Journal of Dermatology最新文献

Toxic epidermal necrolysis (TEN) is a rare, life-threatening dermatologic condition, typically triggered by medications. While increased short-term mortality in this group is well documented, few studies have quantified this risk over extended follow-up. We aimed to examine both short- and long-term mortality and assess whether excess mortality persists beyond the acute phase. We conducted a nationwide, register-based matched cohort study using Danish health registries. Patients with a first-time hospital diagnosis of TEN between 1995 and 2024 were included and matched 1:100 on age and sex with population controls. Short-term mortality (30- and 90-day) was calculated, and long-term mortality was assessed overall and in a landmark analysis restricted to 2-year survivors with newly matched controls. We identified 145 individuals hospitalized with TEN and matched them to 14 500 population controls. The mean age was 56 years, and half were female. Short-term mortality was high, with 28% dying within 90 days. Median survival was 4.77 years (95% CI: 2.02–9.46) for TEN patients versus 25.96 years (95% CI: 25.19–27.20) for controls. Two years after diagnosis, TEN patients still had almost triple the mortality risk compared to controls (HR 3.30; 95% CI: 2.34–4.66). Excess mortality was also observed among patients without any recorded comorbidities at the time of diagnosis, indicating that the increased risk was not solely attributable to preexisting health conditions. Most deaths were due to natural causes; fewer than five were attributed to unnatural or unknown causes. In conclusion, this study quantifies the long-term reduction in survival following TEN. Our estimates captured the real-world excess mortality burden of TEN, arising from the acute disease, sequelae, or comorbidities, thus providing a clinically relevant picture of long-term prognosis. The increased mortality risk was not explained by baseline comorbidity alone, underscoring that TEN itself contributes to a poorer long-term prognosis.

We often experience sustained improvement, even after discontinuation of treatment in psoriasis lesions treated with topical vitamin D₃ (VD₃) or the combination of topical corticosteroids and VD₃. However, the underlying mechanisms of these sustained effects remain unclear. We explored mechanisms for the sustained effects of maxacalcitol (MCT) and combined MCT/betamethasone butyrate propionate (BBP) ointments using a murine psoriasiform dermatitis model induced by imiquimod (IMQ). IMQ was applied once daily to the shaved backs of mice for 6 days to induce psoriasiform dermatitis. MCT, BBP, combined MCT/BBP ointment, or their vehicles were treated for 3 days prior to IMQ application. IMQ was reapplied after 1, 2, or 3 weeks from the first IMQ application to investigate the sustained effects of their ointments. In the first application of IMQ, the administration of MCT, BBP, or MCT/BBP ointment improved clinical and pathological manifestations and reduced Th17-related cytokines. Treatment with MCT or MCT/BBP showed an increase in IL-10 mRNA expression and a higher count of Foxp3⁺ cells within the skin, but not in those with BBP. The induction of IL-10 by MCT and MCT/BBP persisted until reapplication of IMQ 2 weeks later, although their effects diminished 3 weeks later. The reduction in Th17-related cytokines was maintained up to 3 weeks later in MCT/BBP, whereas it was not observed 2 weeks later in MCT. In conclusion, MCT and MCT/BBP showed long-term effects by induction of regulatory T cells and IL-10. Additionally, MCT/BBP downregulated Th17-related cytokines, which could contribute to the sustained improvement after discontinuation observed in clinical practice.

Genetic pigmentary disorders represent a diverse group of genetic conditions characterized by alterations in melanin production and transport and melanocyte development, resulting from single-gene pathological variants. These disorders encompass both hypopigmentary and hyperpigmentary phenotypes, affecting not only skin pigmentation but also ocular, auditory, and systemic manifestations. This review examines the molecular mechanisms underlying major genetic pigmentary disorders, including hypopigmentary (e.g., oculocutaneous albinism, piebaldism, and Waardenburg syndrome) and hyperpigmentary (e.g., dyschromatosis symmetrica hereditaria, dyschromatosis universalis hereditaria, reticulate acropigmentation of Kitamura, and Dowling–Degos disease) disorders. Additionally, we discuss RASopathies, in which pigmentary abnormalities occur alongside multisystem developmental anomalies. Comprehensive understanding of these conditions can provide crucial insights into melanocyte biology and guide future clinical management strategies for affected patients.