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Background Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking.Objectives Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN.Methods Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method.Results Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements.Conclusions We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.

Cellulitis is a common clinical diagnosis in the outpatient and inpatient setting; studies have demonstrated a surprisingly high misdiagnosis rate: nearly one-third of cases are other conditions (ie, pseudocellulitis). This high rate of misdiagnosis is thought to contribute to nearly $515 million in avoidable health care spending in the United States each year; leading to the delayed or missed diagnosis of pseudocellulitis and to delays in appropriate treatment. There is a broad differential diagnosis for pseudocellulitis, which includes inflammatory and noninflammatory conditions of the skin. Accurate diagnosis of the specific condition causing pseudocellulitis is crucial to management, which varies greatly.

Pyoderma gangrenosum (PG) is an inflammatory condition characterized by chronic cutaneous ulcerations. There are three proposed PG diagnostic frameworks (Su, PARACELSUS, Delphi); however, they lack consensus, and their performance has not yet been validated in a well-defined cohort of patients with PG. In this cross-sectional retrospective cohort study, we sought to evaluate and compare the concordance of these diagnostic frameworks within a single-institution cohort of patients with PG. There were 47 patients from an initial 76 identified by International Classification of Diseases-9 and/or International Classification of Diseases-10 codes, where two PG experts agreed in their diagnosis of PG on the basis of clinical descriptions, photographs, and pathology. This group was the PG cohort by which we evaluated the performance and concordance of the diagnostic frameworks. The PARACELSUS score identified the highest proportion of patients with PG (89% [42 of 47 patients]), followed by Delphi and Su criteria, each at 74% (35 of 47 patients). Assessment of multirater agreement found that the three criteria agreed in their diagnoses for 72% of the patients (95% confidence interval = 60-85%); chance-adjusted agreement was determined to be 0.44 (95% confidence = 0.16-0.68, Fleiss' kappa). Future research should seek to refine these diagnostic frameworks and identify targeted methods of testing to reduce rates of PG misdiagnosis and patient misclassification in clinical trials.

Classically known for causing ‘rose gardeners' disease’, Sporothrix schenkii is a dimorphic fungus ubiquitous in plant matter.1 Sporotrichosis infection typically follows minor cutaneous trauma, causing localized lymphocutaneous (up to 95% of cases) or fixed-cutaneous (up to 30%) forms. A rare disseminated-cutaneous form occurs in up to 8%, primarily in immunodeficient patients.1,2

Background Conflict is an unavoidable facet of relationships, and all leaders should have familiarity with the origins of, responses to, and resolutions of conflicts. Objective Readers will become familiar with several concepts key to conflict generation and resolution. Conclusions Conflicts might arise from incompatibility of behaviors or actions between two or more actors. Understanding concepts such as the Ladder of Inference or Thomas-Kilmann response patterns are useful in becoming facile with conflict management and resolution.

Objective To [1] characterize depression symptoms over time and [2] test the hypothesis that adverse metabolic parameters would associate with risk of enduring depression risk in women with polycystic ovary syndrome (PCOS). Design Prospective cohort study. Setting University center. Patient(s) One hundred sixty-three women with PCOS. Intervention(s) The Beck Depression Inventory Fast Screen (BDI-FS) was self-administered at baseline and follow-up to identify depression risk, using a cutoff score >4. Main Outcome Measure(s) BDI-FS scores. Result(s) Median baseline age was 29.0 years, and median follow-up interval was 5.5 years. Fifty-nine of 163 women had positive depression screens at baseline (36%); 52 women (32%) screened positive at follow-up. Median change in BDI-II score was 0 (interquartile range, –2, 1) over the study period. Of the 59 women at risk for depression at baseline, 22 screened negative at follow-up (37%), while 37 women remained at risk (63%). Considering these 59 women with positive depression screens at baseline, higher body mass index (BMI) was associated with increased odds of enduring depression risk at follow-up (adjusted odds ratio = 1.09; 95% confidence interval, 1.00, 1.18), in a multivariate logistic regression model. Compared with women with normal body weight at baseline, obese women (BMI >30 kg/m2) had five-fold increased odds of enduring depression risk at follow-up (adjusted odds ratio = 5.07; 95% confidence interval, 1.07, 24.0). Conclusion(s) The prevalence of depression was relatively stable over time in a cohort of women with PCOS. Elevated BMI is a hallmark of enduring depression risk. These results may assist providers in developing targeted intervention strategies to reduce the prevalence of long-term depressive symptoms in women with PCOS.

Acute generalized exanthematous pustulosis (AGEP) is an acute, self-limited, widespread cutaneous eruption characterized by the development of numerous, non-follicular, sterile pustules on a background of erythematous, edematous skin. The eruption usually develops within hours to days of exposure to medications (antibiotics, antifungals, calcium channel blockers, and carbamazepine most commonly), but it has also been documented to be associated with various infections (mostly viral), spider bites, and herbal medications. After the inciting medication is removed or precipitant infection clears, the skin reaction resolves spontaneously within 1–2 weeks. The histologic hallmark of AGEP is spongiform subcorneal and/or intraepidermal pustules with marked papillary edema, polymorphous perivascular infiltrates with neutrophils, and exocytosis of some eosinophils. AGEP is immunologically mediated by a T cell-orchestrated neutrophil response through the expression of neutrophilotactic chemokines such as CXCL8.

Pyoderma gangrenosum (PG) lacks consensus regarding treatment, and no prior studies assess treatment satisfaction in PG. To determine patient-reported satisfaction in the treatment of PG, and associations with satisfaction. Multicenter one time cross-sectional survey for patients who received systemic medication(s) to treat PG. Thirty-five patients completed the survey (mean age: 54.0 years, 65.7% female, response rate: 81.4%). Mean (± SD) SATMED-Q score was 75.0 (±16.2, range: 67.6-85.3). Older patients (72.6 ± 23.6 for 18-39 years, 74.4 ± 16.1 for 40-59, 77.1 ± 11.6 for 60+), plus those with higher incomes (72.9 ± 20.3 for $0-49 000; 74.0 ± 17.6 for $50000-99 000; 79.0 ± 14.6 for $100000+) and education status (69.4 ± 14.3 for high school equivalent, 72.9 ± 15.9 for undergraduate, 91.7 ± 10.6 for graduate), were more satisfied with treatment. Ulcerative PG had higher SATMED-Q scores (79.0 ± 13.2) than other subtypes (66.2 ± 19.3). For local therapy, wound care, or pain control, 63.2%, 100%, and 75% were satisfied, respectively. The mean DLQI was 8.6 (±7.6, range: 0-29), and higher DLQI was associated with decreased satisfaction. Satisfaction with providers was positively correlated with global satisfaction (Pearson's r = 0.638). The presence of pain and/or depression influenced both SATMED-Q (72.8 ± 18.8 with pain, 78.3 ± 11.2 without; 68.2 ± 18.8 with depression, 80.1 ± 12.2 without) and DLQI scores (12.1 ± 8.1 with pain, 3.9 ± 3.4 without; 10.3 ± 7.1 with depression, 7.4 ± 8.0 without). To optimize the patient experience, non-modifiable associations should be individually considered, and potentially modifiable associations such as satisfaction with specific providers, pain, and depression, may be targeted for management. This article is protected by copyright. All rights reserved.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis with unclear etiology and is associated with notable morbidity. Due to the rarity of PG, there are limited large, multicentered, randomized trials to guide management. We aim to highlight best practices in PG management through survey responses from expert medical dermatologists.

PURPOSE To examine validity evidence for a standardized patient scenario assessing medical students' ability to promote value using patient-centered communication (in response to a patient requesting an unnecessary test) and to explore the potential effect of various implementation and curricular factors on student scores. METHOD Third-year medical students (N = 516) from 5 U.S. MD-granting medical schools completed the communication scenario between 2014 and 2017 as part of a larger objective structured clinical examination (OSCE). Centralized raters assessed performance using an 11-item checklist. The authors collected multiple sources of validity evidence. RESULTS The mean checklist score was 0.85 (standard deviation 0.09). Interrater reliability for checklist scores was excellent (0.87, 95% confidence interval = 0.78-0.93). Generalizability and Phi-coefficients were, respectively, 0.65 and 0.57. Scores decreased as the number of OSCE stations increased (r = -0.15, P = .001) and increased when they were used for summative purposes (r = 0.26, P < .001). Scores were not associated with curricular time devoted to high-value care (r = 0.02, P = .67) and decreased when more clerkships were completed before the assessment (r = -0.12, P = .006). CONCLUSIONS This multisite study provides validity evidence supporting the use of scenario scores to assess the ability of medical students to promote value in clinical encounters using patient-centered communication. Findings illuminate the potential effect of OSCE structure and purpose on student performance and suggest clerkship learning experiences may not reinforce what students are taught in the formal curriculum regarding high-value care. Devoting more time to the topic appears insufficient to counteract this erosion.

Importance Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a spectrum of severe mucocutaneous drug reaction associated with significant morbidity and mortality. A previously developed SJS/TEN-specific severity-of-illness model (Score of Toxic Epidermal Necrolysis [SCORTEN]) has been reported to overestimate and underestimate SJS/TEN-related in-hospital mortality in various populations. Objective To derive a risk prediction model for in-hospital mortality among patients with SJS/TEN and to compare prognostic accuracy with the SCORTEN model in a multi-institutional cohort of patients in the United States. Design, Setting, and Participants Data from a multicenter cohort of patients 18 years and older treated for SJS/TEN between January 1, 2000, and June 1, 2015, were obtained from inpatient consult databases and electronic medical record systems at 18 medical centers in the United States as part of the Society for Dermatology Hospitalists. A risk model was derived based on data from 370 of these patients. Model discrimination (calculated as area under the receiver operating characteristic curve [AUC]) and calibration (calculated as predicted vs observed mortality, and examined using the Hosmer-Lemeshow goodness-of-fit statistic) were assessed, and the predictive accuracy was compared with that of SCORTEN. All analysis took place between December 2016 and April 2018. Main Outcomes and Measures In-hospital mortality. Results Among 370 patients (mean [SD] age 49.0 [19.1] years; 195 [52.7%] women), 54 (15.14%) did not survive to hospital discharge. Five covariates, measured at the time of admission, were independent predictors of in-hospital mortality: age in years (odds ratio [OR], 1.05; 95% CI, 1.02-1.07), body surface area (BSA) in percentage of epidermal detachment (OR, 1.02; 95% CI, 1.01-1.04), serum bicarbonate level below 20 mmol/L (OR, 2.90; 95% CI, 1.43-5.88), active cancer (OR, 4.40; 95% CI, 1.82-10.61), and dialysis prior to admission (OR, 15.94; 95% CI, 3.38-66.30). A severity-of-illness score was calculated by taking the sum of 1 point each for age 50 years or older, epidermal detachment greater than 10% of BSA, and serum bicarbonate level below 20 mmol/L; 2 points for the presence of active cancer; and 3 points for dialysis prior to admission. The score was named ABCD-10 (age, bicarbonate, cancer, dialysis, 10% BSA). The ABCD-10 model showed good discrimination (AUC, 0.816; 95% CI, 0.759-0.872) and calibration (Hosmer-Lemeshow goodness of fit test, P = .30). For SCORTEN, on admission, the AUC was 0.827 (95% CI, 0.774-0.879) and was not significantly different from that of the ABCD-10 model (P = .72). Conclusions and Relevance In this cohort of patients with SJS/TEN, ABCD-10 accurately predicted in-hospital mortality, with discrimination that was not significantly different from SCORTEN. Additional research is needed to validate ABCD-10 in other populations. Future use of a new mortality prediction model may provide improved prognostic information for contemporary patients, including those enrolled in observational studies and therapeutic trials.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, severe mucocutaneous reaction with few large cohorts reported. This multicenter retrospective study included patients with SJS/TEN seen by inpatient consultative dermatologists at 18 academic medical centers in the United States. A total of 377 adult patients with SJS/TEN between January 1, 2000 and June 1, 2015 were entered, including 260 of 377 (69%) from 2010 onward. The most frequent cause of SJS/TEN was medication reaction in 338 of 377 (89.7%), most often to trimethoprim/sulfamethoxazole (89/338; 26.3%). Most patients were managed in an intensive care (100/368; 27.2%) or burn unit (151/368; 41.0%). Most received pharmacologic therapy (266/376; 70.7%) versus supportive care alone (110/376; 29.3%)-typically corticosteroids (113/266; 42.5%), intravenous immunoglobulin (94/266; 35.3%), or both therapies (54/266; 20.3%). Based on day 1 SCORTEN predicted mortality, approximately 78 in-hospital deaths were expected (77.7/368; 21%), but the observed mortality of 54 patients (54/368; 14.7%) was significantly lower (standardized mortality ratio = 0.70; 95% confidence interval = 0.58-0.79). Stratified by therapy received, the standardized mortality ratio was lowest among those receiving both steroids and intravenous immunoglobulin (standardized mortality ratio = 0.52; 95% confidence interval 0.21-0.79). This large cohort provides contemporary information regarding US patients with SJS/TEN. Mortality, although substantial, was significantly lower than predicted. Although the precise role of pharmacotherapy remains unclear, co-administration of corticosteroids and intravenous immunoglobulin, among other therapies, may warrant further study.

Eczema herpeticum (EH) is characterized by viral infection of a preexisting inflammatory dermatosis and is associated with considerable morbidity. In this cross-sectional study, we sought to determine the incidence, risk factors, comorbidities, costs of care, length of stay, and mortality in hospitalized U.S. children with EH. The Nationwide Inpatient Sample 2002–2012, containing a 20% sample of all U.S. hospitalizations, was analyzed. We used a validated approach involving International Classification of Diseases, Ninth Revision–Clinical Modification codes to identify patients primarily admitted for EH (n = 4,655 weighted). The mean annual incidence of hospitalization for EH per million children ranged from 4.03 to 7.30, increasing significantly from 2008 to 2012. EH was associated with younger age (odds ratio [95% confidence interval] for 3–4 years = 1.29 [1.02–1.64]) and nonwhite race/ethnicity (black = 2.93 [2.35–3.66], Asian=4.41 [3.34–5.83], Native American = 2.96 [1.51–5.78]) but was less likely to occur in areas with lower income quartiles (quartile 1, 0.66 [0.53–0.84]; quartile 2, = 0.68 [0.54–0.87]; quartile 3, 0.70 [0.56–0.88]). EH was associated with atopic dermatitis (11.72 [9.48–14.49]). Increased length of stay and costs of care were associated with Asian race. Mean length of stay was 3.86 ± 0.09 days, and mean cost of care was $5,237.6 ± 160.6. Overall mortality was 0.1%. EH in hospitalized children poses a substantial burden to the health care system.

Rosacea fulminans is a rare inflammatory condition of the central face marked by the abrupt onset of erythematous coalescing papules, pustules, nodules, and draining sinuses. Due to infrequent reporting in the literature, the pathophysiology, classification, and nomenclature of this condition remain controversial. This comprehensive review evaluated a total of 135 cases of rosacea fulminans for clinical and histopathologic features and reported treatment strategies. Patients were 91% female with an average age of onset of 31.3 years. Only 19% of cases reported duration of symptoms longer than 3 months, and reports of recurrence were uncommon. A majority of patients had history of rosacea or flushing, and common triggers included hormonal shifts, emotional stress, and medications. Extrafacial or systemic involvement was rare. Though oral and topical antibiotics were frequently utilized to treat rosacea fulminans, there was a clear shift in reported treatments for rosacea fulminans following the introduction of isotretinoin use in 1987, marked by increased reliance on isotretinoin in addition to topical and systemic corticosteroids. Newer treatments were associated with superior improvement compared with antibiotic monotherapy, most notably dramatically reduced rates of scarring, though reduced rates of disease recurrence were not evident. Several patterns revealed through this review reinforce the classification of rosacea fulminans as a severe yet distinct variant of rosacea and highlight key distinguishing clinical features and treatment options for optimal management.

Hormone-based therapies including combined oral contraceptive medications and spironolactone are considered effective therapies to treat adult acne in women. Our objective is to provide a concise and comprehensive overview of the types of hormonal therapy that are available to treat acne and comment on their efficacy and safety profiles for clinical practice. A systematic search using the PubMed Database was conducted to yield 36 relevant studies for inclusion in the review and several conclusions were drawn from the literature. Treatment with oral contraceptive pills leads to significant reductions in lesion counts across all lesion types compared with placebo. There were no consistent differences in efficacy between the different combined oral contraceptive formulations. In terms of risk, oral contraceptive pill users had three-times increased odds of venous thromboembolism versus non-users according to a recent meta-analysis (95% confidence interval 2.46-2.59). Data on oral contraceptive pill use and breast cancer risk are conflicting but individual patient risk factors and histories should be discussed and considered when prescribing these medications. However, use of these medications does confer measurable protection from endometrial and ovarian cancer. Spironolactone was also shown to be an effective alternative treatment with good tolerability. Combined oral contraceptive medications and spironolactone as adjuvant and monotherapies are safe and effective to treat women with adult acne. However, appropriate clinical examinations, screening, and individual risk assessments particularly for venous thromboembolism risk must be conducted prior to initiating therapy.

Background: The complex interplay between ethnicity, Fitzpatrick skin type (FST), and hirsutism in patients with polycystic ovarian syndrome (PCOS) is poorly understood. Objective: In this cross-sectional, retrospective analysis, we examined the prevalence, severity, and distribution of hirsutism with clinician-rated site-specific and total modified Ferriman-Gallwey (mFG) visual scoring in a diverse cohort of American patients with PCOS. Methods: Independent analyses were conducted on the basis of patient-reported FST ratings and ethnicity. Results: In this PCOS cohort, a correlation was found between hirsutism and ethnicity and the highest prevalence of hirsutism and total mFG scores was observed in Hispanic, Middle Eastern, African American, and South Asian patients. A positive correlation between hirsutism and FST was also observed with an increasing prevalence of hirsutism in the group of patients with higher FSTs. Significant trends in the anatomic distribution of hirsutism were observed between ethnic groups as well. A higher facial mFG score was found in African American patients but higher mFG scores in the truncal and extremity regions were observed in Middle Eastern patients. Truncal hirsutism was also associated with higher FSTs. Conclusions: Ethnicity and FST may be important variables in both the quantitative and qualitative presentations of hirsutism in women with PCOS and should be considered in the diagnostic evaluation of any patient who is suspected of having the condition. Previously published studies that examined ethnicity, FST, and hirsutism in homogeneous cohorts limited comparison and generalizability but the strength of this study lies in its detailed analysis within a single large and diverse PCOS cohort. Validated studies are needed to determine whether clinical criteria for hirsutism should be adjusted for ethnicity and FST in the PCOS population and particularly within diverse cohorts and patients of mixed ancestry. Keywords: PCOS, hirsutism, ethnicity, Fitzpatrick skin type, hyperandrogenism, modified Ferriman-Gallwey

A consultative dermatology service plays an important role in patient care and education in the hospital setting. Optimizing education in balance with high-quality dermatology consultative services is both a challenge and an opportunity for dermatology consultation teams. There is an emergence of new information about how dermatology can best be taught in the hospital, much of which relies on principles of workplace learning as well as the science of how learning and teaching best happen in work settings. These best practices are summarized in this narrative review with integrated discussion of concepts from outpatient dermatology education and lessons learned from other inpatient teaching models. In addition, consultative dermatology curricula should utilize a blended curriculum model comprised of patient care and active learning and self-study modalities. Specific educational methods will discuss 2 strategies: (1) direct patient-care activities (ie, bedside teaching rounds) and (2) nonpatient care activities (ie, case presentations, didactic sessions, online modules, and reading lists).

Checkpoint inhibitors used for cancer immunotherapy may also result in autoimmunity.1, 2 We present a case of Wong-type dermatomyositis complicating treatment with nivolumab, an inhibitor of the programmed cell death 1 (PD-1) receptor. Wong-type dermatomyositis is a rare variant of classic dermatomyositis with concurrent clinical and histopathologic findings mimicking pityriasis rubra pilaris.3 Our report of nivolumab-related Wong-type dermatomyositis expands the clinical spectrum of autoimmune diseases associated with checkpoint inhibitors.

Behcet disease (BD) is a neutrophilic dermatosis characterized by relapsing flares of orogenital aphthous ulcers, uveitis, and skin inflammation.1, 2, 3 Extracutaneous manifestations involve gastrointestinal, neurologic, pulmonary, and cardiac systems.1 The spectrum of skin manifestations includes erythema nodosum, papulopustular lesions, and pathergy. Skin abscesses, leukocytoclastic vasculitis, bullae, or lesions mimicking Sweet syndrome or pyoderma gangrenosum (PG) are rare.1, 2, 3 We describe a patient with atypical BD with endocarditis, PG-like ulcerations, and methicillin-resistant Staphylococcus aureus (MRSA)-positive abscesses, highlighting a unique constellation of infection and immune dysregulation.

IMPORTANCE: Alcohol flushing syndrome (AFS, also known as Asian glow and Asian flush) affects 20% to 47% of East Asians and causes significant psychosocial distress. There are no approved treatments for this condition. OBJECTIVE: To determine whether brimonidine gel, 0.33%, decreases facial erythema in patients with AFS after consumption of alcohol. DESIGN, SETTING, AND PARTICIPANTS: In this randomized clinical trial, 20 healthy volunteers of East Asian descent with a self-reported history of AFS were recruited between April 2018 and March 2019. INTERVENTIONS: Participants were randomized to application of brimonidine gel to either the left or right half of their face. Placebo control was applied to the opposite side. After 30 minutes, participants ingested alcohol. MAIN OUTCOMES AND MEASURES: Outcomes were specified before data collection. The difference in erythema between the treated and placebo side of each participant’s face was measured 60 minutes after drug application (primary outcome) and at 90 and 120 minutes after drug application (secondary outcomes). Participants were asked to rate their likelihood of using the medication again and their likelihood of recommending the medication to a friend on a scale of 0 to 10. RESULTS: The mean (SD) age of the 20 individuals enrolled in the study was 30.5 (8.4) years, and there were 10 women (50%). There was a significant difference in erythema at 60 minutes after drug application as measured by the difference in Clinician Erythema Assessment score (2.1; 95% CI, 1.5-2.71; P

Objective To characterize metabolic features of women with polycystic ovary syndrome (PCOS) by exercise behavior and determine relative health benefits of different exercise intensities. Design Cross-sectional study. Setting Tertiary academic institution. Patient(s) Three hundred and twenty-six women aged 14–52 years-old with PCOS by Rotterdam criteria examined between 2006 and 2013. Intervention(s) International Physical Activity Questionnaire (IPAQ) administered to classify patients into three groups based on Department of Health and Human Services (DHHS) Guidelines of vigorous, moderate, and inactive, along with physical examination and serum testing. Main Outcome Measure(s) Blood pressure, body mass index (BMI), waist circumference, fasting lipids, fasting glucose and insulin, 2-hour 75-gram oral glucose tolerance, homeostatic model assessment of insulin resistance (HOMA-IR). Result(s) The DHHS guidelines for adequate physical activity were met by 182 (56%) women. Compared with moderate exercisers and inactive women, the vigorous exercisers had lower BMI and lower HOMA-IR; higher levels of high-density lipoprotein cholesterol and sex hormone-binding globulin; and a reduced prevalence of the metabolic syndrome. In a multivariate logistic regression analysis controlling for age, BMI, and total energy expenditure, every hour of vigorous exercise reduced a patient's odds of metabolic syndrome by 22% (odds ratio 0.78; 95% confidence interval, 0.62, 0.99). Conclusion(s) Women with PCOS who met DHHS guidelines for exercise demonstrated superior metabolic health parameters. Vigorous but not moderate activity is associated with reduced odds of the metabolic syndrome, independent of age, BMI, and total energy expenditure. PCOS patients should be encouraged to meet activity guidelines via vigorous physical activity.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cutaneous T-cell lymphoma characterized by neoplastic α/β T cells infiltrating subcutaneous tissues in a lobular pattern. Few data support the optimal treatment regimen for patients, given the rarity of this condition, and even fewer data describe treatment when diagnosed during pregnancy. We describe a case of SPTCL in a pregnant patient who achieved clinical remission after treatment with corticosteroid monotherapy. Our case suggests that corticosteroids should be considered as first-line treatment in pregnant patients with SPTCL.

Author(s): Shinkai, Kanade; Chen, Chen Amy; Schwartz, Brian S; Loeser, Helen; Ashe, Cynthia; Irby, David M | Abstract: PROBLEM:Academic medical centers struggle to achieve parity in advancement and promotions between educators and discovery-oriented researchers in part because of narrow definitions of scholarship, lack of clear criteria for measuring excellence, and barriers to making educational contributions available for peer review. Despite recent progress in expanding scholarship definitions and identifying excellence criteria, these advances are not integrated into educator portfolio (EP) templates or curriculum vitae platforms. APPROACH:From 2013 to 2015, a working group from the Academy of Medical Educators (AME) at the University of California, San Francisco (UCSF) designed a streamlined, criteria-based EP (EP 2.0) template highlighting faculty members' recent activities in education and setting rigorous evaluation methods to enable educational scholarship to be objectively evaluated for academic advancement, AME membership, and professional development. OUTCOMES:The EP 2.0 template was integrated into the AME application, resulting in high overall satisfaction among candidates and the selection committee and positive feedback on the template's transparency, ease of use, and streamlined format. In 2016, the EP 2.0 template was integrated into the campus-wide curriculum vitae platform and academic advancement system. NEXT STEPS:The authors plan to increase awareness of the EP 2.0 template by educating promotions committees and faculty at UCSF and partnering with other institutions to disseminate it for use. They also plan to study the impact of the template on supporting educators by making their important scholarly contributions available for peer review, providing guidance for professional development, and decreasing disparities in promotions.

Mucous membrane pemphigoid (MMP) is a rare, autoantibody-mediated disease characterized by mucocutaneous blistering including oral, ocular, laryngeal, and skin involvement. Treating MMP is challenging, with few effective therapies available. We report successful treatment of a patient with treatment-refractory MMP with the proteasome inhibitor, bortezomib.

Background Polycystic ovary syndrome (PCOS) is a very common disorder well known to be associated with insulin resistance and metabolic disease. Insulin resistance is likely involved in the promotion of the PCOS reproductive phenotype and may mediate some of the ovarian morphology seen in the disorder. The phenotype of each individual woman with PCOS can vary widely as can her metabolic risk. Methods This is a cross-sectional study of patients seen in a multidisciplinary PCOS clinic at the University of California at San Francisco between 2006 and 2014. All participants underwent systematic evaluation with anthropometric measurements, comprehensive skin exam, transvaginal ultrasound and laboratory studies at the time of their initial visit to the clinic. Serum samples were stored and androgen studies were carried out on all stored samples at the University of Virginia. Logistic regression was employed to evaluate the association between ovarian volume or follicle number and metabolic parameters (fasting insulin, HOMA-IR, fasting glucose, 2 h glucose, waist circumference) and hyperandrogenism (free testosterone, total testosterone, DHEAS, acanthosis nigricans), controlling for age. Results Three-hundred thirteen patients seen during the study period met Rotterdam criteria for PCOS and had sufficient measurements for inclusion in our analysis. The odds ratio of elevated HOMA-IR for patients with a maximum ovarian volume >10 cc was 1.9 compared to those with a maximum ovarian volume of ≤10 cc (95% CI 1.0–3.4). The odds ratio of abnormal fasting insulin for patients with higher ovarian volume was 1.8 (95% CI 1.0–3.4) compared with those with lower ovarian volume. Follicle number was not significantly associated with any metabolic parameters. Conclusions Increased ovarian volume is associated with markers of insulin resistance in PCOS. In concordance with prior studies, we did not find follicle number to be predictive of metabolic risk. Ovarian volume may serve as a useful tool to aid clinicians in their risk stratification and counseling of patients with PCOS.

Importance Toxic epidermal necrolysis (TEN) and Stevens-Johnson Syndrome (SJS) are rare, acute, life-threatening dermatologic disorders involving the skin and mucous membranes. Research into these conditions is hampered by a lack of standardization of case reporting and data collection. Objective To establish a standardized case report form to facilitate comparisons and maintain data quality based on an international panel of SJS/TEN experts who performed a Delphi consensus-building exercise. Evidence Review The elements presented for committee scrutiny were adapted from previous case report forms and from PubMed literature searches of highly cited manuscripts pertaining to SJS/TEN. The expert opinions and experience of the members of the consensus group were included in the discussion. Findings Overall, 21 out of 29 experts who were invited to participate in the online Delphi exercise agreed to participate. Surveys at each stage were administered via an online survery software tool. For the first 2 Delphi rounds, results were analyzed using the Interpercentile Range Adjusted for Symmetry method and statements that passed consensus formulated a new case report form. For the third Delphi round, the case report form was presented to the committee, who agreed that it was “appropriate and useful” for documenting cases of SJS/TEN, making it more reliable and valuable for future research endeavors. Conclusions and Relevance With the consensus of international experts, a case report form for SJS/TEN has been created to help standardize the collection of patient information in future studies and the documentation of individual cases.

Author(s): Davari, Parastoo; Millsop, Jillian W; Johnson, Mary Ann N; Takahashi, Stefani R; Peng, David H; Badger, Joanna; Bahr, Brooks A; Shinkai, Kanade; Li, Chin-Shang; Fazel, Nasim | Abstract: Background/Aims: Limited data are available regarding the undergraduate dermatology clinical clerkship curriculum in the United States. Our primaryaim is to assess medical students’ perspectives of the dermatology clinical clerkship.Methods: A multicenter survey study was conducted, which included four California dermatology academic programs. A 17-item questionnaire was designed to investigate medical student perception with regard tothe overall educational value of the various teaching aspects of the dermatology clinical clerkship.Results: A total of 152 medical student surveys were completed. Over half of the medical students felt proficient in diagnosing the most commondermatologic conditions. Eighty-seven percent of medical students were very satisfied with the dermatology clerkship. Ninety-one percent of students felt the length of the clerkship was appropriate.Conclusions: The vast majority of medical students reported a high level of proficiency in the treatment and diagnosis of common skin disorders. In contrast, our findings suggest that medical students may not begaining sufficient hands-on experience in conducting certain dermatologic procedures following the dermatology clerkship. Overall, medical studentperception of the dermatology clinical clerkship was mostly positive.

IMPORTANCE: Pyoderma gangrenosum and necrotizing Sweet syndrome are diagnostically challenging variants of neutrophilic dermatosis that can clinically mimic the cutaneous and systemic features of necrotizing fasciitis. Improved characterization of these rare variants is needed, as improper diagnosis may lead to inappropriate or delayed treatment and the potential for morbidity. OBJECTIVE: To determine the characteristics of necrotizing neutrophilic dermatosis to improve diagnostic accuracy and distinguish from infection. DESIGN, SETTING, AND PARTICIPANTS: A case series of patients with necrotizing neutrophilic dermatosis treated at 3 academic hospitals (University of California San Francisco, Oregon Health and Science University, and University of Minnesota) from January 1, 2015, to December 31, 2017, was performed along with a literature review of related articles published between January 1, 1980, and December 31, 2017. Data were obtained from medical records as well as Medline and Embase databases. All patients had signs resembling necrotizing infection and had a final diagnosis of pyoderma gangrenosum with systemic features or necrotizing Sweet syndrome. Patients were excluded if a diagnosis other than neutrophilic dermatosis was made, if key clinical information was missing, and if reported in a non-English language. MAIN OUTCOMES AND MEASURES: Description of key characteristics of necrotizing neutrophilic dermatosis. RESULTS: Overall, 54 patients with necrotizing neutrophilic dermatosis were included, of which 40 had pyoderma gangrenosum with systemic features and 14 had necrotizing Sweet syndrome. Of the 54 patients, 29 (54%) were male and 25 (46%) were female, with a mean (SD) age of 51 (19) years. Skin lesions commonly occurred on the lower (19 [35%]) and upper (13 [24%]) extremities and developed after a surgical procedure (22 [41%]) or skin trauma (10 [19%]). Shock was reported in 14 patients (26%), and leukemoid reaction was seen in 15 patients (28%). Of the patients with necrotizing neutrophilic dermatosis, 51 (94%) were initially misdiagnosed as necrotizing fasciitis and subsequently received inappropriate treatment. Debridement was performed in 42 patients (78%), with a mean (SD) of 2 (2 [range, 1-12]) debridements per patient. Four amputations (7%) were performed. Forty-nine patients (91%) received antibiotics when necrotizing neutrophilic dermatosis was misdiagnosed as an infection, and 50 patients (93%) received systemic corticosteroids; all patients responded to immunosuppressants. CONCLUSIONS AND RELEVANCE: A complex spectrum of clinical findings of pyoderma gangrenosum and Sweet syndrome with prominent systemic inflammation exists that defines a new subset of neutrophilic dermatoses, termed necrotizing neutrophilic dermatoses; recognizing the difference between this variant and severe infection may prevent unnecessary surgical procedures and prolonged disease morbidity associated with a misdiagnosis and may expedite appropriate medical management.

Objective To characterize the population of patients excluded from a diagnosis of polycystic ovary syndrome (PCOS) when follicle number criteria are increased to 25 per ovary as suggested by the Androgen Excess and Polycystic Ovary Syndrome Society's recent task force. Design Cross-sectional study. Setting Tertiary academic center. Patient(s) A total of 259 women with PCOS according to Rotterdam criteria who were systematically examined from 2007 to 2015, with 1,100 ovulatory women participating in the Ovarian Aging (OVA) Study as controls. Intervention(s) Anthropometric measurements, serum testing, ultrasonic imaging, and comprehensive dermatologic exams. Main Outcome Measure(s) Body mass index (BMI), waist to hip ratio (WHR), serum cholesterol, fasting glucose and insulin, follicle count per ovary, biochemical hyperandrogenemia, and hirsutism. Result(s) Forty-seven of 259 women meeting the Rotterdam criteria (18.1%) were excluded from a diagnosis of PCOS when the follicle number criteria was increased to 25. These women had clinical evidence of hyperandrogenism (68.1%) and biochemical hyperandrogenemia (44.7%), although fewer reported oligoanovulation (26.8%). The excluded women had elevated total cholesterol, fasting insulin, and homeostatic model of insulin resistance (HOMA-IR) when compared with controls despite controlling for age and BMI. Conclusion(s) The women excluded from the PCOS diagnosis by raising the threshold of follicle number per ovary to ≥25 continue to show evidence of metabolic risk.

Qualitative evidence suggests that hirsutism inflicts significant negative impacts on quality of life and may be associated with depression. Quantitative research is essential to determine best practices in caring for hirsute patients.To quantify quality-of-life impact of hirsutism and evaluate how the degree of hirsutism (as assessed by patients and clinicians) is associated with quality of life and depressive symptoms.This study included 229 patients aged 14 to 52 years consecutively recruited from a polycystic ovarian syndrome (PCOS) clinic between May 18, 2006, and October 25, 2012, who met the Rotterdam PCOS criteria. Data analysis was completed July 2015, and alterations were completed in response to reviewer comments in January 2016.Clinicians and patients rated degree of hirsutism using the modified Ferriman-Gallwey (mFG) instrument, a visual scoring method assessing androgen-dependent hair growth in 9 body areas. Hirsutism-related quality of life was assessed using the Skindex-16, a validated quality of life instrument for skin disorders. Depressive symptoms were assessed using the Beck Depression Inventory-Fast screen.Overall, 229 patients aged 14 to 52 years who met the Rotterdam criteria for polycystic ovarian syndrome rated themselves and were rated by clinicians for hirsutism. Total mean self-rated mFG score for patients was 13.3 out of a total 36 possible points; total mean clinician-rated mFG score for patients was 8.63 (P .001); self-ratings for hirsutism were higher for all body areas except thigh. Hirsutism had a significant negative effect on quality of life; the mean (SD) Skindex-16 score for the emotion domain was 73.9 (29.8) and 44.3 (33.7) for the function domain. Higher degrees of hirsutism (determined by both patients and clinicians) were moderately associated with more negative quality-of-life impact; however, self-ratings (r = 0.19-0.46) were more strongly associated than clinician ratings (r = 0.14-0.32)(P .05 for all). Only self-ratings of hirsutism were significantly associated with risk of depression (r = 0.14; P .05).There is notable discordance in the perception of hirsutism between patients and clinicians; patients view their hirsutism as more severe than clinicians do. Quality-of-life impacts of hirsutism are consistent with that reported for other serious skin conditions. This negative impact is only partially associated with the degree of hirsutism, with self-ratings being more highly associated with quality of life impact than clinician ratings. These results support guidelines recommending that treatment be guided largely by patient distress with hair growth and subjective perceptions as opposed to clinician judgment of degree. Patient self-rating is critical information for patient-centered care for hirsute patients.

This study investigates how new parent leave policies are perceived by dermatology program directors and residents.

Background Peristomal pyoderma gangrenosum (PPG) is an uncommon subtype of pyoderma gangrenosum. PPG is a challenging condition to diagnose and treat; no evidence-based guidelines exist. Objective We sought to identify important clinical features of PPG and effective treatments available for its management. Methods A systematic literature review of PPG was performed using PubMed, Medline, and Embase databases. Results We describe 335 patients with PPG from 79 studies. Clinical features include a painful, rapidly progressing ulcer with undermined, violaceous borders with a history of ostomy leakage and local skin irritation or trauma. Systemic steroids are first-line therapy; infliximab and adalimumab provide concomitant control of active inflammatory bowel disease. Combination local and systemic therapy was commonly used. Wound dressings, vehicle selection, and appropriate ostomy devices to minimize leakage, irritation, and pressure-induced ischemia can improve healing. Distinct from classic ulcerative pyoderma gangrenosum, surgical approaches, such as stoma closure and resection of active inflammatory bowel disease, have an effective role in PPG management. Limitations PPG is a rare disease lacking randomized trials or diagnostic guidelines. Treatment duration and follow-up time among studies are variable. Conclusions Key clinical characteristics of PPG are highlighted. Several treatments, including a more prominent role for surgical intervention, may be effective for PPG treatment.

Importance Pyoderma gangrenosum is a rare inflammatory skin condition that is difficult to diagnose. Currently, it is a “diagnosis of exclusion,” a definition not compatible with clinical decision making or inclusion for clinical trials. Objective To propose and validate diagnostic criteria for ulcerative pyoderma gangrenosum. Evidence Review Diagnostic criteria were created following a Delphi consensus exercise using the RAND/UCLA Appropriateness Method. The criteria were validated against peer-reviewed established cases of pyoderma gangrenosum and mimickers using k-fold cross-validation with methods of multiple imputation. Findings Delphi exercise yielded 1 major criterion—biopsy of ulcer edge demonstrating neutrophilic infiltrate—and 8 minor criteria: (1) exclusion of infection; (2) pathergy; (3) history of inflammatory bowel disease or inflammatory arthritis; (4) history of papule, pustule, or vesicle ulcerating within 4 days of appearing; (5) peripheral erythema, undermining border, and tenderness at ulceration site; (6) multiple ulcerations, at least 1 on an anterior lower leg; (7) cribriform or “wrinkled paper” scar(s) at healed ulcer sites; and (8) decreased ulcer size within 1 month of initiating immunosuppressive medication(s). Receiver operating characteristic analysis revealed that 4 of 8 minor criteria maximized discrimination, yielding sensitivity and specificity of 86% and 90%, respectively. Conclusions and Relevance This Delphi exercise produced 1 major criterion and 8 minor criteria for the diagnosis of ulcerative pyoderma gangrenosum. The criteria may serve as a guideline for clinicians, allowing for fewer misdiagnoses and improved patient selection for clinical trials.

Healthcarecostsare increasing faster in theUnitedStates than in any other industrialized nation, with estimates of lowvalue services—services that can be eliminated without adversely affectingpatient care—composingup to 30%ofhealth care spending.1,2(pp65-108)3 Consistent with the Physician Charter on Medical Professionalism,physicianshave a responsibility to balance individual patient needswith “care that isbasedonthewiseandcost-effectivemanagementof limitedclinical resources,” including“scrupulousavoidanceof superfluous tests.”3(p245) Specialty-specific efforts are critical to identifying the relevant areaswhere cost-effective treatment can be delivered in balance with quality and efficiency; dermatologists havebegun to join thenational efforts toward this end.4,5 In this issueof JAMADermatology, Lee andcolleagues6 present their contribution in assessing laboratory test monitoringduring isotretinoin therapy foracne,much-neededwork to create a strong foundation of information on which to establish clear recommendations for safe, high-value, costconscious care within our specialty practice. The focus of their article is isotretinoin therapy, a medicationcommonlyprescribedbydermatologists that todatehas had limited evidence-based recommendations for laboratory monitoring during treatment. A 2004 consensus statement published by the American Academy of Dermatology previously recommended periodic monitoring of serum triglyceride and cholesterol levels, as well as monitoring of transaminase levels, citing lack of consensus onmonitoring additional laboratory parameters.7 The isotretinoin package insert provides general guidelines for monitoring for lipid abnormalities before starting therapy and thenatweekly or biweekly intervals “until the lipid response to [isotretinoin] is established, which usually occurswithin 4weeks”8(p7) ormore frequently inhigh-riskpatients;weeklyor biweeklymonitoring forhepatotoxicity is also recommended.Monthly, as well as pretreatment and posttreatment, pregnancy testing for female patients is recommended in strict accordancewith the iPLEDGE registry system. Theactualpracticeofdermatologistsprescribing thismedication is poorly understood. Audience response data from attendees of a 2014AmericanAcademyofDermatology symposiumon isotretinoinmonitoring showedsignificant variability in thewaydermatologists screen.Before thepresentation, the audiencewas asked“what typeofmonitoringdoyoudo inpatients taking isotretinoin?”Of the 2343 anonymous electronic audience responses, 33% order baseline and monthly complete blood cell count, 26%order baseline andmonthly transaminase level testing, 13%order baseline andmonthly cholesterol panels, 2.4% order baseline and monthly renal function and/orelectrolyte level tests, 13.8%orderbaselineandmonthly pregnancy screening in female patients, 0.21% do not order monitoring laboratory tests inmale patients, and8.42%order laboratory test monitoring distinct from the options presented. These data suggest a clear and potentially important practice gap regarding isotretinoin monitoring. In their report, Lee and colleagues6 performed a systematic review looking at mean laboratory values and adverse events of isotretinoin therapy, capturing both analyzed data, as well as a narrative summary of the data excluded from the systematic review. The key finding was that although adverse effectswere noted for all laboratory tests (especially for cholesterol and triglyceride levels), severe or high-risk effects were rare. A critical consideration is that the authors reported changes inmeanvalueof laboratory test resultswithin a population although these are likely not themost clinically relevant to individual patient management. The second important findingwas that timingmatters; most laboratory test abnormalities were detected early (typically between 6 and 8 weeks) during the treatment period. There were no substantial late adverse effects of isotretinoin therapy, at least for the parameters measured—triglyceride and total cholesterol levels—corroboratingearlier studies, although laterdatawerenot available for all laboratory parameters examined. A thirdmajor finding of the study was that a wide range of discontinuation rateswasnoted (between0.71%and22.5%,mean5%). Importantly, thedirect relationshipbetweenabnormal laboratory test findings and therapy discontinuation was not reported. An earlier small study (515 patients, 574 isotretinoin treatment courses) published in 2013 showed that while isotretinoin adverse effects are relatively rare, the rate of laboratory test abnormality resulting in treatment discontinuation was exceedingly low—suggesting that screening may not ultimately affect the course of treatment.9 This is a central point because the latter study supports the notion of reduced laboratory testmonitoring as not only cost conscious but also providinghigh-valuecareduringisotretinointreatment.Inthepresentstudy, theauthorsused integrationof thesystematic review and thenarrative analyses to inform their clinical recommendations for individual patient care; in their conclusion, they argue that monthly laboratory screening is not indicated for patients receiving standard doses of isotretinoin. There are additional considerations in the interpretation of this study.Due to the rigorous inclusion criteria,most of the Related article page 35 Opinion

Syringomas are benign adnexal tumors with distinct histopathologic features, including the characteristic comma ("tadpole") shaped tail comprised of dilated, cystic eccrine ducts. Clinically, syringomas typically present in adolescent females predominantly in the periorbital region. They may present as solitary or multiple lesions, and more rare sites of involvement include the genitals, palms, scalp, and the chest. Over the past 50 years, there have been >800 reported cases of syringoma either alone or in conjunction with a systemic syndrome, most commonly Down syndrome. The primary aim of this systematic review is to discuss the clinical features and associations of syringomas with a focus on the patient with multiple syringomas. Its secondary aims are to explore pathophysiology with a focus on multiple syringomas and provide comprehensive data on both traditional and novel treatments. Importantly, multiple syringomas present across a broad clinical spectrum. Though noted in many textbooks to be related to tumor syndromes, the association of syringomas with inherited tumor syndromes is only rarely reported in the literature. Despite multiple reported cases of syringoma, the pathophysiology remains poorly understood and treatment continues to pose a significant challenge.

Nippostrongylus brasiliensis infection and ovalbumin-induced allergic lung pathology are highly interleukin (IL)-4/IL-13 dependent, but the contributions of IL-4/IL-13 from adaptive (T helper [Th]2 cells) and innate (eosinophil, basophils, and mast cells) immune cells remain unknown. Although required for immunoglobulin (Ig)E induction, IL-4/IL-13 from Th2 cells was not required for worm expulsion, tissue inflammation, or airway hyperreactivity. In contrast, innate hematopoietic cell–derived IL-4/IL-13 was dispensable for Th2 cell differentiation in lymph nodes but required for effector cell recruitment and tissue responses. Eosinophils were not required for primary immune responses. Thus, components of type 2 immunity mediated by IL-4/IL-13 are partitioned between T cell–dependent IgE and an innate non-eosinophil tissue component, suggesting new strategies for interventions in allergic immunity.

Background Grover disease is an acantholytic disorder that typically occurs on the trunk of older individuals, primarily white men, in association with heat and xerosis. Cases with extensive and/or atypical distributions have been reported. Objective To review the literature characterizing the population, morphology, associations, and disease course of extensive or atypical eruptions of Grover disease. Methods A systematic literature review identified 50 articles with 69 cases. Results Patient age ranged from 14 to 83 years (mean age, 56 ± 15), with 71% of patients being male and 29% female. Areas of involvement included the trunk (90%), upper and lower extremities (63% and 61%, respectively), face/scalp (28%), neck (21%), groin (11%), buttocks (8%), and axillae (6%). The most common associations included a history of malignancy (61%), recent chemotherapy (38%), and recent transplant (20%). Limitations Extensive cases with typical clinical morphology may not have been examined by biopsy or reported; thus, this review may have publication bias toward more severe or atypical presentations. Conclusions Greater variability exists among patients affected by extensive or atypical Grover disease than among those with typical disease. Malignancy is a common association, and there may be a role for immunosuppression in the pathogenesis of extensive or atypical Grover disease.