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严重大疱性类天疱疮的不典型表现:一例报告。

Atypical Presentation of Severe Bullous Pemphigoid: A Case Report.

机构信息

Internal Medicine Residency Program, University of South Dakota Sanford School of Medicine.

Sanford USD Medical Center, Sioux Falls, South Dakota.

出版信息

S D Med. 2024 Aug;77(8):362-364.

Abstract

Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disorder. Typically, patients will present with tense bullae and intense generalized pruritus with a skin biopsy demonstrating subepidermal split with eosinophils and a direct immunofluorescence highlighting autoantibodies against the basement membrane zone. Prognosis varies, and treatment involves an assessment of the severity of disease to determine whether to initiate topical or systemic immunosuppressive agents. We present an atypical presentation of BP that presented as a 3-to-4-week duration of pruritic small vesicular lesions in the upper chest, scabbed circular lesions along the upper extremity and pinnas of bilateral ear. Initially thought to be herpes zoster infection initially treated with valacyclovir for a week following a prior concern of a concomitant superficial skin infection with cephalexin and prednisone. With no clinical improvement, tissue biopsy was performed that confirmed bullous pemphigoid and treatment with steroid taper, doxycycline, and triamcinolone acetonide 0.1% cream was started. The aim of this case report is to present an atypical presentation of BP and to highlight maintaining a high index of suspicion of BP in patients presenting with disseminated significantly pruritic lesions.

摘要

大疱性类天疱疮(BP)是最常见的自身免疫性表皮下疱病。通常,患者会出现紧张性大疱和强烈的全身性瘙痒,皮肤活检显示表皮下分离伴嗜酸性粒细胞和直接免疫荧光突出针对基底膜带的自身抗体。预后各不相同,治疗包括评估疾病的严重程度,以确定是否开始使用局部或全身免疫抑制药物。我们提出了一个不典型的 BP 表现,表现为上胸部持续 3-4 周的瘙痒性小水疱性病变,上肢和双侧耳朵尖上结痂的圆形病变。最初被认为是带状疱疹感染,最初用伐昔洛韦治疗了一周,因为之前曾担心同时存在浅层皮肤感染,使用了头孢氨苄和泼尼松。在没有临床改善的情况下,进行了组织活检,证实为大疱性类天疱疮,并开始使用类固醇减量、多西环素和曲安奈德 0.1%乳膏治疗。本病例报告的目的是提出一个不典型的 BP 表现,并强调在出现广泛明显瘙痒性病变的患者中保持对 BP 的高度怀疑。

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