Italian Journal of Dermatology and Venereology最新文献

Morphea is a rare, clinically diverse inflammatory fibrosing skin condition presenting in children and adults. More severe subtypes of morphea can result in significant cosmetic and functional morbidity and warrant early systemic therapeutic intervention to minimize the risk of permanent tissue damage. Significant challenges exist in consensus classification and the development of robust validated measures of disease activity and damage. As a consequence, there are presently no approved therapies, and widely utilized consensus treatment guidelines are also lacking. At the core of the treatment challenge of morphea, is a limited understanding of disease immunopathogenic mechanisms. Pleasingly, some progress has been made in recent years, with advances in transcriptomic and immunologic profiling now defining morphea as a skin-directed, pro-inflammatory disorder which is likely a type 1 interferonopathy. Lesional skin demonstrates enrichment of type I interferon pathways, Th1 and Th17 cytokines, and B-cell activation, with growing evidence implicating keratinocyte-derived signaling in disease propagation. Accordingly, agents including Janus kinase (JAK) inhibitors, abatacept, and tocilizumab have shown some encouraging outcomes in small case series and observational studies, particularly in generalized, deep and/or treatment-resistant disease. Potential novel approaches targeting epidermal-dermal crosstalk, plasmacytoid dendritic cells, or fibroblast positional identity may offer future precision in treatment, but require expansion of our knowledge of molecular etiopathogenesis to be progressed. This narrative review aims to integrate current knowledge of clinical heterogeneity, molecular etiopathogenesis, and therapeutic clinical data to propose a forward-looking, mechanism-based framework for the clinical management of morphea.

Background: Kaposi sarcoma (KS) is an angioproliferative tumor linked to Human Herpesvirus 8 (HHV8) infection, presenting in five clinical variants. While much research has focused on its clinical characteristics and treatment, limited data exist on its psychological impact and effect on quality of life (QoL). This study aims to evaluate the emotional burden of KS in a large cohort of Italian patients.

Methods: A cross-sectional observational study was conducted at a tertiary care center in Milan, Italy, from January to December 2023. Patients with KS were recruited and completed the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ-C30) and the Depression Anxiety Stress Scales short version (DASS-21). Clinical and demographic data were collected, and statistical analysis was performed using logistic regression and Student's t-test to compare QoL scores with general population norms.

Results: A total of 121 patients (82.6% male; mean age 75.7 years) participated. The majority (93.3%) had lesions on the lower limbs, with 40.8% presenting visible lesions. Compared to the general population, KS patients had significantly lower cognitive functioning scores (P=0.0217) but higher global health status scores (P=0.0070). Moderate-severe depression, anxiety, and stress were reported in 14.2%, 15.8%, and 15.0% of patients, respectively. Visceral involvement was significantly associated with moderate-severe depression (P=0.013), whereas lesion staging did not correlate with psychological distress.

Conclusions: Despite stable physical health, KS patients experience a significant psychological burden, particularly those with visceral involvement or visible lesions. These findings underscore the need for integrated psychological support to improve coping strategies and overall well-being in KS patients. Future longitudinal studies are warranted to further investigate the evolving psychological impact of KS.

Background: Systemic type 2 inflammation, driven by T helper 2 (Th2)-type immune responses, is central to allergic inflammatory diseases, including atopic dermatitis, allergic rhinitis, asthma, nasal polyposis, and food allergy. These conditions frequently coexist and share common immunological mechanisms. The objective of this study is to describe the design and development of T2-Reg, a regional registry for Th2-mediated diseases, aimed at collecting real-life data to improve patient management and research.

Methods: This multicentric retrospective and prospective observational study has been conducted through an electronic web registry. Ethical approval was obtained (N° 22045). Patients with atopic dermatitis, allergic asthma, or allergic rhinitis have been enrolled from dermatology, pulmonology, and otolaryngology units across multiple hospitals in Tuscany, Italy. Data have been collected via REDCap, ensuring security and compliance with GDPR. The registry includes demographic, clinical, and laboratory data, treatment history, and disease-specific clinometric scores.

Results: From June 2022 to December 2024, 619 patients were enrolled, with an equal gender distribution and diverse educational backgrounds. Atopic comorbidities were present in 73% of cases, with allergic rhinitis being the most common. Disease severity indices and treatment history were recorded, including systemic and biologic therapies.

Conclusions: T2-Reg serves as a valuable tool for tracking disease progression, treatment outcomes, and comorbidities. Its integration with AI and interoperability with national and international registries enhances research potential. Despite some limitations, such as data entry burden and missing information, T2-Reg contributes to personalized medicine and public health initiatives in atopic diseases.

Cheilitis refers to an acute or chronic inflammation of the lips. It may occur in isolation, affecting only the vermilion border, or may be associated with oral or perioral involvement. Numerous dermatological and systemic diseases may involve the lips, making the clinical assessment of cheilitis particularly challenging. Various types of cheilitis distinguished by frequency, cause, and duration have been described in the literature. However, there are no univocal recommendations on classification. This article aims to introduce a classification of cheilitis based on etiology, according to the following categories: 1) isolated cheilitis, referring to inflammation confined exclusively to the lips, without systemic manifestations or oral cavity involvement. This group includes cheilitis simplex, contact cheilitis, exfoliative cheilitis, granulomatous cheilitis, glandular cheilitis, plasma cell cheilitis, actinic cheilitis, and infectious cheilitis; 2) cheilitis associated with dermatological conditions that exhibit little to no systemic involvement, such as atopic dermatitis, lichen planus, autoimmune bullous diseases, discoid lupus erythematosus, and psoriasis; 3) cheilitis associated with systemic diseases when lip inflammation occurs in the context of systemic conditions, including systemic lupus erythematosus, orofacial granulomatosis, sarcoidosis, Crohn's disease, Melkersson-Rosenthal syndrome and nutritional deficiencies; and 4) drug-induced cheilitis, when a clear causal relationship is established between drug exposure and lip inflammation, as observed in cases of cheilitis induced by oral retinoids, erythema multiforme, Stevens-Johnson syndrome, toxic epidermal necrolysis, and DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome. This proposed classification may offer clinicians a practical tool for the systematic evaluation and management of patients presenting with cheilitis.