Cathal O'Connor

Background Morphea, or localized scleroderma, is an inflammatory, fibrosing skin disorder that can be progressive and debilitating. Infrared thermography frequently has false positive results. The aim of this study was to assess the ability of multispectral imaging to predict disease progression in children with morphea. Methods Children with morphea were recruited between 2016 and 2022. Multispectral images of affected and matched contralateral unaffected sites were obtained using the Antera™ 3D camera. Clinical assessment was performed using the Localized Scleroderma Assessment Tool (LoSCAT). Children were followed up every 3 months for imaging and clinical review. The main outcome measurement was correlation of hemoglobin gradient between affected and matched contralateral unaffected tissue and progression. Results Of 17 children, the average age was 12 years (range 6–18 years); most were female (76.5%) and white (94.1%). Nearly two‐thirds (64.7%) had linear morphea, 35.2% had plaque morphea; 58.8% had been treated with systemic agents. The average LoSCAT score was 20.6 (range 5–73). The average hemoglobin gradient between affected and matched contralateral unaffected skin was four times higher in those who had progression (average differential 0.3, range 0.1–0.4) compared to those who did not (average differential 0.08, range 0.02–0.15). Using a cut off of a 0.18 hemoglobin gradient between affected and unaffected skin, the sensitivity of multispectral imaging for detecting progression in pediatric morphea is 90% with specificity of 100%. Conclusions Multispectral imaging is a novel assessment tool with promising accuracy in predicting progression as an adjunct to clinical assessment in pediatric morphea. Further research should examine its performance against thermography.

Background Rituximab (RTX) has been utilised off‐label for a variety of dermatological indications beyond pemphigus vulgaris. Efficacy has been reported in other immunobullous disorders, inflammatory dermatoses and connective tissue diseases. Objectives To assess the off‐label use of RTX in our centre with respect to indications, frequency and duration of treatment, efficacy, and adverse events. Methods Charts were retrospectively reviewed for all patients who received dermatologist‐prescribed RTX infusions off‐label at our centre between 2009 and 2022. Efficacy was categorised based on reported percentage reduction of disease activity: very good (75%–100%), good (50%–74%), partial (25%–49%) and none (0%–24%). Results Twenty‐nine patients received RTX off‐label during this time period. Infusions were discontinued in 28% ( n = 8), due to insufficient clinical response. Median treatment duration for those on 6‐12‐monthly regular infusions was 2.4 years (range 0.5–11 years). Indications included cutaneous lupus ( n = 9), mucous membrane pemphigoid ( n = 5), pyoderma gangrenosum ( n = 6), lichen planus ( n = 2), dermatomyositis ( n = 1), livedoid vasculitis ( n = 1), sarcoidosis ( n = 1), bullous pemphigoid ( n = 1), pemphigus vulgaris, foliaceus and vegetans ( n = 1 each). Clinical improvement was documented in 79% ( n = 23); very good in 48% ( n = 14), good in 17% ( n = 5), and moderate in 14% ( n = 4). Clinical efficacy in immunobullous disorders was 100% (9/9), 67% in cutaneous lupus (6/9), 33% in pyoderma gangrenosum (2/6), and 50% in lichen planus (1/2). No side effects were documented for 79% ( n = 23). Adverse peri‐infusion events were seen in three patients (10%). Four patients died during follow up; one due to neutropenic sepsis with a background of advanced malignancy, and three due to Covid‐19. Conclusions RTX was prescribed for multiple off‐label dermatological indications, often for recalcitrant disease. Responses were good overall, with a reassuring safety profile. Three patients died of Covid‐19; knowledge of the impact of RTX on the immune response and efficacy of vaccines is expanding and will continue to inform guidelines for RTX use in the post‐Covid era.

Background Targeted biologic therapies have revolutionised the treatment of severe atopic dermatitis (AD). Objectives To assess effects of dupilumab on patient re‐engagement, quality of life (QOL), and burden of treatment (BOT) in severe AD. Methods Adults on dupilumab for AD completed questionnaires on QOL, BOT, and provided qualitative reflections, with a subset interviewed to explore experience of leaving and re‐engaging with dermatology. Prior treatments, adverse events, and clinical severity scoring were evaluated. Statements and interviews were qualitatively reviewed. Results Of 41 patients; median age was 34 years, 68% were male; and 93% ( n = 38) had trialled ≥1 immunomodulatory therapies before dupilumab. Median dermatology life quality index was 21 (range 9–30, SD ± 5.1) pre‐dupilumab, and 2 (range 0–11, SD ± 3.4) post‐dupilumab. Median eczema area and severity index was 31.4 (range 10–46.4, SD ± 11.8) pre‐dupilumab, and 6.4 (range 0.4–13.2, SD ± 3.6) on dupilumab. Burden of treatment scores on dupilumab were low (median 0–3/10) across all domains. Themes identified pre‐dupilumab included sleep disturbance, low self‐esteem, social isolation, disempowerment, frustration with ineffective treatments, and high financial costs. Benefits included confidence reacquisition, enhanced sleep, liberation from time‐consuming ‘messy’ topical regimes, improved relationships, and reclaimed autonomy. Side effects included red/itchy eyes (37%, n = 13) and facial dermatitis (20%, n = 7). Twelve patients had deeper interviews. Regarding disengagement with dermatology, themes included ineffectiveness and toxicity of older treatments, attendance futility, dermatologist fatigue, and ‘fizzling out’. Regarding re‐engagement with dermatology, themes included social media influence, novelty, exasperation with QOL, and life‐changing improvements seen with dupilumab. Conclusions The emergence of novel effective treatments for AD has significant implications for dermatology workforce and financial planning.