Prurigo-like atopic dermatitis in a child with CARD11-associated severe combined immunodeficiency successfully treated with dupilumab.

A 12-year-old boy affected by severe combined immunodeficiency due to a heterozygous variant in the CARD domain of CARD11, c.169G>A; p.Glu57Lys, developed severe atopic dermatitis and alopecia areata. After failure of conventional systemic therapy, dupilumab was administered at a dose of 400 mg subcutaneously, followed by 200 mg every 14 days. The patient had an excellent clinical response after 1 month and complete remission after a year, with the absence of side effects, demonstrating good efficacy and safety profile.

Efficacy and Safety of Dupilumab in the Treatment of Hand Eczema: A Retrospective Study.

Background: Hand eczema (HE) is a prevalent chronic condition that exerts a substantial and enduring adverse effect on quality of life (QoL) and imposes an economic burden on society. Managing HE poses challenges due to the limited effectiveness and potential adverse effects associated with many currently available topical and systemic treatments. Methods: This article examines twenty-one patients affected by HE treated with dupilumab, a fully human monoclonal antibody targeting interleukin IL-4 and IL-13 signaling. This involves a retrospective descriptive statistical analysis. Results: At week 6, HECSI-75 was achieved by 12 patients (57.9%). The proportion of patients meeting the HECSI-75 criteria steadily increased over the observation weeks, reaching 90% at week 16 and 100% at week 104. Furthermore, HECSI-90 and HECSI-100 were achieved by 75% and 60% of patients at week 16 and by 100% and 85% of patients at week 68, respectively. All patients who reached week 104 maintained complete disease remission according to HECSI 100. Conclusions: In all patients, dupilumab was shown to be an effective drug in achieving disease clearance, as indicated by all the parameters considered at each evaluation point (Week 6, Week 16, Week 32, Week 52, Week 68, Week 84, and Week 104), in comparison to the initial baseline.

Overview of germline and somatic genetic alterations in basal and squamous cell carcinoma: a review of the current literature.

Non-melanoma skin cancers (NMSCs), including basal and squamous cell carcinomas, represent the most common malignancies among Caucasians. Over two million cases of NMSC occur each year in the US, with a progressive increase in incidence. There are well-known environmental risk factors, such as iatrogenic or ionizing radiation exposure, but the effect of chronic ultraviolet radiation, especially ultraviolet B radiation, is one of the main predisposing factors. Currently, based on our knowledge of the pathophysiology of most NMSCs, we have a better understanding of the associated genetic risk factors. The aim of this study was to evaluate the factors that contribute to the onset of NMSC through a comprehensive literature research.

Use of Guselkumab for the Treatment of Moderate-to-Severe Plaque Psoriasis: A 1 Year Real-Life Study.

Little information is available from real-life studies evaluating the efficacy of guselkumab in moderate-to-severe psoriasis. In this real-life study, we retrospectively examined a database of 52 patients with moderate-to-severe psoriasis treated with guselkumab (100 mg, s.c.) and followed for 1 year. Disease severity and treatment response was assessed by the Psoriasis Area and Severity Index (PASI) at baseline and after 4, 12, 20, 28, 36, 44, and 52 weeks. Predictors of a PASI response were evaluated by univariate and multivariate regression. After 12 months, 84.2% of patients (mean age 51.3 ± 14.1 years) treated with guselkumab achieved a PASI score of <3. Furthermore, PASI score decreased from 20 ± 13.3 at baseline to 4.4 ± 4.7 and 2.7 ± 3.9 at 12 and 20 weeks, and PASI 75, 90, and 100 response was achieved in 84.2%, 78.9%, and 63.2% of patients respectively at 12 months. Stepwise multivariate regression analysis revealed that previous biological treatment and the presence of comorbidities were associated with poorer response between 28-44 weeks, however the presence of obesity per se was not associated with poorer response. Difficult-to-treat areas were also improved as early as 12 weeks following guselkumab. Guselkumab was observed to be effective and safe in patients with moderate-severe chronic psoriasis in a real world-setting.