Italian Journal of Dermatology and Venereology最新文献

Intravenous immunoglobulin (IVIG) therapy has emerged as a promising treatment option for various dermatological autoimmune diseases due to its immunomodulatory potential and low incidence of severe side effects. Despite its widespread use, the mechanism of action of IVIG in treating autoimmune diseases remains a topic of debate. IVIG is derived from the plasma fractionation of a large pool of donors, primarily consisting of the IgG isotype. Its main mechanisms of action involve neutralizing circulating autoantibodies via the F(ab')2 portion, inhibiting complement-mediated tissue destruction, and reducing the half-life of circulating autoantibodies through the Fc portion. This paper explores the growing utilization of IVIG as an off-label therapy in dermatological autoimmune or immune-mediated diseases, including autoimmune bullous disease (AIBS), dermatomyositis (DM), lupus erythematosus (LE), systemic sclerosis, scleromyxedema, Stevens-Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), pyoderma gangrenosum (PG), and necrobiotic xanthogranuloma (NXG). In this context, the sole large prospective, randomized trial was the 2022 ProDERM study, which demonstrate efficacy of IVIG in improving cutaneous manifestations among 95 DM patients compared to the placebo group. Moreover, although considered off-label, the use of IVIG is regarded as the first-line therapy for patients with scleromyxedema. As a first line of therapy, IVIg is only approved for Kawasaki Disease (KD) in the setting of vasculitis. The treatment in all other indications is mostly considered as adjuvant therapy only after failure of immunosuppressive therapy or in the presence of contraindications.

Introduction: Lichen striatus (LS) is an acquired blaschkitis, with typical linear and unilateral distribution. It occurs mainly in children and is self-resolving, yet its etiopathogenesis remains widely unknown.

Evidence acquisition: A review of the literature on LS cases was performed using the keyword "lichen striatus" to retrieve all relevant articles through PubMed and Google Scholar.

Evidence synthesis: A total of 27 articles describing 440 LS patients were included in the present review. Mean age of patients was 3.8 years; male: female: ratio was 1:1.9. The present review confirms LS as a primarily pediatric condition, mainly affecting females. Dysregulation of the immune system might be involved in its pathogenesis, with cytotoxic T-cells attacking mosaic keratinocytes after loss of immune tolerance. The review confirms LS is mainly diagnosed clinically (80%) based on its clinical characteristics: erythematous (70%) or hypopigmented (24%) papules, distributed along the lines of Blashko. The benignity of this clinical entity is suggested by the rather short duration of disease (9.5 months on average) and by the uncommonness of therapy, adopted in only 20% of cases, and when needed, administered topically.

Conclusions: This review examines the complexities of LS but acknowledges limitations in data sources and calls for further research.

Background: Prurigo nodularis (PN) is a chronic, inflammatory skin disease characterized by intense itch. Little evidence exists on the burden of PN in Italy. This real-world analysis aimed to investigate the healthcare resource consumption and related direct costs of patients hospitalized for PN.

Methods: The analysis utilized the administrative databases of healthcare units that cover approximately 12 million inhabitants across Italy. Adult patients were included by ICD-9-CM=698.3 (lichen simplex chronicus; neurodermatitis circumscripta; PN) as proxy of PN diagnosis, from 01/2010 to 09/2021, and had 1 year of data availability before (baseline) and after (follow-up) hospitalization (index date). These patients were 1:2 matched by age, sex, and index date (year) to adults without such hospitalization in the baseline period (matched non-PN controls).

Results: The analysis comprised 295 PN-hospitalized patients, matched with 590 non-PN individuals (mean age 63.2 years, 43.7% female). At baseline, patients had a greater comorbidity burden than non-PN controls, including higher prevalence of hypertension (56.6% vs. 36.6%, respectively), dyslipidemia (26.4% vs. 18.0%), diabetes (24.4% vs. 12.5%) and mental health conditions (14.9% vs. 7.8%). During 1-year follow-up, PN-hospitalized patients had significantly higher resource consumption than matched controls, in terms of mean number of prescriptions most commonly prescribed in PN patients (5.1 vs. 1.9, P<0.001), other drugs (11.7 vs. 6.5, P<0.001), all-cause hospitalization (1.4 vs. 0.1, P<0.001) and outpatient services (5.4 vs. 2.5, P<0.001). Mean annual all-cause healthcare costs for patients over 1-year follow-up were € 3847 total (€ 875 drugs, € 2652 hospitalization, € 320 outpatient services), higher than those for the matched controls, of € 711 total (P<0.001) (€ 353 drugs, € 228 hospitalization, € 130 outpatient services).

Conclusions: Patients hospitalized for PN had a higher comorbidity burden at baseline and greater healthcare resource consumption during 1-year follow-up compared to matched controls, with a 5.4-fold increase in all-cause healthcare costs, indicating substantial clinical burden and remaining unmet need in these patients.

Background: The JADE COMPARE was a multicenter, phase 3 randomized, double-blind, placebo-controlled trial with the objective of comparing the 16-week efficacy and safety of oral abrocitinib 100 or 200 mg once daily, dupilumab 300 mg subcutaneous injection every 2 weeks, or placebo in adults with moderate-to-severe atopic dermatitis (AD). Pharmacoeconomic evaluation of these new drugs for AD is lacking. The objective of our study was to compare the cost per responder of abrocitinib versus dupilumab in patients with AD using the data from the JADE COMPARE trial.

Methods: The cost per responder was calculated by multiplying the cost of treatment by the number needed to treat for each therapy, as obtained from the JADE COMPARE trial. The 12-week primary endpoint was the Investigator global assessment (IGA) 0/1 and eczema area and severity index improvement of at least 75% (EASI75) response rate. The key secondary end points were itch response (defined as an improvement of ≥4 points in the score on the Peak Pruritus Numerical Rating Scale [PP-NRS; scores range from 0 to 10]) at week 2 and IGA and EASI-75 responses at week 16. The cost per responder model was based on the perspective of the Italian National Health System. Regarding the costs of drugs, ex-factory wholesale purchase prices were used, including the mandatory discounts according to the national legislation (5% discount, plus a further 5% reduction on the discount result). Abrocitinib 100 mg and 200 mg have flat price in Italy.

Results: The 12-week IGA 0/1 cost per responder was € 3955.77 and € 2984.94 for abrocitinib 100 mg and 200 mg, respectively, versus € 7467.96 for dupilumab; as for 12-week EASI75 the cost were € 2463.30 and € 2057.58 vs. € 4705.09, respectively. As far as the secondary end points, the costs per responder were always lower for abrocitinib 100 and 200 mg compared to dupilumab, including the PP-NRS (€ 3791.55 and € 2451.22, vs. € 6462.65), the IGA 0/1 at week 16 (€ 6931.05 and € 4854.15 vs. € 8787.85) and the EASI75 at week 16 (€ 3984.75 and € 3381.00 vs. € 5197.45).

Conclusions: According to JADE COMPARE trial data, the costs per responder of abrocitinib were considerably lower than dupilumab. The results of the study are limited to the short time frame of JADE COMPARE trial.