類天疱瘡（後天性表皮水疱症を含む。）

Bullous pemphigoid (BP) is an autoimmune blistering disease with an increasing incidence in recent years; however, the underlying immune regulatory mechanisms remain largely unclear. As a critical signalling hub linking innate and adaptive immunity, stimulator of interferon genes (STING) has recently been implicated in the pathogenesis of various autoimmune diseases and may regulate tissue inflammation and immune homeostasis through modulation of CD4+ T cell responses. In this study, we found that STING expression was significantly increased in lesional skin tissues from patients with BP compared with healthy controls. Transcriptomic analysis further revealed that differentially expressed genes in peripheral blood CD4+ T cells from BP patients were primarily enriched in the JAK-STAT signalling pathway, T cell activation and differentiation, and type I interferon (IFN-I)-related pathways. Pharmacological inhibition of STING markedly attenuated the aberrant activation of these signalling pathways. Moreover, qRT-PCR analysis confirmed that the mRNA levels of STING1, JAK1, and CXCR5 were significantly elevated in BP patients, whereas treatment with the STING inhibitor C176 suppressed the expression of these molecules. Collectively, our findings suggest that STING may contribute to BP immunopathogenesis by regulating the JAK-STAT signalling axis and promoting abnormal CD4+ T cell activation and differentiation, providing new insights into the molecular mechanisms underlying BP and identifying potential therapeutic targets.

Pemphigus comprises autoimmune blistering diseases with heterogeneous clinical presentations, but pre-referral provisional diagnoses in regional tertiary dermatology practice have been less well characterized. We retrospectively reviewed 35 consecutive patients diagnosed with pemphigus at Toyama University Hospital, a regional tertiary dermatology center in Japan, between January 2011 and May 2026. Final diagnoses were established using clinical, histopathological, immunopathological, and serological findings. We extracted referral characteristics, pre-referral provisional diagnoses, final diagnoses, disease severity, and dates of symptom onset and referral visit from medical records. The median age was 69 years, and 20 patients (57.1%) were female. Final diagnoses were pemphigus vulgaris in 15 patients (42.9%), pemphigus foliaceus in 10 (28.6%), paraneoplastic pemphigus in 3 (8.6%), intercellular IgG/IgA dermatosis in 3 (8.6%), pemphigus herpetiformis in 2 (5.7%), pemphigus vegetans in 1 (2.9%), and unclassified pemphigus in 1 (2.9%). Only 10 patients (28.6%) had pemphigus recorded as the pre-referral diagnosis; other pre-referral diagnoses included impetigo, Behçet's disease, refractory stomatitis, dermatitis/eczema, pemphigoid, and drug eruption. Exploratory cosinor analysis suggested possible annual variation in the interval from symptom onset to referral visit, with longer intervals around winter and shorter intervals around summer. By mapping pre-referral diagnoses to final pemphigus diagnoses, this study highlights heterogeneity in diagnostic subtypes and referral pathways through which pemphigus reaches regional tertiary dermatology care.