Gen Dent. 2022 Nov-Dec;70(6):22-26.
Pemphigus vulgaris (PV) is a rare painful and blistering autoimmune mucocutaneous disorder that appears in middle-aged adults. Oral lesions typically precede cutaneous involvement and tend to be more recalcitrant to therapy. The objective of this article is to present a case of oral and cutaneous PV in an atypical patient, a 23-year-old woman. The case was distinguished by the patient's age, which was 2 to 3 decades younger than the reported mean age of onset, and its coincidence with celiac disease, an immunopathologic process rarely seen in association with PV. Intravenous administration of the monoclonal antibody rituximab provided rapid clinical improvement in the cutaneous lesions and gradual improvement in the oral lesions after 2 infusions. Dental practitioners should remain vigilant for oral manifestations of dermatologic disease and refer affected patients to appropriate healthcare providers for long-term management.
寻常型天疱疮（PV）是一种罕见的疼痛性水疱性自身免疫性黏膜皮肤疾病，多见于中年人。口腔病变通常先于皮肤受累，且往往对治疗更具抵抗性。本文的目的是报告一例发生于非典型患者（一名 23 岁女性）的口腔和皮肤寻常型天疱疮病例。该病例的特征为患者年龄较报告的平均发病年龄年轻 20-30 岁，且与乳糜泻同时发生，后者是一种免疫病理过程，很少与寻常型天疱疮相关。静脉注射单克隆抗体利妥昔单抗使皮肤病变迅速得到临床改善，在 2 次输注后口腔病变逐渐改善。牙科医生应警惕皮肤疾病的口腔表现，并将受影响的患者转介给适当的医疗保健提供者进行长期管理。
