European Journal of Dermatology最新文献

Generalised pustular psoriasis (GPP) is a rare, chronic, inflammatory skin disease that may manifest as a relapsing disease with recurrent flares or a persistent disease with intermittent flares. GPP is associated with a significant clinical burden, including pain, fatigue, and comorbidities, leading to severe, life-threatening complications. The frequency and severity of GPP flares have not been characterised comprehensively in epidemiological studies. Comprehensive literature searches were conducted to identify studies evaluating mortality, prevalence, comorbidities, and flares in the GPP population, with 121 studies included in total. As expected, mortality rates were generally higher in studies with longer durations; in three large European studies, mortality rates were 30.8%, 24.4%, and 4.8% across the years 2004-2015, 2010-2018, and 2016-2020, respectively. The most frequently reported cause of death was sepsis, followed by cardiovascular complications, and disease-related mortality. GPP prevalence was 0.88-198 patients per million in the general population (9/121 studies), although the higher estimate may be inflated due to methodological approaches. Plaque psoriasis and joint disorders (affecting >40% and <20% patients in most studies, respectively) were the most frequently reported GPP-associated comorbidities (73/121 studies). In two of the largest database studies, patients with GPP experienced approximately one flare per patient-year. Mean hospitalisation duration was 8.6-30 days. This comprehensive analysis highlighted substantial between-study heterogeneity, emphasising the need for standardization of diagnostic criteria and aspects of study design (including long-term studies with sufficient patient numbers and consistent follow-up durations). Nevertheless, the epidemiological evidence indicates that GPP is a rare, potentially lethal disease with frequent comorbidities. New treatment alternatives that might improve the management of patients with GPP, to treat and prevent flares, should be evaluated urgently.

Mastocytosis encompasses a heterogeneous group of clonal mast cell disorders ranging from cutaneous forms in children to advanced systemic mastocytosis (AdvSM) with significant morbidity. This review covers epidemiology, pathogenesis, diagnosis, and management, integrating World Health Organization (WHO)/International Consensus Classification (ICC) 2022 classification updates, molecular insights, and therapeutic advances. Pediatric cutaneous mastocytosis (CM) usually has a favourable prognosis and often resolves spontaneously, whereas adult-onset disease frequently reflects systemic involvement and warrants bone marrow biopsy. KIT D816V and non-D816V variants serve as key diagnostic and prognostic markers, guiding targeted therapy. Midostaurin and avapritinib have reshaped the treatment of AdvSM, while next-generation tyrosine-kinase inhibitors (TKIs) are in clinical trials. Multidisciplinary care is critical, and knowledge gaps remain in paediatric risk stratification, optimal sequencing of therapies, and disease progression assessment. This review highlights recent advances and future priorities for personalized, evidence-based care.

Severe Demodex infestation causes demodicosis, but its impact on skin function remains unclear due to a lack of sensitive detection methods. To develop an accurate, user-friendly detection method and assess the effects of Demodex on skin function. A modified squeezing/scraping method (MSS) was developed, combining mud pretreatment cleaning with traditional squeezing and scraping. Skin function was then assessed using Antera3D and a lactic acid sting test. The proposed method not only led to more consistent results compared with the traditional squeezing/scraping method, but also showed a higher detection rate by increasing the positive infestation threshold from 5 to 13 mites. Based on a subsequent assessment for skin physiological parameters, we found that aging was a risk factor for Demodex mite infestation. In addition, Demodex mite infestation was associated with skin barrier damage, characterized as reduction in skin water content, elevated transepidermal water loss, stimulated sebum secretion, and increased skin roughness, which may eventually lead to sensitive skin. The MSS method improves Demodex detection. Infestation may contribute to sensitive skin and may be a therapeutic target.