Proposal for a 6-step approach for differential diagnosis of neonatal erythroderma

The broad differential diagnosis of neonatal erythroderma often poses a diagnostic challenge. Mortality of neonatal erythroderma is high due to complications of the erythroderma itself and the occasionally severe and life-threatening underlying disease. Early correct recognition of the underlying cause leads to better treatment and prognosis. Currently, neonatal erythroderma is approached on a case-by-case basis. The purpose of this scoping review was to develop a diagnostic approach in neonatal erythroderma. After a systematic literature search in Embase (January 1990 - May 2020, 74 cases of neonatal erythroderma were identified, and 50+ diagnoses could be extracted. Main causes were the ichthyoses (40%) and primary immunodeficiencies (35%). Congenital erythroderma was present in 64% (47/74) of the cases, predominantly with congenital ichthyosis (11/11; 100%), Netherton syndrome (12/14, 86%) and Omenn syndrome (11/23, 48%). Time until diagnosis ranged from 102 days to 116 days for cases of non-congenital erythroderma and congenital erythroderma respectively. Among the 74 identified cases a total of 17 patients (23%) died within a mean of 158 days and were related to Omenn syndrome (35%), graft-versus-host disease (67%) and Netherton syndrome (18%). Disease history and physical examination are summarized in this paper. Age of onset and a collodion membrane can help to narrow the differential diagnoses. Investigations of blood, histology, hair analysis, genetic analysis and clinical imaging are summarized and discussed. A standard blood investigation is proposed, and the need for skin biopsies with lympho-epithelial Kazal-type related Inhibitor staining is highlighted. Overall, this review shows that diagnostic procedures narrow the differential diagnosis in neonatal erythroderma. A 6-step flowchart for the diagnostic approach for neonatal erythroderma during the first month of life is proposed. The approach was made with the support of expert leaders from international multidisciplinary collaborations in the European Reference Network Skin-subthematic group Ichthyosis.Peer reviewe

Dupilumab Drug Survival and Associated Predictors in Patients With Moderate to Severe Atopic Dermatitis Long-term Results From the Daily Practice BioDay Registry

IMPORTANCE Long-term data on dupilumab drug survival in patients with atopic dermatitis (AD) are scarce. Furthermore, little is known about the factors associated with drug survival of dupilumab in AD.OBJECTIVE To describe the drug survival of dupilumab in patients with AD and to identify associated predictors.DESIGN, SETTING, AND PARTICIPANTS This cohort studywas based on data from the multicenter prospective daily practice BioDay registry, in which 4 university and 10 nonuniversity hospitals in the Netherlands participated. Analysis included patients (age &gt;= 18 years) participating in the BioDay registry with a follow-up of at least 4 weeks. The first patient treated with dupilumab was recorded in the BioDay registry in October 2017; data lock took place in December 2020, and data analysis was performed from October 2017 to December 2020.MAIN OUTCOMES AND MEASURES Drug survivalwas analyzed by Kaplan-Meier survival curves and associated characteristics by using univariate and multivariate Cox regression analysis.RESULTS A total of 715 adult patients with AD (mean [SD] age, 41.8 [16.0] years; 418 [58.5%] were male) were included with a 1-year, 2-year, and 3-year overall dupilumab drug survival of 90.3%, 85.9%, and 78.6%, respectively. Characteristics associated with shorter drug survival owing to ineffectiveness were the use of immunosuppressant drugs at baseline (hazard ratio [HR], 2.64; 95% CI, 1.10-6.37) and being a nonresponder at 4 weeks (HR, 8.68; 95% CI, 2.97-25.35). Characteristics associated with shorter drug survival owing to adverse effects were the use of immunosuppressant drugs at baseline (HR, 2.69; 95% CI, 1.32-5.48), age 65 years or older (HR, 2.94; 95% CI, 1.10-7.87), and Investigator Global Assessment score of very severe AD (HR, 3.51; 95% CI, 1.20-10.28).CONCLUSIONS AND RELEVANCE This cohort study demonstrated a good overall 1-year, 2-year, and 3-year dupilumab drug survival. Patients using immunosuppressive therapy at baseline and those with an absence of treatment effect at week 4 tended to discontinue treatment owing to ineffectiveness more frequently. Using immunosuppressant drugs at baseline, older age, and Investigator Global Assessment score of very severe AD were characteristics associated with an increased risk for discontinuation owing to adverse effects. These data provide more insight and new perspectives regarding dupilumab treatment in AD and can contribute to the optimization of patient outcomes.</p

Stroke severity, its correlates and impact on thrombolysis in a population-based study

OBJECTIVE: Data about the distribution of stroke severity and its correlates are sparse. In a population-based approach, we determined the NIH Stroke Scale Score (NIHSSS) and studied associations with demographic variables, stroke unit care, etiology, the onset assessment interval (OAI), and the rate of thrombolysis. METHODS: We performed a databank-based post-hoc analysis of data ascertained during the prospective, population-based stroke study among the 188,015 permanent residents of Basel City, Switzerland. RESULTS: In 246/269 (91.4%) patients, NIHSSS was available. The median NIHSSS was 5.0 +/- 6.0. NIHSSS 0-6, 7-15, and 0.001), shorter OAI (p = 0.009), and thrombolytic therapy (p > 0.001). In multivariate regression analyses, age, OAI, and thrombolysis correlated independently with higher NIHSSS. Stroke unit patients differed from non-stroke unit patients in shorter OAI, younger age, and higher NIHSSS. CONCLUSION: In a geographically defined stroke population, 1/3 patients had moderate-to-severe stroke. Patients with less severe strokes were younger, sought medical attention later and were less likely to receive thrombolysis. Thus, public stroke awareness programs might consider targeting also younger individuals and stress that also mild-to-moderate strokes benefit from emergency medical care

Analysis of the relationship between total cholesterol, age, body mass index among males and females in the WHO MONICA Project

OBJECTIVE: To explore the relationship between hypercholesterolaemia, age and BMI among females and males. DESIGN: Population-based cross-sectional survey. SUBJECTS: The data came from the initial surveys of the WHO MONICA Project. In all, 27 populations with 48 283 subjects (24 017 males and 24 266 females) aged 25-64 y were used for the analysis. MEASUREMENTS: Total cholesterol, weight, height, BMI, prevalence of hypercholesterolaemia (PHC) defined as cholesterol >/=6.5 mmol/l, and the prevalence of obesity (POB) defined as BMI >/=30 kg/m(2). RESULTS: PHC increased with age, with PHC in males being significantly higher than in females at age range 25-49 y and significantly lower than in females at age range 50-64 y. Age-related increase in hypercholesterolaemia was steeper in females than in males. There was a statistically significant positive association between hypercholesterolaemia and BMI. Multiple logistic regression analysis revealed a negative statistically significant (P<0.001) effect modification involving age and BMI on the risk of having hypercholesterolaemia both in females and males. The relation between PHC and BMI became weaker in higher age groups, with no statistically significant association in females aged 50-64 y. CONCLUSION: Public health measures should be directed at the prevention of obesity in young adults since the strongest effect of obesity on the risk of hypercholesterolaemia has been found in subjects aged 25-39 y

Cutaneous lesions in COVID-19 patients

Aside from the typical respiratory symptoms resulting from an infection with SARS-CoV-2, there are reports of cutaneous lesions in patients diagnosed with a SARS-CoV-2 infection. There are reports of multiple groups of skin lesions presenting in different stages of this diagnosis. The most common reported groups are chilblains, vesicular eruptions, morbilliformexanthems, acute urticaria and livedo. It is unlikely that all these groups of skin lesions are distinctive of an infection with SARS-CoV-2. Chilblains of new onset, however, could possibly be a distinctive symptom of a mild/asymptomatic infection with SARS-CoV-2. It is recommended to consider an infection with SARS-CoV-2 in the differential diagnosis in patients presenting with these groups of skin lesions. Consider testing for SARS-CoV-2 and consult the dermatologist if needed, especially in case of chilblains, to ensure histopathological evaluation of the skin lesions to increase knowledge of the underlying pathophysiology

Cutaneous lesions in COVID-19 patients

Aside from the typical respiratory symptoms resulting from an infection with SARS-CoV-2, there are reports of cutaneous lesions in patients diagnosed with a SARS-CoV-2 infection. There are reports of multiple groups of skin lesions presenting in different stages of this diagnosis. The most common reported groups are chilblains, vesicular eruptions, morbilliformexanthems, acute urticaria and livedo. It is unlikely that all these groups of skin lesions are distinctive of an infection with SARS-CoV-2. Chilblains of new onset, however, could possibly be a distinctive symptom of a mild/asymptomatic infection with SARS-CoV-2. It is recommended to consider an infection with SARS-CoV-2 in the differential diagnosis in patients presenting with these groups of skin lesions. Consider testing for SARS-CoV-2 and consult the dermatologist if needed, especially in case of chilblains, to ensure histopathological evaluation of the skin lesions to increase knowledge of the underlying pathophysiology