Acta dermato-venereologica最新文献

Basal cell nevus syndrome is caused by mutations in the Sonic hedgehog pathway and characterized by early-onset basal cell carcinoma. The features of basal cell carcinoma in basal cell nevus syndrome compared with sporadic basal cell carcinoma have not been explored. This study is a retrospective study of patients with basal cell nevus syndrome in two medical centres in Taiwan from 1991 to 2021 and patients with sporadic basal cell carcinoma excised from 2015 to 2020. An analysis of 18 patients with basal cell nevus syndrome showed an older mean age at the first diagnosis of basal cell carcinoma (37.5 years) than reported in Western countries. The majority of basal cell carcinomas were located in the head and neck region (80.7%), with nodular BCC being the most common tumour type (47.0%). Compared with sporadic basal cell carcinomas, basal cell carcinomas in basal cell nevus syndrome patients occurred more frequently on the scalp (34.7% vs 6.1%, p < 0.001). In addition, the superficial type of basal cell carcinoma was more likely to be seen in basal cell nevus syndrome (24.7% vs 10.4%, p < 0.001). The limitations were that some features of the basal cell nevus syndrome patients might not have been present yet at the time of examination or they did not receive thorough screening. In conclusion, the distinct features of basal cell carcinomas in basal cell nevus syndrome patients have important implications for the prevention, diagnosis, and management of basal cell carcinoma in basal cell nevus syndrome patients.

Cutaneous T-cell lymphomas are a heterogeneous group of non-Hodgkin lymphomas. Early stages are often controlled with skin-directed therapy, such as topical corticosteroids, topical chlormethine gel, or UV therapy, whereas advanced stages often warrant a more aggressive approach with systemic antibody targeted therapy including mogamulizumab, brentuximab vedotin, or alemtuzumab. A retrospective cohort case series of 27 patients from Aarhus University Hospital, Denmark is presented, evaluating real-world outcomes of patients with cutaneous T-cell lymphomas treated with intravenous systemic targeted therapies from 2013 to 2023. The median age was 72 and the majority had Sézary syndrome or mycosis fungoides. All patients had relapsed/refractory advanced stage cutaneous T-cell lymphoma. Six patients received mogamulizumab, 12 patients received brentuximab vedotin, and 15 patients received alemtuzumab. Six patients received more than 1 of the systemic targeted treatments. Overall response rates were 78% for mogamulizumab, 65% for brentuximab vedotin, and 61% for alemtuzumab. Median time to progression was 2.5, 4, and 11 months, respectively. In conclusion, this paper offers a unique perspective on the complexities of clinical practice when managing advanced-stage cutaneous T-cell lymphomas and demonstrates the effectiveness of the therapies described, with particular emphasis on the promising results observed with alemtuzumab administered in a low-dose protocol.

Chronic pruritus (≥ 6 weeks) is a frequent symptom in atopic diseases, with phenotypes ranging from non-lesional skin to inflammatory diseases like atopic dermatitis. Data on patients' needs and treatment goals depending on the skin phenotype and disease burden are limited. This study aimed to analyse the impact of distinct phenotypes of chronic atopic pruritus on disease burden and treatment goals. Another objective was to investigate whether the disease burden influences the treatment goals. Patient-reported outcomes of 1,086 adult patients (n = 529 with atopic dermatitis, n = 557 with chronic pruritus on non-lesional skin with atopic skin diathesis) were analysed age- and gender-matched (mean age 49.7 ± 19.0 years; n = 605 female [55.7%]), comparing pruritus intensity (Numeric Rating Scale), quality of life (Dermatology Life Quality Index, ItchyQol), anxiety and depression (Hospital Anxiety and Depression Scale), and patient needs (Patient Needs Questionnaire of the Patient Benefit Index-Pruritus). Although the disease burden was significantly higher in patients with atopic dermatitis (prolonged disease duration, increased quality of life impairment, higher pruritus intensity), the treat-ment goals of both phenotypes matched in 92.6%. The most important needs were to no longer experience itching, find a clear diagnosis and therapy, and have confidence in the therapy.