寻常型天疱疮（PV）是一种罕见的自身免疫性疾病，其特征是皮肤粘膜起泡和侵蚀。PV的发病机制涉及B和T细胞，靶向皮肤和口腔粘膜上皮内的细胞间粘附分子，导致棘皮松解术.通常,表现包括口腔粘膜起泡，通常是皮肤病变。考虑到与PV相关的相当大的发病率和死亡率风险，早期诊断至关重要,通常依赖于临床特征的组合，组织病理学,和直接免疫荧光。Bruton酪氨酸激酶（BTK）在自身免疫性疾病和炎症的病理生理中起着重要作用。在这里，我们介绍了一例肺静脉患者,表现出对类固醇一线治疗的抵抗.随后，开始使用BTK抑制剂依鲁替尼治疗，产生有利的结果。这个案例强调了靶向治疗的潜力,如BTK抑制剂，在管理传统治疗方式难治性PV方面。
Pemphigus vulgaris (PV) stands as a rare autoimmune disorder characterized by blistering and erosion of mucocutaneous membranes. The pathogenesis of PV implicates both B and T cells, which target cell-to-cell adhesion molecules within the epithelia of the skin and oral mucosa, leading to acantholysis. Typically, the presentation involves blistering of the oral mucosa, often followed by cutaneous lesions. Given the considerable risk of morbidity and mortality associated with PV, early diagnosis is crucial, typically relying on a combination of clinical features, histopathology, and direct immunofluorescence. Bruton tyrosine kinase (BTK) plays a significant role in the pathophysiology of autoimmune diseases and inflammation. Herein, we present a case of PV that demonstrated resistance to first-line therapy with steroids. Subsequently, treatment with the BTK inhibitor ibrutinib was initiated, yielding favorable outcomes. This case underscores the potential of targeted therapies, such as BTK inhibitors, in managing PV refractory to conventional treatment modalities.
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