acne., Methods: We performed a retrospective case series assessing the efficacy of spironolactone treatment of a cohort of women evaluated at Mayo Clinic in Rochester, Minnesota, from 2007 through 2017., Results: In total, 395 patients (median age, 32 years) received a median spironolactone dose of 100 mg daily. Approximately two-thirds of patients (66.1%) had a complete response; 85.1% had a complete response or a partial response greater than 50%. Median times to initial response and maximum response were 3 and 5 months. Efficacy was observed across all severity subtypes of acne, including those with papulopustular and nodulocystic acne. Patients received long-term treatment with spironolactone (median duration, 13 months) and had few adverse effects., Conclusions: Spironolactone is a safe and effective treatment of acne for women., (© 2020 European Academy of Dermatology and Venereology.)

Background: Wet dressings combined with topical corticosteroids are beneficial for patients with generalized and refractory dermatosis; however, to our knowledge, serum levels after topical corticosteroid absorption during intensive therapy have not been reported previously., Aim: To examine serum levels of triamcinolone acetonide (TAC) after topical corticosteroid application during intensive wet-dressing therapy., Methods: We performed a retrospective study of adult patients admitted for inpatient wet-dressing therapy from 7 November 2015 to 24 June 2016. Data were collected on sex, age, body surface area, TAC serum levels, number of wet-dressing changes after 24 and 48 h, and type of wet dressing., Results: In total, 29 patients (14 men, 15 women) were assessed. Median [interquartile range (IQR)] age was 57 years (51.5-67.0 years) and involved body surface area was 1.98 m 2 (1.88-2.15) m 2 . Before the 24-hour blood draw, patients had received 1-3 dressing changes. Median (IQR) TAC level at 24 h was 0.33 µg/dL (0.20-0.58 µg/dL), with no significant difference noted between the number of dressing changes and TAC serum level. At 48 h, results of a serum TAC test were available for 22 patients with 2-6 dressing changes. Mean (IQR) serum level was 0.30 µg/dL (0.30-0.87 µg/dL). For each additional dressing change, there was an estimated 0.21 µg/dL increase in TAC serum level (95% CI 0.11-0.31; P < 0.001). TAC serum level was not significantly associated with sex, age, body surface area or dressing type., Conclusions: Intensive, inpatient wet-dressing therapy is associated with detectable TAC serum levels. However, we suspect that topical TAC has a primarily local therapeutic effect on the skin., (© 2019 British Association of Dermatologists.)

Background: Erdheim-Chester disease (ECD) is a rare condition and there is limited information available regarding its cutaneous manifestations., Objectives: To describe the clinical and histopathological features of cutaneous involvement in ECD., Methods: This study is a single-centre retrospective analysis of patients 18 years old and older with biopsy-proven diagnosis of ECD between 1 January 1990 and 1 April 2017. Patients from this cohort were screened for cutaneous manifestations, and BRAF c.1799T>A (p.V600E) mutational analysis was conducted in novel skin manifestations. Primary outcomes included cutaneous manifestations (morphology and topography of lesions) and BRAF mutation status in novel cutaneous findings., Results: Of 71 patients with ECD, 15 patients (21%; median age 52 years) presented with cutaneous manifestations. The most common finding was the presence of xanthelasma-like lesions (n = 8). Two patients had nonfacial cutaneous xanthomas. Seven patients presented with nonxanthomatous cutaneous involvement, with the most common finding being subcutaneous nodules (n = 5). A single patient presented with granuloma annulare-like lesions. Another patient with mixed ECD and Langerhans cell histiocytosis presented with lightly scaling, pink-red macules. In three patients, the appearance of skin lesions was the first manifestation of the disease. Most patients presented with bone/extremity pain, weight loss and other constitutional symptoms at the time of diagnosis. The BRAF V600E mutation was not found in patients with panniculitis-like and granuloma annulare-like lesions., Conclusions: The most common presentation in ECD is the presence of periorbital xanthelasma-like lesions. Other presentations include nonfacial cutaneous xanthomas, panniculitis-like lesions and granuloma annulare-like lesions. Associated symptoms at presentation include bone/extremity pain and weight loss. What's already known about this topic? Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis characterized by lipid-laden macrophage infiltration of tissue and subsequent fibrosis. Cutaneous involvement is found in approximately 25% of patients, with the majority presenting with periorbital xanthelasma-like lesions. What does this study add? We report two novel cutaneous findings: panniculitis-like lesions and granuloma annulare-like lesions. Associated bone/extremity pain and weight loss should raise suspicion for Erdheim-Chester disease., (© 2019 British Association of Dermatologists.)

Histiocytic neoplasms, a rare and heterogeneous group of disorders, primarily include Erdheim-Chester disease, Langerhans cell histiocytosis, and Rosai-Dorfman disease. Due to their diverse clinical manifestations, the greatest challenge posed by these neoplasms is the establishment of a diagnosis, which often leads to a delay in institution of appropriate therapy. Recent insights into their genomic architecture demonstrating mitogen-activated protein kinase/extracellular signal-regulated kinase pathway mutations have now enabled potential treatment with targeted therapies in most patients. This consensus statement represents a joint document from a multidisciplinary group of physicians at Mayo Clinic who specialize in the management of adult histiocytic neoplasms. It consists of evidence- and consensus-based recommendations on when to suspect these neoplasms and what tests to order for the diagnosis and initial evaluation. In addition, it also describes the histopathologic and individual organ manifestations of these neoplasms to help the clinicians in identifying their key features. With uniform guidelines that aid in identifying these neoplasms, we hope to improve the awareness that may lead to their timely and correct diagnosis., (Copyright © 2019 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.)

Background: Angioimmunoblastic T-cell lymphoma (AITL) is a systemic peripheral T-cell lymphoma with a follicular helper T-cell (T FH ) immunophenotype that frequently involves the skin. However, the histopathology of cutaneous involvement by AITL has not been fully established., Methods: We reviewed the clinicopathological features of 19 patients seen at our institution with AITL involving the skin. Pan-T-cell and T FH marker expression was evaluated by immunohistochemistry. Epstein-Barr virus (EBV) was detected using in situ hybridization (ISH) for Epstein-Barr virus-encoded small RNA (EBER). T-cell receptor (TCR) gene rearrangement was evaluated by PCR., Results: AITL affected both trunk and extremities in 15/19 cases (79%). Perivascular infiltration by small and/or medium-sized lymphocytes was seen in 18/19 (95%). Granulomatous inflammation was identified in 4/19 (21%). Aberrant loss of CD2, CD5, or CD7 was identified in 1/18 (6%), 2/18 (11%), or 7/19 (37%) cases, respectively. Seventeen of eighteen evaluable cases (95%) expressed 2 to 3 T FH markers: PD-1 in 19/19 (100%), BCL6 in 94% (17/18), and CD10 in 37% (7/19). EBV-positive cells were detected in 3/18 (17%) with varying density. Clonal TCR gene rearrangement was identified in 9/11 (82%)., Conclusions: Cutaneous involvement by AITL shows relatively non-specific histopathological features. However, an immunohistochemical panel including T FH markers and EBER ISH is useful in differential diagnosis., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, the most common periodic disorder of childhood, presents with the cardinal symptoms of periodic fever, aphthous stomatitis, pharyngitis, and adenitis typically before age 5. This review presents the recent literature on PFAPA and summarizes key findings in the pathogenesis, evaluation, and treatment of the disease. Theories surrounding the pathogenesis of PFAPA include a faulty innate immunologic response in conjunction with dysregulated T-cell activation. A potential genetic link is also under consideration. Mediterranean fever (MEFV) gene variants have been implicated and appear to modify disease severity. In individuals with the heterozygous variant, PFAPA episodes are milder and shorter in duration. Diagnostic criteria include the traditional clinical signs, in addition to the following biomarkers: elevated C-reactive protein in the absence of elevated procalcitonin, vitamin D, CD64, mean corpuscular volume, and other nonspecific inflammatory mediators in the absence of an infectious explanation for fever. Treatment of PFAPA includes tonsillectomy, a single dose of corticosteroids, and, most recently, interleukin 1 blockers such as anakinra, rilonacept, and canakinumab. Tonsillectomy remains the only permanent treatment modality., (Copyright © 2016 Elsevier Inc. All rights reserved.)