A core domain set for pyoderma gangrenosum trial outcomes: an international eDelphi and consensus study from the UPGRADE initiative.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare ulcerative skin condition with no current standardized outcomes or outcome measures. With a rich investigational therapeutic pipeline, standardization of outcomes and improvement of data quality and interpretability will promote the appropriate and consistent evaluation of potential new therapies. Core outcome sets (COS) are agreed, standardized sets of outcomes that represent the minimum that should be measured and reported in all clinical trials of a specific condition. OBJECTIVES: To identify and reach a consensus on which domains (what to be measured) should be included in the Understanding Pyoderma Gangrenosum: Review and Analysis of Disease Effects (UPGRADE) core domain set for clinical trials in PG. METHODS: Collaborative discussions between patients and PG experts, and a systematic review of the literature identified items and prospective domains. A three-round international eDelphi exercise was performed to prioritize the domains and refine the provisional items (consensus: ≥ 70% of participants rating a domain as 'extremely important' and < 15% of participants voting 'not important'), followed by an international meeting to reach consensus on the core domain set (consensus: < 30% disagreement). Item-generation discussions and consensus meetings were hosted via online videoconferences. The eDelphi exercise and consensus voting were performed using Qualtrics survey software. Participants were adults with PG, healthcare professionals, researchers and industry representatives. RESULTS: Collaborative discussions and systematic reviews yielded 115 items, which were distilled into 15 prospective domains. The eDelphi exercise removed the three lowest-priority domains ('laboratory tests', 'treatment costs' and 'disease impact on family') and ranked 'pain', 'quality of life' and 'physical symptoms' as the highest-priority prospective domains. Consensus was reached on the domains of 'pain', 'quality of life' and 'clinical signs'. The domain of 'disease course/disease progression' narrowly failed to reach consensus for inclusion in the core set (32% of participants voted 'no'). Refinement of this domain definition will be required and presented for consideration at future consensus meetings. CONCLUSIONS: The UPGRADE core domain set for clinical trials in PG has been agreed by international multistakeholder consensus. Future work will develop and/or select outcome measurement instruments for these domains to establish a COS.

Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms. Part II diagnosis and management.

Drug-induced hypersensitivity syndrome, also known as drug reaction with eosinophilia and systemic symptoms, is a severe cutaneous adverse reaction characterized by an exanthem, fever, and hematologic and visceral organ involvement. The differential diagnosis includes other cutaneous adverse reactions, infections, inflammatory and autoimmune diseases, and neoplastic disorders. Three sets of diagnostic criteria have been proposed; however, consensus is lacking. The cornerstone of management is immediate discontinuation of the suspected drug culprit. Systemic corticosteroids remain first-line therapy, but the literature on steroid-sparing agents is expanding. Longitudinal evaluation for sequelae is recommended. Adjunctive tests for risk stratification and drug culprit identification remain under investigation. Part II of this continuing medical education activity begins by exploring the differential diagnosis and diagnosis of drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms and concludes with an evidence-based overview of evaluation and treatment.

Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms. Part I. Epidemiology, pathogenesis, clinicopathological features, and prognosis.

Drug-induced hypersensitivity syndrome (DiHS), also known as drug reaction with eosinophilia and systemic symptoms (DRESS), is a severe cutaneous adverse reaction (SCAR) characterized by an exanthem, fever, and hematologic and visceral organ involvement. Anticonvulsants, antibiotics, and allopurinol are the most common triggers. The pathogenesis involves a complex interplay between drugs, viruses, and the immune system primarily mediated by T-cells. DiHS/DRESS typically presents with a morbilliform eruption 2-6 weeks after drug exposure, and is associated with significant morbidity, mortality, and risk of relapse. Long-term sequelae primarily relate to organ dysfunction and autoimmune diseases. Part I of this continuing medical education activity on DiHS/DRESS provides an update on epidemiology, novel insights into pathogenesis, and a description of clinicopathological features and prognosis.

Evaluation of a pilot forensic dermatology curriculum in the USA.

Physician participation in asylum medicine through forensic medical evaluations increases the likelihood that asylum seekers are granted legal status. Based on a review of existing literature and input from content experts, we designed and implemented a forensic dermatology curriculum for dermatology residents at University of California, San Francisco. Our pilot curriculum led to improvements in learners' attitudes regarding physician participation in asylum medicine and their confidence in describing skin findings of torture or abuse.

Treatment of pyoderma gangrenosum: A multicenter survey-based study assessing satisfaction and quality of life.

Pyoderma gangrenosum (PG) lacks consensus regarding treatment, and no prior studies assess treatment satisfaction in PG. The objective of this study was to determine patient-reported satisfaction in the treatment of PG, and associations with satisfaction. Methodology was a multicenter 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 $50 000-99 000; 79.0 ± 14.6 for $100 000+) 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.

Diagnosis and management of peristomal pyoderma gangrenosum: A systematic review.

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.

Atypical features and systemic associations in extensive cases of Grover disease: A systematic review.

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.

Communication strategies for a successful inpatient dermatology consultative service: a narrative review.

Inpatient dermatology consultative services care for hospitalized patients with skin disease in collaboration with the primary inpatient team. Effective, efficient communication is important. A consultation service must develop strong relationships with primary inpatient teams requesting consults in order to provide optimal patient care. Prior studies have identified effective communication practices for inpatient consultative services. This narrative review provides a summary of effective communication practices for an inpatient dermatology consultation service organized into 5 domains: (1) features of the initial consult request; (2) best practices in responding to the initial consult; (3) effective communication of recommendations; (4) interventions to improve consultations; and (5) handling curbside consultations. Recommendations include identifying the specific reason for consult; establishing urgency; secure sharing of sensitive clinical information such as photographs; ensuring timely responses; providing clear yet brief documentation of the differential diagnosis, problem list, final diagnosis and recommendations; and limiting curbside consultations. Future studies are needed to validate effective strategies to enhance communication practices within an inpatient dermatology consultative service.

Optimizing education on the inpatient dermatology consultative service.

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).

Characteristics of research tracks in dermatology residency programs: a national survey.

Pursuing research is encouraged in dermatology residency programs. Some programs offer specific research or investigative tracks. Currently, there is little data on the structure or scope of research tracks in dermatology residency programs. An anonymous online survey was distributed to the Association of Professors of Dermatology listserve in 2016. Program directors of dermatology residency programs in the United States were asked to participate and 38 of the 95 program directors responded. The survey results confirmed that a 2+2 research track, which is two years of clinical training followed by two years of research, was the most common investigator trackmodel and may promote an academic career at the resident's home institution. Further studies will help determine the most effective research track models to promote long-term outcomes.

Dermatology Medical Education: A Multicenter Survey Study of the Undergraduate Perspective of the Dermatology Clinical Clerkship.

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.

Evaluation and management of the patient with multiple syringomas: A systematic review of the literature.

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.

Evidence-based approach to cutaneous hyperandrogenism in women.

Hirsutism, acne, and androgenetic alopecia are classically considered signs of cutaneous hyperandrogenism (CHA). These common skin findings have significant impacts on the quality of patients' lives and pose the diagnostic challenge of excluding underlying disorders. Many with CHA have normal serum androgen levels. Hirsutism is more strongly associated with hyperandrogenism than are acne or androgenetic alopecia. Variable association of CHA with hyperandrogenemia results from the complexity of the underlying pathophysiology, including factors local to the pilosebaceous unit. CHA often occurs in the setting of polycystic ovary syndrome, the most common disorder of hyperandrogenism, but can also present in uncommon conditions, including nonclassic adrenal hyperplasia and androgen-producing tumors. A thorough history and full skin examination are important to guide appropriate diagnostic evaluation. Oral contraceptive pills with or without antiandrogens can provide therapeutic benefit for hirsutism and acne. Medical options for androgenetic alopecia remain limited. Multidisciplinary approaches may be needed given endocrine, metabolic, reproductive, and psychiatric disorders associated with CHA. More high-quality studies into the mechanisms of CHA and the benefits of antiandrogenic therapies are needed. We provide an evidence-based review of key diagnostic and therapeutic considerations in the treatment of women with CHA.

Mycoplasma pneumoniae-induced rash and mucositis as a syndrome distinct from Stevens-Johnson syndrome and erythema multiforme: a systematic review.

BACKGROUND: Mycoplasma pneumoniae infection is associated with extrapulmonary complications, including mucocutaneous eruptions. These eruptions, which have been termed either "Stevens-Johnson syndrome" or "erythema multiforme" in the literature, may differ from drug-induced Stevens-Johnson syndrome or viral-associated erythema multiforme. OBJECTIVE: We sought to review the literature characterizing morphology and disease course of M pneumoniae-associated mucocutaneous disease. METHODS: A comprehensive literature search identified 95 articles with 202 cases. RESULTS: Patients were often young (mean age: 11.9 years) and male (66%). Cutaneous involvement ranged from absent (34%), to sparse (47%), to moderate (19%). Oral, ocular, and urogenital mucositis was reported in 94%, 82%, and 63% of cases, respectively. Treatments included antibiotics (80%), systemic corticosteroids (35%), supportive care alone (8%), and/or intravenous immunoglobulin (8%). Complications included mucosal damage (10%), cutaneous scarring (5.6%), recurrence (8%), and mortality (3%). LIMITATIONS: Mild cases may not have been published; thus this review may have a bias toward more severe disease. CONCLUSION: M pneumoniae-associated mucocutaneous disease has prominent mucositis and sparse cutaneous involvement, although cutaneous involvement varies. Because of the distinct morphology, mild disease course, and potentially important clinical implications regarding treatment, we propose a revision of the nomenclature system and suggest the term "Mycoplasma-induced rash and mucositis" for these cases.

Inpatient dermatology consultation aids diagnosis of cellulitis among hospitalized patients: A multi-institutional analysis.

BACKGROUND: Given its nonspecific physical examination findings, accurately distinguishing cellulitis from a cellulitis mimicker (pseudocellulitis) is challenging. OBJECTIVE: We sought to investigate the national incidence of cellulitis misdiagnosis among inpatients. METHODS: We conducted a retrospective review of inpatient dermatology consultations at Massachusetts General Hospital, University of Alabama at Birmingham Medical Center, University of California Los Angeles Medical Center, and University of California San Francisco Medical Center in 2008. All consults requested for the evaluation of cellulitis were included. The primary outcomes were determining the incidence of cellulitis misdiagnosis, evaluating the prevalence of associated risk factors, and identifying common pseudocellulitides. RESULTS: Of the 1430 inpatient dermatology consultations conducted in 2008, 74 (5.17%) were requested for the evaluation of cellulitis. In all, 55 (74.32%) patients evaluated for cellulitis were given a diagnosis of pseudocellulitis. There was no statistically significant difference in the rate of misdiagnosis across institutions (P = .12). Patient demographics and associated risk factor prevalence did not statistically vary in patients given a diagnosis of cellulitis versus those with pseudocellulitis (P > .05). LIMITATIONS: This study was unable to evaluate all patients admitted with cellulitis and was conducted at tertiary care centers, which may affect the generalizability of the results. CONCLUSIONS: Cellulitis is commonly misdiagnosed in the inpatient setting. Involving dermatologists may improve diagnostic accuracy and decrease unnecessary antibiotic use.