A Multicenter Retrospective Cohort Study to Characterize Patients Hospitalized With Multisystem Inflammatory Syndrome in Adults and Coronavirus Disease 2019 in the United States, 2020-2021.

BACKGROUND: The diagnosis of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated multisystem inflammatory syndrome in adults (MIS-A) requires distinguishing it from acute coronavirus disease 2019 (COVID-19) and may affect clinical management. METHODS: In this retrospective cohort study, we applied the US Centers for Disease Control and Prevention case definition to identify adults hospitalized with MIS-A at 6 academic medical centers from 1 March 2020 to 31 December 2021. Patients MIS-A were matched by age group, sex, site, and admission date at a 1:2 ratio to patients hospitalized with acute symptomatic COVID-19. Conditional logistic regression was used to compare demographic characteristics, presenting symptoms, laboratory and imaging results, treatments administered, and outcomes between cohorts. RESULTS: Through medical record review of 10 223 patients hospitalized with SARS-CoV-2-associated illness, we identified 53 MIS-A cases. Compared with 106 matched patients with COVID-19, those with MIS-A were more likely to be non-Hispanic black and less likely to be non-Hispanic white. They more likely had laboratory-confirmed COVID-19 ≥14 days before hospitalization, more likely had positive in-hospital SARS-CoV-2 serologic testing, and more often presented with gastrointestinal symptoms and chest pain. They were less likely to have underlying medical conditions and to present with cough and dyspnea. On admission, patients with MIS-A had higher neutrophil-to-lymphocyte ratio and higher levels of C-reactive protein, ferritin, procalcitonin, and D-dimer than patients with COVID-19. They also had longer hospitalization and more likely required intensive care admission, invasive mechanical ventilation, and vasopressors. The mortality rate was 6% in both cohorts. CONCLUSIONS: Compared with patients with acute symptomatic COVID-19, adults with MIS-A more often manifest certain symptoms and laboratory findings early during hospitalization. These features may facilitate diagnosis and management.

Linear IgA bullous dermatosis in the setting of angioimmunoblastic T-cell lymphoma.

We report an 80-year-old male developing linear IgA bullous dermatosis (LAD) in the setting of angioimmunoblastic T-cell lymphoma (AITL). This phenomenon is rare, as only three cases have been described in the literature. The pathophysiologic process can be attributed to dysregulation in somatic hypermutation and the expression of chemokine receptor 5 in AITL, contributing to increased IgA. Immunoglobulin production resulting from clonal plasma cell expansion may be because of the B-cell promotional effect by neoplastic follicular helper T-cells. Beyond providing a pathophysiologic platform for AITL-associated LAD, we also briefly summarized prior cases. This report demonstrates the importance of considering LAD in the differential diagnosis for patients with a bullous eruption in the setting of AITL.

Adverse pregnancy and maternal outcomes in women with hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) disproportionately affects women of childbearing age. However, pregnancy and maternal outcomes for women with HS are unknown. OBJECTIVE: To compare risk of adverse pregnancy and maternal outcomes among women with and without HS and to evaluate the influence of comorbid conditions. METHODS: Retrospective cohort analysis between January 1, 2011, and September 30, 2015. RESULTS: Compared to control pregnancies (n = 64,218), HS pregnancies (n = 1862) had a higher risk of spontaneous abortion (15.5% vs 11.3%), preterm birth (9.1% vs 6.7%), gestational diabetes mellitus (11.6% vs 8.4%), gestational hypertension (6.1% vs 4.4%), preeclampsia (6.6% vs 3.8%), and cesarean section (32.4% vs 27.1%). Relative risk of some pregnancy and maternal outcomes were attenuated after comorbidity adjustment. In the fully adjusted model, HS pregnancies were independently associated with spontaneous abortion (odds ratio, 1.20; 95% CI, 1.04-1.38), gestational diabetes mellitus (odds ratio, 1.26; 95% CI, 1.07-1.48), and cesarean section (odds ratio, 1.09; 95% CI, 1.004-1.17). LIMITATIONS: We could not evaluate potential influences of disease duration, activity, or severity. Newborn outcomes could not be evaluated. CONCLUSION: HS appears to be an independent risk factor for adverse pregnancy and maternal outcomes. This risk is influenced by comorbidities that may be modifiable with early identification and management.

Mucocutaneous disease and related clinical characteristics in hospitalized children and adolescents with COVID-19 and multisystem inflammatory syndrome in children.

BACKGROUND: Little is known about mucocutaneous disease in acutely ill children and adolescents with COVID-19 and multisystem inflammatory syndrome in children (MIS-C). OBJECTIVE: To characterize mucocutaneous disease and its relation to clinical course among hospitalized patients with COVID-19 and MIS-C. METHODS: Descriptive cohort study of prospectively and consecutively hospitalized eligible patients between May 11, 2020 and June 5, 2020. RESULTS: In COVID-19 patients, 4 of 12 (33%) had rash and/or mucositis, including erythema, morbilliform pattern, and lip mucositis. In MIS-C patients, 9 of 19 (47%) had rash and/or mucositis, including erythema, morbilliform, retiform purpura, targetoid and urticarial patterns, along with acral edema, lip mucositis, tongue papillitis, and conjunctivitis. COVID-19 patients with rash had less frequent respiratory symptoms, pediatric intensive care unit admission, invasive ventilation, and shorter stay versus COVID-19 patients without rash. MIS-C patients with rash had less frequent pediatric intensive care unit admission, shock, ventilation, as well as lower levels of C-reactive protein, ferritin, D-dimer, and troponin (vs MIS-C without rash). Neutrophil-to-lymphocyte ratio was similar for patients with and without rash in both groups. None of the MIS-C patients met criteria for Kawasaki disease. LIMITATIONS: Small sample sizes. CONCLUSIONS: Mucocutaneous disease is common among children and adolescents with COVID-19 and MIS-C. Laboratory trends observed in patients with rash may prognosticate a less severe course.

Eruptions and related clinical course among 296 hospitalized adults with confirmed COVID-19.

BACKGROUND: Limited information exists on mucocutaneous disease and its relation to course of COVID-19. OBJECTIVE: To estimate prevalence of mucocutaneous findings, characterize morphologic patterns, and describe relationship to course in hospitalized adults with COVID-19. METHODS: Prospective cohort study at 2 tertiary hospitals (Northwell Health) between May 11, 2020 and June 15, 2020. RESULTS: Among 296 hospitalized adults with COVID-19, 35 (11.8%) had at least 1 disease-related eruption. Patterns included ulcer (13/35, 37.1%), purpura (9/35, 25.7%), necrosis (5/35, 14.3%), nonspecific erythema (4/35, 11.4%), morbilliform eruption (4/35, 11.4%), pernio-like lesions (4/35, 11.4%), and vesicles (1/35, 2.9%). Patterns also showed anatomic site specificity. A greater proportion of patients with mucocutaneous findings used mechanical ventilation (61% vs 30%), used vasopressors (77% vs 33%), initiated dialysis (31% vs 9%), had thrombosis (17% vs 11%), and had in-hospital mortality (34% vs 12%) compared with those without mucocutaneous findings. Patients with mucocutaneous disease were more likely to use mechanical ventilation (adjusted prevalence ratio, 1.98; 95% confidence interval, 1.37-2.86); P < .001). Differences for other outcomes were attenuated after covariate adjustment and did not reach statistical significance. LIMITATIONS: Skin biopsies were not performed. CONCLUSIONS: Distinct mucocutaneous patterns were identified in hospitalized adults with COVID-19. Mucocutaneous disease may be linked to more severe clinical course.

Overall and subgroup prevalence of pyoderma gangrenosum among patients with hidradenitis suppurativa: A population-based analysis in the United States.

BACKGROUND: Hidradenitis suppurativa (HS) and pyoderma gangrenosum (PG) are reported to coexist, although the prevalence of PG among patients with HS has not been systematically evaluated. OBJECTIVE: To evaluate PG prevalence among patients with HS. METHODS: Cross-sectional analysis of adults with PG among patients with HS and patients without HS through use of electronic health records data from a population-based sample of 55 million patients. RESULTS: The prevalence of PG among 68,232 patients with HS was 0.18% (125 of 68,232), compared with 0.01% (1835 of 31,435,166) among those without HS (P < .0001). Prevalence was markedly higher among patients with HS and Crohn's disease (CD) (3.68%) than among patients with HS but without CD (0.12%). The odds of having PG were 21.14 (95% confidence interval [CI], 17.51-25.51) times greater among patients with HS than among those without HS. Patients with HS with CD had 12.38 (95% CI, 9.15-16.74) times the odds of having PG than did patients without HS but with CD. Among patients without CD, compared with patients without HS, those with HS had 26.51 (95% CI, 21.07-33.36) times the odds of having PG. LIMITATIONS: We could not establish HS phenotype among those having coexistent PG, nor could we distinguish syndromic from nonsyndromic cases. CONCLUSION: Patients with HS have an increased prevalence of PG, regardless of CD status. Painful ulcerations among patients with HS warrant additional evaluation for PG.

Outcome Measurement Instruments Used to Evaluate Dermatologic Adverse Events in Cancer Trials: A Systematic Review.

Importance: Assessment of type, severity, and impact of dermatologic adverse events (DAEs) necessitates well-developed and validated clinician-reported outcome measures (ClinROMs) and patient-reported outcome measures (PROMs) that evaluate concepts specific to mucocutaneous toxic effects and that allow appropriate interpretation and comparison of DAEs across trials. Objective: To evaluate heterogeneity and quality of ClinROMs and PROMs used to assess DAEs from systemic cancer therapy. Evidence Review: Two systematic reviews were conducted by searching PubMed and Embase databases from inception through March 7, 2023, and April 12, 2023. The first search included randomized clinical trials and observational studies reporting systemic cancer treatment-induced DAEs assessed by a ClinROM or PROM. The second included studies evaluating measurement properties of frequently used ClinROM and PROM instruments. The Consensus-Based Standards for the Selection of Health Measurement Instruments risk of bias tool was used to evaluate methodologic quality of validation assessments. Findings: A total of 395 studies were included. The Common Terminology Criteria for Adverse Events (CTCAE) was utilized in 331 studies meeting inclusion criteria (83.8%). At least 1 skin-related PROM was infrequently utilized in systemic chemotherapy clinical trials (79 studies [20.0%]). Most frequently utilized PROMs were the Dermatology Life Quality Index (DLQI; 34 studies [8.6%]) and Skindex-16 (20 studies [5.1%]). Among studies capturing DAEs, 115 (29.1%) reported a nondescript term (ie, rash) as the only DAE. Eight studies described 44 property assessments of the CTCAE, DLQI, and Skindex. There were no studies evaluating content validity, intrarater reliability, or measurement error for the CTCAE, DLQI, or Skindex. There were no studies evaluating structural validity, internal consistency, and responsiveness of DLQI or Skindex. Interrater reliability and responsiveness were each assessed for 1 DAE-related component of the CTCAE. Construct validity for CTCAE, DLQI, and Skindex was evaluated in 29 (65.9%), 3 (6.8%), and 9 (20.5%) assessments, respectively. Conclusions and Relevance: In this systematic review, there was a narrow spectrum of ClinROMs and PROMs with limited validity for the measurement of DAEs in the context of systemic chemotherapy interventions in clinical trials. Report of trial DAEs often had low morphologic specificity and meaning. Based on existing gaps in measurement and report of DAEs, a frequent and impactful adverse event to chemotherapy, the framework for evaluating cutaneous toxic effects in oncology trials may need collaborative reevaluation.

Management of Nail Disease in Patients With Psoriatic Arthritis: An Updated Literature Review Informing the 2021 GRAPPA Treatment Recommendations.

OBJECTIVE: Nail psoriasis is common, impairs fine motor finger functioning, affects cosmesis, and is associated with a lower quality of life. This review updates the previous Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) treatment recommendations for nail psoriasis. METHODS: This systematic literature review of the PubMed, MEDLINE, Embase, and Cochrane databases examined the updated evidence since the last GRAPPA nail psoriasis treatment recommendations published in 2014. Recommendations are based on preformed PICO (Patient/Population - Intervention - Comparison/Comparator - Outcome) questions formulated by an international group of dermatologists, rheumatologists, and patient panel members. Data from this literature review were evaluated in line with Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology. RESULTS: Overall, there is insufficient evidence to make any recommendation for the use of topical corticosteroids, topical calcipotriol, topical tazarotene, topical cyclosporine, dimethyl fumarates/fumaric acid esters, phototherapy, and alitretinoin. There is a low strength of evidence to support the use of calcipotriol and corticosteroid preparations, topical tacrolimus, oral cyclosporine, oral methotrexate, intralesional corticosteroids, pulsed dye laser, acitretin, Janus kinase inhibitors, and apremilast. CONCLUSION: The highest strength of supporting evidence is for the recommendation of biologic agents including tumor necrosis factor inhibitors, and interleukin 12/23, 17, and 23 inhibitors.

Outcome Measures for the Evaluation of Treatment Response in Hidradenitis Suppurativa for Clinical Practice: A HiSTORIC Consensus Statement.

Importance: Although several clinician- and patient-reported outcome measures have been developed for trials in hidradenitis suppurativa (HS), there is currently no consensus on which measures are best suited for use in clinical practice. Identifying validated and feasible measures applicable to the practice setting has the potential to optimize treatment strategies and generate generalizable evidence that may inform treatment guidelines. Objective: To establish consensus on a core set of clinician- and patient-reported outcome measures recommended for use in clinical practice and to establish the appropriate interval within which these measures should be applied. Evidence Review: Clinician- and patient-reported HS measures and studies describing their psychometric properties were identified through literature reviews. Identified measures comprised an item reduction survey and subsequent electronic Delphi (e-Delphi) consensus rounds. In each consensus round, a summary of outcome measure components and scoring methods was provided to participants. Experts were provided with feasibility characteristics of clinician measures to aid selection. Consensus was achieved if at least 67% of respondents agreed with use of a measure in clinical practice. Findings: Among HS experts, response rates for item reduction, e-Delphi round 1, and e-Delphi round 2 surveys were 76.4% (42 of 55), 90.5% (38 of 42), and 92.9% (39 of 42), respectively; among patient research partners (PRPs), response rates were 70.8% (17 of 24), 100% (17 of 17), and 82.4% (14 of 17), respectively. The majority of experts across rounds were practicing dermatologists with 18 to 19 years of clinical experience. In the final e-Delphi round, most PRPs were female (12 [85.7%] vs 2 males [11.8%]) and aged 30 to 49 years. In the final e-Delphi round, HS experts and PRPs agreed with the use of the HS Investigator Global Assessment (28 [71.8%]) and HS Quality of Life score (13 [92.9%]), respectively. The most expert-preferred assessment interval in which to apply these measures was 3 months (27 [69.2%]). Conclusions and Relevance: An international group of HS experts and PRPs achieved consensus on a core set of HS measures suitable for use in clinical practice. Consistent use of these measures may lead to more accurate assessments of HS disease activity and life outcomes, facilitating shared treatment decision-making in the practice setting.

Hospital practices and clinical outcomes associated with behavioral symptoms in persons with dementia.

BACKGROUND: Hospitalized persons living with dementia (PLWD) often experience behavioral symptoms that challenge medical care. OBJECTIVE: This study aimed to identify clinical practices and outcomes associated with behavioral symptoms in hospitalized PLWD. DESIGN: A retrospective cross-sectional study. SETTINGS AND PARTICIPANTS: The study included PLWD (65+) admitted to one of severe health system hospitals in 2019. INTERVENTION: Behavioral symptoms were defined as the presence of (1) a psychoactive medication for behavioral symptoms; (2) an order for physical restraints or constant observation; and/or (3) physician documentation of delirium, encephalopathy, or behavioral symptoms. MAIN OUTCOME AND MEASURES: Associations between behavioral symptoms and patient characteristics and hospital practices (e.g., bladder catheter) were examined. Multivariable logistic/linear regression was used to evaluate the association between behavioral symptoms and clinical outcomes (e.g., mortality). RESULTS: Of hospitalized PLWD (N = 8637), the average age was 84.5 years (IQR = 79-90), 61.7% were female, 60.1% were white, and 9.4% (n = 833) were Hispanic. Behavioral symptoms were identified in 40.6% (N = 3606) of individuals. Behavioral symptoms were significantly associated with male gender (40.3% vs. 36.9%, p = .001), white race (62.7% vs. 58.3%, p < .001), and residence in a facility prior to admission (26.6% vs. 23.7%, p < .001). Regarding hospital practices, indwelling bladder catheters (11.2% vs. 6.0%, p < .001) and dietary restriction (41.9% vs. 33.8%, p < .001) were associated with behavioral symptoms. In multivariable models, behavioral symptoms were associated with increased hospital mortality (odds ratio [OR]: 1.90, CI95%: 1.57-2.29), length of stay (parameter estimate: 2.10, p < .001), 30-day readmissions (OR: 1.14, CI95%: 1.014-1.289), and decreased discharge home (OR: 0.59, CI95%: 0.53-0.65, p < .001). CONCLUSIONS: Given the association between behavioral symptoms and poor clinical outcomes, there is an urgent need to improve the provision of care for hospitalized PLWD.