Clinical Characteristics, Disease Course, and Outcomes of Patients With Acute Generalized Exanthematous Pustulosis in the US.

Importance: Acute generalized exanthematous pustulosis (AGEP) is a rare, severe cutaneous adverse reaction associated with systemic complications. Currently available data are largely limited to small retrospective case series. Objective: To describe the clinical characteristics, disease course, and outcomes of a heterogeneous group of patients with AGEP across the US. Design, Setting, and Participants: A retrospective review of a case series of patients was conducted from January 1, 2000, through July 31, 2020. All 340 included cases throughout 10 academic health systems in the US were scored retrospectively using the EuroSCAR scoring system, and patients with a score corresponding to probable or definite AGEP and aged 18 years or older were included. Main Outcomes and Measures: Patient demographic characteristics, clinical course, suspected causative agent, treatment, and short- and long-term outcomes. Results: Most of the 340 included patients were women (214 [62.9%]), White (206 [60.6%]), and non-Hispanic (239 [70.3%]); mean (SD) age was 57.8 (17.4) years. A total of 154 of 310 patients (49.7%) had a temperature greater than or equal to 38.0 °C that lasted for a median of 2 (IQR, 1-4) days. Of 309 patients, 263 (85.1%) developed absolute neutrophilia and 161 patients (52.1%) developed either absolute or relative eosinophilia. Suspected causes of AGEP were medications (291 [85.6%]), intravenous contrast agents (7 [2.1%]), infection (3 [0.9%]), or unknown (39 [11.5%]). In 151 cases in which a single medication was identified, 63 (41.7%) were ß-lactam antimicrobials, 51 (33.8%) were non-ß-lactam antimicrobials, 9 (6.0%) were anticonvulsants, and 5 (3.3%) were calcium channel blockers. The median time from medication initiation to AGEP start date was 3 (IQR, 1-9) days. Twenty-five of 298 patients (8.4%) had an acute elevation of aspartate aminotransferase and alanine aminotransferase levels, with a peak at 6 (IQR, 3-9) days. Twenty-five of 319 patients (7.8%) experienced acute kidney insufficiency, with the median time to peak creatinine level being 4 (IQR, 2-5) days after the AGEP start date. Treatments included topical corticosteroids (277 [81.5%], either alone or in combination), systemic corticosteroids (109 [32.1%]), cyclosporine (10 [2.9%]), or supportive care only (36 [10.6%]). All-cause mortality within 30 days was 3.5% (n = 12), none of which was suspected to be due to AGEP. Conclusions and Relevance: This retrospective case series evaluation of 340 patients, the largest known study cohort to date, suggests that AGEP onset is acute, is usually triggered by recent exposure to an antimicrobial, may be associated with liver or kidney complications in a minority of patients, and that discontinuation of the triggering treatment may lead to improvement or resolution.

Challenges of securing insurance approval for oral tofacitinib for the treatment of alopecia areata: a multi-institution retrospective review.

Alopecia areata (AA) is a psychologically distressing disorder for which few reliable treatments exist. Although oral tofacitinib has demonstrated efficacy in treating AA, it is not approved by the Food and Drug Administration (FDA) for this indication. To investigate and identify the challenges associated with securing insurance approval for oral tofacitinib for AA. We conducted a retrospective review of patient records from two academic medical centers to identify patients with AA in whom insurance approval was sought for oral tofacitinib from 2015-2019. We recorded information on prior authorization (PA) submissions, appeals, and peer-to-peer reviews. We noted whether patients were documented to experience negative impact on mood/QOL or suicidal ideation (SI) due to their disease. We identified 37 patients in whom insurance approval was sought for oral tofacitinib for the treatment of AA. PAs were initially denied for 36/37 (97%) patients. The most commonly cited reason for denial was "tofacitinib not covered for AA/off-label medication use" (n = 26/36; 72%). 26/37 (70%) patients ultimately failed to obtain coverage. Of the 11 (30%) patients who obtained coverage, 10 (91%) were privately insured, 0 (0%) had Medicare and 1 (9%) had Medicaid. 13 patients (34%) experienced documented diminished QOL/mood (including SI) due to their disease burden; 6/13 (46%) of these patients eventually secured insurance approval. Lack of FDA approval of oral tofacitinib for the treatment of AA creates challenges in caring for patients with this disease. Policymakers should consider the negative implications lack of FDA approval may have for patients with recalcitrant dermatologic conditions.

Evaluation of a Case Series of Patients With Generalized Pustular Psoriasis in the United States.

IMPORTANCE: Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data. OBJECTIVE: To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States. DESIGN, SETTING, AND PARTICIPANTS: A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018. MAIN OUTCOMES AND MEASURES: The primary outcome is to describe the patient characteristics, associated medical comorbidities, treatment patterns complications, and GPP-specific health care utilization. RESULTS: Sixty-seven of 95 patients (70.5%) were women (mean age, 50.3 years [SD, 16.1 years]). In the initial encounter, 35 patients (36.8%) were hospitalized and 64 (67.4%) were treated with systemic therapies. In total, more than 20 different systemic therapies were tried. During the follow-up period, 19 patients (35.8%) reported hospitalizations at a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6). Women had a decreased risk of an emergency department or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83). CONCLUSIONS AND RELEVANCE: Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.

Evaluation of a Case Series of Patients With Palmoplantar Pustulosis in the United States.

IMPORTANCE: Palmoplantar pustulosis (PPP) is a is a chronic, orphan disease with limited epidemiological data. OBJECTIVE: To describe the clinical characteristics, treatments, longitudinal disease course, and health care utilization in adults with PPP across the US. DESIGN, SETTING, AND PARTICIPANTS: This retrospective, longitudinal case series from 20 academic dermatology practices in the US included a consecutive sample of 197 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for PPP between January 1, 2007, and December 31, 2018. Data analysis was performed June 2020 to December 2020. MAIN OUTCOMES AND MEASURES: The primary outcome was to describe the patient characteristics, associated medical comorbidities, treatment patterns, complications, and PPP-specific health care utilization. RESULTS: Of 197 patients, 145 (73.6%) were female, and the mean (SD) age at presentation was 53.0 (12.6) years, with a mean (SD) follow-up time of 22.1 (28.0) months. On initial presentation, 95 (48.2%) patients reported skin pain, and 39 (19.8%) reported difficulty using hands and/or feet. Seventy patients (35.5%) were treated with systemic treatments, and use of more than 20 different systemic therapies was reported. In patients with at least 6 months of follow-up (n = 128), a median (IQR) of 3.7 (4-10) dermatology visits per year were reported; 24 (18.8%) patients had 5 or more visits during the study period. CONCLUSIONS AND RELEVANCE: In this case series, PPP was associated with persistent symptoms, continued health care utilization, and a lack of consensus regarding effective treatments, emphasizing the unmet medical need in this population. Additional research is necessary to understand treatment response in these patients.

OnabotulinumtoxinA for Systemic Sclerosis-associated Raynaud's Phenomenon: A Multi-Institutional Study on Accessibility and Effectiveness.

Raynaud’s phenomenon (RP), a common presenting symptom of systemic sclerosis (SSc), is a painful and debilitating condition of the digits caused by increased vascular reactivity.1,2.

Prevalence of Misrepresentation of Nonphysician Clinicians at Dermatology Clinics.

INTRODUCTION: To evaluate the use of inaccurate terminology used by dermatology practices to describe the training and qualifications of their nonphysician clinicians (NPCs) when new patients are booking appointments. METHODS: Clinics were randomly selected and called to determine the first available appointment for a new patient with a new and changing mole. If the receptionist confirmed the first-offered appointment was with an NPC, the encounter was included in this study. If receptionists used inaccurate terminology to describe the NPCs and their qualifications, this instance was recorded along with the specific language that they used. RESULTS: A total of 344 unique dermatology clinics were contacted on February 27, 2020, in 25 states. Phone calls at 128 clinics (37.2%) met our inclusion criterion. Inaccurate language was used to describe NPCs at 23 (18%) unique clinic locations across 12 states, with "dermatologist," "doctor," "physician," and "board-certified" being used to describe NPCs as the most common inaccurate terms. CONCLUSION: These findings demonstrate that front office staff at dermatology clinics use inaccurate and potentially misleading terminology to refer to NPCs working in their clinics. While we cannot establish whether this is intentional or due to a lack of training, additional focus should be placed on accurately representing provider qualifications to patients.

Comparison of Shave and Punch Biopsy Utilization Among Dermatology Practices.

In 2019, the 2 Current Procedural Terminology (CPT) codes for skin biopsies were replaced with 6 new CPT codes to allow for technique specification and differential reimbursement. We sought to evaluate whether the concurrent decrease in reimbursement for shave biopsies and increase in reimbursement for punch biopsies led to utilization changes. We examined shave and punch biopsies submitted for pathologic examination at 3 academic centers in May 2018 and May 2019. We performed χ2 tests to evaluate for changes in the ratio of biopsy utilization over time, with subgroup analyses by practice setting and provider type. Totals included 11,785 (12.11% punch) and 11,291 (12.08% punch) biopsies submitted in May 2018 and May 2019, respectively. Our results demonstrate small yet important changes in biopsy use patterns within the context of recent reimbursement changes when analyzing academic and private practices separately. Although small in magnitude, this change in behavior may have a substantial impact when extrapolated to behavior across the nation.

Nicotinamide for Keratinocyte Carcinoma Chemoprevention: A Nationwide Survey of Mohs Surgeons.

BACKGROUND: Nicotinamide has been shown to reduce keratinocyte carcinoma (KC) in high-risk patients in a randomized controlled trial setting. Data on nicotinamide's use for KC prevention are limited. OBJECTIVE: To characterize nicotinamide prescribing patterns among Mohs surgeons. METHODS AND MATERIALS: We surveyed 1,500 members of the American College of Mohs Surgeons regarding their demographics, use of nicotinamide, and safety perceptions. We performed multiple logistic regression analysis to evaluate correlations between physician characteristics and nicotinamide prescribing habits. RESULTS: 76.9% of survey respondents recommend nicotinamide for KC prevention. Twenty percent of respondents have recommended nicotinamide to more than 100 patients in the past year. Forty-five percent of respondents report a duration of use of 2 years or more in their patients. 63.8% of respondents had no concerns over nicotinamide's safety with long-term use. Individuals who answered "yes," "maybe," or "uncertain" to having safety concerns over long-term nicotinamide use and individuals in practice for more than 10 years were less likely (odds ratio [OR] 0.30, 95% confidence interval [CI] 0.13-0.71 and OR 0.20, 95% CI 0.05, 0.82, respectively) to have recommended nicotinamide to patients for KC prevention. CONCLUSION: Given the widespread nicotinamide use among Mohs surgeons, additional studies on nicotinamide cost-effectiveness, safety, and use patterns are needed.

Insurance Acceptance, Appointment Wait Time, and Dermatologist Access Across Practice Types in the US.

Importance: In the 15 years since dermatology access was last investigated on a national scale, the practice landscape has changed with the rise of private equity (PE) investment and increased use of nonphysician clinicians (NPCs). Objective: To determine appointment success and wait times for patients with various insurance types at clinics with and without PE ownership. Design, Setting, and Participants: In this study, PE-owned US clinics were randomly selected and matched with 2 geographically proximate clinics without PE ownership. Researchers called each clinic 3 times over a 5-day period to assess appointment/clinician availability for a fictitious patient with a new and changing mole. The 3 calls differed by insurance type specified, which were Blue Cross Blue Shield (BCBS) preferred provider organization, Medicare, or Medicaid. Main Outcomes and Measures: Appointment success and wait times among insurance types and between PE-owned clinics and control clinics. Secondary outcomes were the provision of accurate referrals to other clinics when appointments were denied and clinician and next-day appointment availability. Results: A total of 1833 calls were made to 204 PE-owned and 407 control clinics without PE ownership across 28 states. Overall appointment success rates for BCBS, Medicare, and Medicaid were 96%, 94%, and 17%, respectively. Acceptance of BCBS (98.5%; 95% CI, 96%-99%; P = .03) and Medicare (97.5%; 95% CI, 94%-99%; P = .02) were slightly higher at PE-owned clinics (compared with 94.6% [95% CI, 92%-96%] and 92.8% [95% CI, 90%-95%], respectively, at control clinics). Wait times (median days, interquartile range [IQR]) were similar for patients with BCBS (7 days; IQR, 2-22 days) and Medicare (7 days; IQR, 2-25 days; P > .99), whereas Medicaid patients waited significantly longer (13 days; IQR, 4-33 days; P = .002). Clinic ownership did not significantly affect wait times. Private equity-owned clinics were more likely than controls to offer a new patient appointment with an NPC (80% vs 63%; P = .001) and to not have an opening with a dermatologist (16% vs 6%; P < .001). Next-day appointment availability was greater at PE-owned clinics than controls (30% vs 21%; P = .001). Conclusions and Relevance: Patients with Medicaid had significantly lower success in obtaining appointments and significantly longer wait times regardless of clinic ownership. Although the use of dermatologists and NPCs was similar regardless of clinic ownership, PE-owned clinics were more likely than controls to offer new patient appointments with NPCs.

Clinical informatics subspecialists: characterizing a novel evolving workforce.

BACKGROUND: The growing complexity of data systems in health care has precipitated increasing demand for clinical informatics subspecialists. The first board certification exam for the clinical informatics subspecialty was offered in 2013. Characterizing trends in this novel workforce is important to inform its development. METHODS: We conducted an exploratory analysis of American Board of Medical Specialties data on individuals certified in clinical informatics from 2013 to 2019 to review trends and demographic characteristics of current subspecialists. RESULTS: 2018 physicians were certified in clinical informatics from 2013 to 2019. The annual number of awarded certifications declined after 2016. The majority of primary certifications held by clinical informaticians were in broad-based medical specialties relative to primarily procedural specialties. CONCLUSIONS: Disparities may exist within the clinical informatics physician workforce with respect to primary specialty certifications and geographic distribution. There remains a need for the creation of fellowship programs to sustain the growth of this workforce.