Development of a Skin-Directed Scoring System for Stevens-Johnson Syndrome and Epidermal Necrolysis: A Delphi Consensus Exercise.

Importance: Scoring systems for Stevens-Johnson syndrome and epidermal necrolysis (EN) only estimate patient prognosis and are weighted toward comorbidities and systemic features; morphologic terminology for EN lesions is inconsistent. Objectives: To establish consensus among expert dermatologists on EN terminology, morphologic progression, and most-affected sites, and to build a framework for developing a skin-directed scoring system for EN. Evidence Review: A Delphi consensus using the RAND/UCLA appropriateness criteria was initiated with a core group from the Society of Dermatology Hospitalists to establish agreement on the optimal design for an EN cutaneous scoring instrument, terminology, morphologic traits, and sites of involvement. Findings: In round 1, the 54 participating dermatology hospitalists reached consensus on all 49 statements (30 appropriate, 3 inappropriate, 16 uncertain). In round 2, they agreed on another 15 statements (8 appropriate, 7 uncertain). There was consistent agreement on the need for a skin-specific instrument; on the most-often affected skin sites (head and neck, chest, upper back, ocular mucosa, oral mucosa); and that blanching erythema, dusky erythema, targetoid erythema, vesicles/bullae, desquamation, and erosions comprise the morphologic traits of EN and can be consistently differentiated. Conclusions and Relevance: This consensus exercise confirmed the need for an EN skin-directed scoring system, nomenclature, and differentiation of specific morphologic traits, and identified the sites most affected. It also established a baseline consensus for a standardized EN instrument with consistent terminology.

Extragenital lichen sclerosus: a comprehensive review of clinical features and treatment.

Lichen sclerosus (LS) is a chronic inflammatory skin disease commonly affecting the anogenital area with less frequent extragenital occurrence. Extragenital LS cutaneous manifestations vary and precipitating factors are not well described. Recent evidence for etiology and clinical associations of extragenital LS provide insight into disease recognition and pathogenesis. Novel diagnostic techniques as well as treatment standardization have the potential to improve management of this rare condition. This review details both past and new insights into the pathogenesis, clinical manifestations, and treatment options of extragenital LS.

Risk of psoriasis according to body mass index: A retrospective cohort analysis.

BACKGROUND: Psoriasis has been linked to obesity, although data on the incidence of psoriasis according to body mass index (BMI) are limited. OBJECTIVE: To compare incidence of psoriasis among patients stratified by BMI category (normal or underweight, overweight, obese class 1, obese class 2/3). METHODS: Retrospective cohort analysis of a demographically heterogeneous sample of over 1.5 million patients in the United States between January 1, 2008 and September 9, 2019. RESULTS: Crude incidence of psoriasis per 10,000 person-years was 9.5 (95% confidence interval [CI], 9.1-10.0) among normal or underweight patients, 11.9 (95% CI, 11.4-12.4) among overweight patients, 14.2 (95% CI, 13.6-14.9) among obese class 1 patients, and 17.4 (95% CI, 16.6-18.2) among obese class 2/3 patients. Compared to patients with BMI < 25.0, those who were overweight (adjusted hazard ratio, 1.19; 95% CI, 1.12-1.27; P < .001), obese class 1 (adjusted hazard ratio, 1.43; CI, 1.34-1.53; P < .001) and obese class 2/3 (adjusted hazard ratio, 1.83; CI, 1.71-1.95; P < .001) significantly greater risks for developing psoriasis. LIMITATIONS: Influence of obesity on psoriasis severity could not be measured. CONCLUSION: BMI independently influences the development of psoriasis. There appears to be a graded association between BMI and risk of psoriasis.

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.

Incidence of pneumocystosis among patients exposed to immunosuppression.

BACKGROUND: The decision to administer prophylaxis to patients receiving immunosuppression against pneumocystosis remains a dilemma. OBJECTIVE: To determine overall and age-specific 5-year pneumocystosis incidence within a population exposed to immunosuppressants. METHODS: Retrospective cohort analysis identifying incident pneumocystosis cases among adults without HIV, AIDS, or cancer exposed to immunosuppressant, corticosteroid therapy, or both. RESULTS: We identified 406 new cases among patients prescribed an immunosuppressant, corticosteroid, or both. Overall incidence of pneumocystosis was 0.012% (406/3,366,086). Incidence was highest in those exposed to immunosuppressant and corticosteroid medications (0.199%), followed by those exposed to immunosuppressant alone (0.012%), corticosteroid alone (0.008%), and neither medication (0.001%) (P < .001). The greatest risk differences were noted between groups exposed to immunosuppressant and corticosteroid compared with neither (0.198%, 95% confidence interval [CI] 0.166%-0.230%) or immunosuppressant alone (0.188%, 95% CI 0.155%-0.221%). The greatest relative risks (RRs) were noted among those receiving immunosuppressant and corticosteroid compared with those exposed to neither (RR 122.5, 95% CI 100.9-148.8) or immunosuppressant alone (RR 16.5, 95% CI 7.3-37.4). LIMITATIONS: We could not confirm dose and duration of exposures. CONCLUSION: Incidence of pneumocystosis among patients exposed to immunosuppressants is very low. Prophylaxis for patients receiving combination immunosuppressant and corticosteroid therapy, the group at highest risk, might be warranted.

Investing in the future of inpatient dermatology: the evolution and impact of specialized dermatologic consultation in hospitalized patients.

Inpatient dermatology has transitioned from units that admitted and cared for patients with chronic dermatoses to consultative services that provide a wide breadth of care, leading to a paradigm shift in the role and impact of dermatologists in the inpatient setting. Consultative dermatology provides a distinct and essential service in the care of hospitalized patients, leading to improved care quality along with reductions in inappropriate health care spending.

The complexity of diagnosing latent tuberculosis infection in older adults in long-term care facilities.

OBJECTIVES: In the USA, tuberculosis disease rates are highest in older adults. Diagnostic testing for latent tuberculosis infection (LTBI) has not been evaluated carefully in this group. The aim of this study was to define the relationship between tuberculin skin test (TST) results, T-SPOT.TB results, and T-cell responses to Mycobacterium tuberculosis antigens. METHODS: Long-term care facility residents with known prior TST results (positive or negative) were retested with TSTs and T-SPOT.TB. Prior exposure to M. tuberculosis was assessed by quantifying T-cell activation to mycobacterial antigens in vitro. RESULTS: The median age of the 37 participants was 77 years (range 57-98 years). Among 18 participants with a prior positive TST, three (16.7%) had a negative TST when retested (TST reversion); two had a negative T-SPOT.TB. Of the 15 who were historically and currently TST-positive, four (26.7%) had a negative T-SPOT.TB and one (6.7%) had a borderline result. Percentages of CD4+ T-cells responding to mycobacterial antigens were higher in participants with positive TST and T-SPOT.TB (18.2%) compared to those with a positive TST but negative T-SPOT.TB (6.4%, p=0.16) and negative TST and T-SPOT.TB (5.9%, p<0.001). CONCLUSIONS: LTBI testing in older adults is complicated by TST reversion and TST-positive/T-SPOT.TB-negative discordance, which may reflect clearance of infection or waning immunity.

B cell responses in older adults with latent tuberculosis: Considerations for vaccine development.

Reactivation of latent tuberculosis (LTBI) is more common among the aging population and may contribute to increased transmission in long-term health care facilities. Difficulties in detecting LTBI due to potential blunting of the tuberculin skin test (TST), and the lowered ability of the elderly to tolerate the course of antibiotics, underscore the need for an effective vaccine. Immuno-senescence reduces the capacity of vaccines to induce sufficient levels of protective immunity against many pathogens, further increasing the susceptibility of the elderly to infectious diseases. We sought to evaluate the response of B cells to Mycobacterium tuberculosis (Mtb) in residents of long-term care facilities to determine the feasibility of using a vaccine to control infection and transmission from reactivated LTBI. Our results demonstrate that although B cell responses were higher in subjects with LTBI, Mtb antigens could stimulate B cell activation and differentiation in vitro in TST negative subjects. B cells from elderly subjects expressed high basal levels of Toll-like receptor (TLR)2 and TLR4 and responded strongly to Mtb ligands with some activation pathways dependent on TLR2. B cells derived from blood, tonsil and spleen from younger subjects responded similarly and to the same magnitude. These results suggest that B cell responses are robust in the elderly and modifications to a TB vaccine, such as TLR2 ligand-based adjuvants, may help increase immune responses to a protective level.