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Autoimmune skin disorders, including Psoriasis, Lichen Planus, Vitiligo, Atopic Dermatitis, and Alopecia Areata, arise from a combination of genetic predisposition, external factors, and immunological dysfunction. It is well-documented that there is a strong correlation between autoimmune thyroid diseases and a range of dermatological disorders, especially urticaria. This review investigates possible links between autoimmune thyroiditis and a broader spectrum of autoimmune skin conditions, analyzing shared genetic markers, immunological mechanisms, and clinical correlations. Common pathogenic mechanisms include disrupted immune tolerance and oxidative stress, leading to chronic inflammation. Genetic factors, such as IL-23 receptor gene variants, increase the risk for Psoriasis, Alopecia Areata, and Hashimoto's thyroiditis. Additionally, CTLA-4 mutations enhance susceptibility to autoimmune thyroid and skin disorders. Shared genetic susceptibility was also reported in Lichen Planus and Vitilgo, even if different genetic loci might be involved. The breakdown of the immune system can determine a pro-inflammatory state, facilitating the development of autoimmunity and auto-antibody cross-reactions. The presence of similar antigens in skin cells and thyrocytes might explain why both tissues are affected. The significant overlap between these conditions emphasizes the necessity for a comprehensive diagnosis workup and treatment. Future research should focus on clarifying specific immunological pathways and identifying novel biomarkers. [ABSTRACT FROM AUTHOR]

Hypereosinophilic syndrome (HES) encompasses a heterogeneous and complex group of different subtypes within the wider group of hypereosinophilic disorders. Despite increasing research interest, several unmet needs in terms of disease identification, pathobiology, phenotyping, and personalized treatment remain to be addressed. Also, the prospective burden of non-malignant HES and, more in general, HE disorders is currently unknown. On a practical note, shortening the diagnostic delay and the time to an appropriate treatment approach probably represents the most urgent issue, even in light of the great impact of HES on the quality of life of affected patients. The present document represents the first action that the Italian Society of Allergy, Asthma, and Clinical Immunology (SIAAIC) has finalized within a wider project aiming to establish a collaborative national network on HES (InHES—Italian Network on HES) for patients and physicians. The first step of the project could not but focus on defining a common language as well as sharing with all of the medical community an update on the most recent advances in the field. In fact, the existing literature has been carefully reviewed in order to critically integrate the different views on the topic and derive practical recommendations on disease identification and treatment approaches. [ABSTRACT FROM AUTHOR]

Lysosomal storage diseases (LSDs) are rare genetic metabolic disorders that cause the accumulation of glycosaminoglycans in lysosomes due to enzyme deficiency or reduced function. Enzyme replacement therapy (ERT) represents the gold standard treatment, but hypersensitivity reaction can occur resulting in treatment discontinuation. Thus, desensitization procedures for different culprit recombinant enzymes can be performed to restore ERT. We searched desensitization procedures performed in LSDs and focused on skin test results, protocols and premedication performed, and breakthrough reactions occurred during infusions. Fifty‐two patients have been subjected to desensitization procedures successfully. Skin tests, with the culprit recombinant enzyme, deemed positive in 29 cases, doubtful in two cases, and not performed in four patients. Moreover, 29 of the 52 desensitization protocols used at the first infusion were breakthrough reaction free. Different desensitization strategies have proved safe and effective in restoring ERT in patients with previous hypersensitivity reactions. Most of these events seem to be Type I hypersensitivity reactions (IgE‐mediated). Standardized in vivo and in vitro testing is necessary to better estimate the risk of the procedure and find the safest individualized desensitization protocol. [ABSTRACT FROM AUTHOR]

This document is a reply to a response to an article titled "Hypersensitivity reaction during enzyme replacement therapy in lysosomal storage disorders: a systematic review of desensitization strategies." The response acknowledges and appreciates the feedback provided by the authors of the response. The article discusses the challenges of managing allergic reactions during enzyme replacement therapy (ERT) for metabolic disorders. It highlights the use of a combined method of rapid desensitization and Allergen Immunotherapy-like (AIT-like) desensitization as a promising strategy. The role of IgG-mediated responses in these reactions is still unclear, and further research is needed. Premedication strategies tailored to the nature of previous reactions are crucial in managing ERT-induced hypersensitivity reactions. The decision to reintroduce ERT after a reaction must consider premedication strategies and the patient's overall health. The document emphasizes the need for ongoing research and evolving strategies to ensure optimal patient outcomes and the efficacy of life-saving therapies. [Extracted from the article]

AbstractObjectiveMethodsResultsConclusionsExacerbations and suboptimal disease control are common in severe asthma with an eosinophilic phenotype (SAep). Mepolizumab, an anti-interleukin-5 monoclonal antibody, has demonstrated efficacy and safety in randomized controlled trials (RCTs). We aimed to strengthen the real-world evidence base for mepolizumab in SAep.We analyzed data from Italian participants of REALITI-A, a global, real-world, prospective, observational study (primary outcome: rate of clinically significant exacerbations [CSEs]). Using these data and those from Italian real-world studies of mepolizumab (identified by systematic literature review), we performed a meta-analysis.In the Italian cohort of REALITI-A (n = 244), mean CSE rate was lower 12 months post-mepolizumab initiation versus 12 months pre-mepolizumab (0.67 vs. 3.74 CSEs/patient/year; relative risk [RR], 0.18; 95% confidence interval (CI), 0.15–0.22; p < .001). The meta-analysis included 863 patients. Mean CSE rate decreased from 4.2/patient/year at baseline to 0.71/patient/year post-mepolizumab initiation. Mean oral corticosteroid (OCS) dose reduced by 8.66 mg/day (95% CI, 6.17–11.16 mg/day; p < .0001) from baseline (10.0 mg/day). The RR for OCS maintenance, post- versus pre-mepolizumab, was 0.37 (95% CI, 0.27–0.52; p < .0001). A mean increase in Asthma Control Test score of 6.50 (95% CI, 5.67–7.33; p < .00001) was observed. Proportions of patients reporting adverse events were low.Real-world experience in this unified health care system identifies that mepolizumab has a low adverse event rate and provides consistent clinical benefits. Mepolizumab represents an important treatment option for patients with SAep. [ABSTRACT FROM AUTHOR]