Search

Earlier monitoring is unnecessary for those individuals on combined therapies (i.e. hydroxychloroquine and mepacrine) as the recommendations relate only to the use of hydroxychloroquine and chloroquine. Dear Editor, The Royal College of Ophthalmologists (RCOphth) has published new recommendations for retinal monitoring for hydroxychloroquine and chloroquine users in the UK (2020).1 The recommendations aim to protect both patients and prescribers by reducing the risk of irreversible visual loss from hydroxychloroquine retinopathy. [Extracted from the article]

BACKGROUND Dupilumab, a monoclonal antibody against interleukin (IL)-4 receptor alpha that inhibits IL-4/IL-13 signalling is indicated in dermatology for the treatment of moderate-to-severe atopic dermatitis (AD) in adult and adolescent patients 12 years and older and severe AD in children 6-11 years, who are candidates for systemic therapy. Dupilumab received Early Access to Medicines Scheme (EAMS) approval for adults in March 2017. OBJECTIVES The purpose of this study was to assess the efficacy outcomes of treatment with dupilumab in EAMS. METHODS A retrospective analysis of adult patients enrolled in the dupilumab EAMS in the UK. Scores were assessed at baseline and follow up, including the Eczema Area and Severity Index (EASI), Investigator's Global Assessment Score (IGA) and Dermatology Life Quality Index (DLQI). RESULTS Data were available for 57 adult patients treated with dupilumab for at least 12 weeks; 73.6% of patients had received prior treatment with 3 or 4 immunosuppressants. Baseline scores for the EASI and DLQI were 27.93 (standard deviation, SD 13.09) and 18.26 (SD 6.18) respectively. AD severity scores showed statistically significant improvement at week 16+4 weeks (p <0.001 for all). The mean change in EASI was 14.13 points with 66.7% and 36.7% achieving a 50% (EASI-50) and 75% (EASI-75) improvement in EASI, respectively at 16+/- 4 weeks. IGA scores improved by at least two categories for 75% patients. DLQI scores decreased by a mean of 9.0 points, with 80% patients demonstrating a MCID 4-point improvement. For 85% patients, clinicians rated the treatment response as being either 'better' (19%) or 'much better' (65%). CONCLUSIONS Dupilumab is associated with a significant and clinically relevant improvements in AD as measured by patient- and physician-reported outcome measures. Importantly, the clinical efficacy, despite the refractory disease of this EAMS cohort, is comparable to that previously reported in clinical trials. [ABSTRACT FROM AUTHOR]

Summary: Toxic epidermal necrolysis (TEN) and Stevens–Johnson syndrome (SJS) are characterized by widespread skin and mucosal blistering and necrosis. The triggers and long‐term sequelae in children may differ from those reported for adults. Bronchiolitis obliterans (BO) is an uncommon complication, with only 15 previously reported cases, but can lead to significant long‐term morbidity, requiring lung transplantation in some cases. We report three children with nondrug‐related SJS (n = 1) and TEN (n = 2) who developed BO. Two were treated with intravenous immunoglobulin therapy (2–2.4 g/kg) and all three survived. We highlight salient learning points from our cases and potential pitfalls in diagnosis of BO, including delayed onset, and we also review the literature. [ABSTRACT FROM AUTHOR]

Dear Editor, Frontal fibrosing alopecia (FFA) is an inflammatory primary scarring alopecia of uncertain aetiology that represents a variant of lichen planopilaris.1 It predominantly, although not exclusively, affects postmenopausal women.2 Its pathogenesis is characterized by immune-mediated follicular destruction at the level of the hair bulge, which leads to a clinical phenotype of progressive frontotemporal hair and eyebrow loss that is often preceded by widespread body-hair loss.2 Histologically, a lichenoid inflammatory infiltrate surrounds the isthmus and infundibulum of the hair follicle, and this progresses to follicular scarring and dropout in advanced disease.2 We recently completed the first genome-wide association study (GWAS) in FFA coupled with transcriptomic and metabolomic analyses, which have provided important insights into its pathogenesis.3 We have conducted and herein present a descriptive cross-sectional study of the clinical phenotype in women from the FFA UK GWAS Cohort. Patients with a formal diagnosis of FFA made by a consultant dermatologist from 20 secondary care dermatology departments across the UK were eligible for inclusion. In keeping with the immune-mediated pathogenesis of FFA, 20-7% of participants reported at least one comorbid autoimmune disease (Table 1). [Extracted from the article]

Click here for the corresponding questions to this CME article. [ABSTRACT FROM AUTHOR]

Click here for the corresponding questions to this CME article. [ABSTRACT FROM AUTHOR]