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.

Clinical effectiveness and cost-effectiveness of teledermatology: Where are we now, and what are the barriers to adoption?

There has been rapid growth in teledermatology over the past decade, and teledermatology services are increasingly being used to support patient care across a variety of care settings. Teledermatology has the potential to increase access to high-quality dermatologic care while maintaining clinical efficacy and cost-effectiveness. Recent expansions in telemedicine reimbursement from the Centers for Medicare & Medicaid Services (CMS) ensure that teledermatology will play an increasingly prominent role in patient care. Therefore, it is important that dermatologists be well informed of both the promises of teledermatology and the potential practice challenges a continuously evolving mode of care delivery brings. In this article, we will review the evidence on the clinical and cost-effectiveness of teledermatology and we will discuss system-level and practice-level barriers to successful teledermatology implementation as well as potential implications for dermatologists.

Future considerations for clinical dermatology in the setting of 21st century American policy reform: The Relative Value Scale Update Committee.

The American Medical Association-Specialty Society Relative Value Scale Update Committee, also known as the RUC, plays a critical role in assessing the relative value of physician services and procedures. This committee provides access for all physicians, including dermatologists, to the reimbursement process. Since the introduction of the Resource-Based Relative Value Scale by Medicare, the RUC has done important work to evaluate and refine reimbursement for physician services. The RUC recommendations have also led the Current Procedural Terminology (CPT) Editorial Panel to develop additional reimbursement codes as new procedures and services are developed. In this article (from the series Future Considerations for Clinical Dermatology in the Setting of 21st Century American Policy Reform), we will review the RUC, including its history and membership, the RUC update process, and a brief discussion of a few issues of particular importance to dermatologists.

Future considerations for clinical dermatology in the setting of 21st century American policy reform: Accountable Care Organizations.

An Accountable Care Organization (ACO) is a network of providers that collaborates to manage care and is financially incentivized to realize cost savings while also optimizing standards of care. Since its introduction as part of the 2010 Patient Protection and Affordable Care Act, ACOs have grown to include 16% of Medicare beneficiaries and currently represent Medicare's largest payment initiative. Although ACOs are still in the pilot phase with multiple structural models being assessed, incentives are being introduced to encourage specialist participation, and dermatologists will have the opportunity to influence both the cost savings and quality standard aspects of these organizations. In this article, part of a health care policy series targeted to dermatologists, we review what an ACO is, its relevance to dermatologists, and essential factors to consider when joining and negotiating with an ACO.

Future considerations for clinical dermatology in the setting of 21st century American policy reform: The Medicare Access and Children's Health Insurance Program Reauthorization Act and the Merit-based Incentive Payment System.

As the implementation of the Medicare Access and Children's Health Insurance Program Reauthorization Act begins, many dermatologists who provide Medicare Part B services will be subject to the reporting requirements of the Merit-based Incentive Payment System (MIPS). Clinicians subject to MIPS will receive a composite score based on performance across 4 categories: quality, advancing care information, improvement activities, and cost. Depending on their overall MIPS score, clinicians will be eligible for a positive or negative payment adjustment. Quality will replace the Physician Quality Reporting System and clinicians will report on 6 measures from a list of over 250 options. Advancing care information will replace meaningful use and will assess clinicians on activities related to integration of electronic health record technology into their practice. Improvement activities will require clinicians to attest to completion of activities focused on improvements in care coordination, beneficiary engagement, and patient safety. Finally, cost will be determined automatically from Medicare claims data. In this article, we will provide a detailed review of the Medicare Access and Children's Health Insurance Program Reauthorization Act with a focus on MIPS and briefly discuss the potential implications for dermatologists.

Future considerations for clinical dermatology in the setting of 21st century American policy reform: The Medicare Access and Children's Health Insurance Program Reauthorization Act and Alternative Payment Models in dermatology.

With the introduction of the Medicare Access and Children's Health Insurance Program Reauthorization Act, clinicians who are not eligible for an exemption must choose to participate in 1 of 2 new reimbursement models: the Merit-based Incentive Payment System or Alternative Payment Models (APMs). Although most dermatologists are expected to default into the Merit-based Incentive Payment System, some may have an interest in exploring APMs, which have associated financial incentives. However, for dermatologists interested in the APM pathway, there are currently no options other than joining a qualifying Accountable Care Organization, which make up only a small subset of Accountable Care Organizations overall. As a result, additional APMs relevant to dermatologists are needed to allow those interested in the APMs to explore this pathway. Fortunately, the Medicare Access and Children's Health Insurance Program Reauthorization Act establishes a process for new APMs to be approved and the creation of bundled payments for skin diseases may represent an opportunity to increase the number of APMs available to dermatologists. In this article, we will provide a detailed review of APMs under the Medicare Access and Children's Health Insurance Program Reauthorization Act and discuss the development and introduction of APMs as they pertain to dermatology.

Contribution of health care factors to the burden of skin disease in the United States.

The American Academy of Dermatology has developed an up-to-date national Burden of Skin Disease Report on the impact of skin disease on patients and on the US population. In this second of 3 manuscripts, data are presented on specific health care dimensions that contribute to the overall burden of skin disease. Through the use of data derived from medical claims in 2013 for 24 skin disease categories, these results indicate that skin disease health care is delivered most frequently to the aging US population, who are afflicted with more skin diseases than other age groups. Furthermore, the overall cost of skin disease is highest within the commercially insured population, and skin disease treatment primarily occurs in the outpatient setting. Dermatologists provided approximately 30% of office visit care and performed nearly 50% of cutaneous surgeries. These findings serve as a critical foundation for future discussions on the clinical importance of skin disease and the value of dermatologic care across the population.

The burden of skin disease in the United States.

Since the publication of the last US national burden of skin disease report in 2006, there have been substantial changes in the practice of dermatology and the US health care system. These include the development of new treatment modalities, marked increases in the cost of medications, increasingly complex payer rules and regulations, and an aging of the US population. Recognizing the need for up-to-date data to inform researchers, policy makers, public stakeholders, and health care providers about the impact of skin disease on patients and US society, the American Academy of Dermatology produced a new national burden of skin disease report. Using 2013 claims data from private and governmental insurance providers, this report analyzed the prevalence, cost, and mortality attributable to 24 skin disease categories in the US population. In this first of 3 articles, the presented data demonstrate that nearly 85 million Americans were seen by a physician for at least 1 skin disease in 2013. This led to an estimated direct health care cost of $75 billion and an indirect lost opportunity cost of $11 billion. Further, mortality was noted in half of the 24 skin disease categories.

Early Detection and Prognostic Assessment of Cutaneous Melanoma: Consensus on Optimal Practice and the Role of Gene Expression Profile Testing.

Importance: Therapy for advanced melanoma has transformed during the past decade, but early detection and prognostic assessment of cutaneous melanoma (CM) remain paramount goals. Best practices for screening and use of pigmented lesion evaluation tools and gene expression profile (GEP) testing in CM remain to be defined. Objective: To provide consensus recommendations on optimal screening practices and prebiopsy diagnostic, postbiopsy diagnostic, and prognostic assessment of CM. Evidence Review: Case scenarios were interrogated using a modified Delphi consensus method. Melanoma panelists (n = 60) were invited to vote on hypothetical scenarios via an emailed survey (n = 42), which was followed by a consensus conference (n = 51) that reviewed the literature and the rationale for survey answers. Panelists participated in a follow-up survey for final recommendations on the scenarios (n = 45). Findings: The panelists reached consensus (≥70% agreement) in supporting a risk-stratified approach to melanoma screening in clinical settings and public screening events, screening personnel recommendations (self/partner, primary care provider, general dermatologist, and pigmented lesion expert), screening intervals, and acceptable appointment wait times. Participants also reached consensus that visual and dermoscopic examination are sufficient for evaluation and follow-up of melanocytic skin lesions deemed innocuous. The panelists reached consensus on interpreting reflectance confocal microscopy and some but not all results from epidermal tape stripping, but they did not reach consensus on use of certain pigmented lesion evaluation tools, such as electrical impedance spectroscopy. Regarding GEP scores, the panelists reached consensus that a low-risk prognostic GEP score should not outweigh concerning histologic features when selecting patients to undergo sentinel lymph node biopsy but did not reach consensus on imaging recommendations in the setting of a high-risk prognostic GEP score and low-risk histology and/or negative nodal status. Conclusions and Relevance: For this consensus statement, panelists reached consensus on aspects of a risk-stratified approach to melanoma screening and follow-up as well as use of visual examination and dermoscopy. These findings support a practical approach to diagnosing and evaluating CM. Panelists did not reach consensus on a clearly defined role for GEP testing in clinical decision-making, citing the need for additional studies to establish the clinical use of existing GEP assays.

Multiple Spitz nevi: a clinicopathologic study of 9 patients.

BACKGROUND: Whether agminated, within a speckled lentiginous nevus/nevus spilus, or randomly scattered, multiple Spitz nevi (SN), defined as two or more SN, is a rare entity. OBJECTIVE: The aim of this study was to evaluate patients presenting with multiple SN. METHODS: We conducted a search of the database of the Yale Spitzoid Neoplasm Repository from May 1990 to June 2010 to identify patients with multiple SN. The clinicopathologic features of these patients are the subject of this study. RESULTS: Nine patients with a total of 38 SN were identified retrospectively during a 20-year period. Patients ranged in age from 2 to 34 years (mean 22 years). The total number of histologically confirmed SN in each patient ranged from 2 to 13 (mean 5; median 2). These SN were more commonly diagnosed clinically as atypical nevi rather than as SN. The histopathologic findings ranged from those of a classic SN to an atypical Spitzoid neoplasm that was difficult to distinguish from a Spitzoid melanoma. Of the SN examined histologically in this series, the majority (53%) showed no atypical histopathologic features and none had recurred after a re-excision. LIMITATIONS: The low number of patients with multiple SN is a limitation. CONCLUSION: The presence of multiple SN is a rare phenomenon. The majority display histopathologic characteristics of classic SN or a few atypical features.

AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: a report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery.

The appropriate use criteria process synthesizes evidence-based medicine, clinical practice experience, and expert judgment. The American Academy of Dermatology in collaboration with the American College of Mohs Surgery, the American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery has developed appropriate use criteria for 270 scenarios for which Mohs micrographic surgery (MMS) is frequently considered based on tumor and patient characteristics. This document reflects the rating of appropriateness of MMS for each of these clinical scenarios by a ratings panel in a process based on the appropriateness method developed by the RAND Corp (Santa Monica, CA)/University of California-Los Angeles (RAND/UCLA). At the conclusion of the rating process, consensus was reached for all 270 (100%) scenarios by the Ratings Panel, with 200 (74.07%) deemed as appropriate, 24 (8.89%) as uncertain, and 46 (17.04%) as inappropriate. For the 69 basal cell carcinoma scenarios, 53 were deemed appropriate, 6 uncertain, and 10 inappropriate. For the 143 squamous cell carcinoma scenarios, 102 were deemed appropriate, 7 uncertain, and 34 inappropriate. For the 12 lentigo maligna and melanoma in situ scenarios, 10 were deemed appropriate, 2 uncertain, and 0 inappropriate. For the 46 rare cutaneous malignancies scenarios, 35 were deemed appropriate, 9 uncertain, and 2 inappropriate. These appropriate use criteria have the potential to impact health care delivery, reimbursement policy, and physician decision making on patient selection for MMS, and aim to optimize the use of MMS for scenarios in which the expected clinical benefit is anticipated to be the greatest. In addition, recognition of those scenarios rated as uncertain facilitates an understanding of areas that would benefit from further research. Each clinical scenario identified in this document is crafted for the average patient and not the exception. Thus, the ultimate decision regarding the appropriateness of MMS should be determined by the expertise and clinical experience of the physician.

AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: a report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery.

The appropriate use criteria process synthesizes evidence-based medicine, clinical practice experience, and expert judgment. The American Academy of Dermatology in collaboration with the American College of Mohs Surgery, the American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery has developed appropriate use criteria for 270 scenarios for which Mohs micrographic surgery (MMS) is frequently considered based on tumor and patient characteristics. This document reflects the rating of appropriateness of MMS for each of these clinical scenarios by a ratings panel in a process based on the appropriateness method developed by the RAND Corp (Santa Monica, CA)/University of California-Los Angeles (RAND/UCLA). At the conclusion of the rating process, consensus was reached for all 270 (100%) scenarios by the Ratings Panel, with 200 (74.07%) deemed as appropriate, 24 (8.89%) as uncertain, and 46 (17.04%) as inappropriate. For the 69 basal cell carcinoma scenarios, 53 were deemed appropriate, 6 uncertain, and 10 inappropriate. For the 143 squamous cell carcinoma scenarios, 102 were deemed appropriate, 7 uncertain, and 34 inappropriate. For the 12 lentigo maligna and melanoma in situ scenarios, 10 were deemed appropriate, 2 uncertain, and 0 inappropriate. For the 46 rare cutaneous malignancies scenarios, 35 were deemed appropriate, 9 uncertain, and 2 inappropriate. These appropriate use criteria have the potential to impact health care delivery, reimbursement policy, and physician decision making on patient selection for MMS, and aim to optimize the use of MMS for scenarios in which the expected clinical benefit is anticipated to be the greatest. In addition, recognition of those scenarios rated as uncertain facilitates an understanding of areas that would benefit from further research. Each clinical scenario identified in this document is crafted for the average patient and not the exception. Thus, the ultimate decision regarding the appropriateness of MMS should be determined by the expertise and clinical experience of the physician.

"Clark/dysplastic" nevi with florid fibroplasia associated with pseudomelanomatous features.

BACKGROUND: Melanocytic nevi may exhibit histologic features in common with cutaneous melanoma, creating diagnostic difficulties. OBJECTIVE: We sought to assess the clinical behavior of melanocytic nevi with pseudomelanomatous features in association with dermal fibrosis. METHODS: Forty-two melanocytic nevi with pronounced fibrosis and associated pseudomelanomatous changes were collected and studied clinically and histologically. RESULTS: The fibrosis was centrally located and laminated in appearance. It imparted a trizonal appearance: a junctional component with prominent single cells and/or irregular nests, underlying fibrosis, and a mature dermal component. No recurrence or metastases were evident over an average follow-up period of 2 years. LIMITATIONS: The follow-up period was short. CONCLUSIONS: The central location and laminated appearance of the fibrosis suggest that this may represent the extreme end of a spectrum of fibroplastic changes in "Clark/dysplastic" nevi. Adjacent features of "Clark/dysplastic" nevi and limitation of pseudomelanomatous features to the perifibrotic focus are important in accurately identifying these lesions. Although melanocytic nevi with exaggerated fibroplasia may show foci with melanoma-like features, they do not appear to exhibit aggressive clinical behavior.