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一名老年男性的寻常型天疱疮:病例报告及文献综述

Pemphigus Vulgaris in a Sexagenarian Male: A Case Report and Review of Literature.

作者信息

Dieguez Santiago Duniesky, Abdelmoneim Sahar S, Perez Fonte Angelica, Baglar Esra, Frontela Odalys

机构信息

Family Medicine, Larkin Community Hospital Palm Springs Campus, Hialeah, USA.

Internal Medicine, Larkin Community Hospital Palm Springs Campus, Hialeah, USA.

出版信息

Cureus. 2024 Nov 25;16(11):e74409. doi: 10.7759/cureus.74409. eCollection 2024 Nov.

Abstract

Pemphigus vulgaris (PV) is a rare autoimmune disorder characterized by mucocutaneous blistering due to autoantibodies targeting desmoglein proteins, leading to acantholysis. This case report presents a 60-year-old Hispanic male patient with a history of hypertension who developed PV, initially presenting with pruritic scalp lesions that progressively spread to the nares, mouth, chest, neck, and inguinal region. Despite initial management with topical treatments, the lesions persisted, prompting hospitalization. Physical examination revealed characteristic skin and mucosal lesions, and a punch biopsy confirmed PV. The patient's management involved systemic corticosteroids and supportive care, resulting in significant improvement of the lesions. This case emphasizes the challenges in recognizing and managing PV. The symptom progression from isolated scalp involvement to multiple mucocutaneous sites highlights PV's clinical variability, which can complicate early recognition. Prompt diagnosis and a multidisciplinary approach are crucial for optimizing patient outcomes, preventing disease progression, and addressing the challenges posed by comorbid conditions.

摘要

寻常型天疱疮(PV)是一种罕见的自身免疫性疾病,其特征是针对桥粒芯糖蛋白的自身抗体导致黏膜皮肤水疱形成,进而引起棘层松解。本病例报告介绍了一名60岁有高血压病史的西班牙裔男性患者,他患上了PV,最初表现为头皮瘙痒性病变,逐渐蔓延至鼻孔、口腔、胸部、颈部和腹股沟区。尽管最初采用局部治疗,但病变持续存在,促使患者住院治疗。体格检查发现了特征性的皮肤和黏膜病变,穿刺活检确诊为PV。患者的治疗包括全身使用皮质类固醇和支持性护理,病变得到了显著改善。本病例强调了识别和管理PV的挑战。症状从孤立的头皮受累发展到多个黏膜皮肤部位,突出了PV的临床变异性,这可能使早期识别变得复杂。及时诊断和多学科方法对于优化患者预后、预防疾病进展以及应对合并症带来的挑战至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8faf/11669324/89a9cc49fb29/cureus-0016-00000074409-i01.jpg

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