37岁女性大疱性类天疱疮1例报告并文献复习

Stefon Monique D. Oxley, Brian M. Denney
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摘要

大疱性类天疱疮是一种起泡性疾病,主要影响70岁以上的老年人。它是由自身免疫对基底角化细胞中半粒染色体蛋白的反应引起的,引起炎症级联和随后的大泡形成。在婴儿、儿童和中年人中很少遇到。在此,我们报告一位37岁女性病患的大疱性类天疱疮。患者有3个月的面部、颈部、躯干、四肢屈肌和伸肌表面多处浆液充盈的紧张性水泡病史,直至大腿下部,伴有擦伤、脱皮、糜烂和结痂。未见粘膜受累。病灶在破裂时伴有强烈的瘙痒和疼痛。患者无其他主诉,既往病史一般。患者最初接受抗生素治疗,但未见病情改善。对皮肤进行组织学检查,发现皮下水疱性皮炎伴明显的中性粒细胞浸润。未见菌丝或孢子。根据这些发现,患者入院并接受全身类固醇、抗生素和抗瘙痒药物治疗。直接免疫荧光检查结果与大疱性类天疱疮的诊断一致。患者对治疗的总体反应令人满意。大疱性类天疱疮与其他表皮下大疱性疾病的区别是重要的,因为其他疾病可能有全身表现和并发症。本病例报告强调了临床、组织病理学和免疫学结果在确诊大疱性类天疱疮中的重要性。
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Bullous Pemphigoid in a 37-Year Old Female: A Case Report and Literature Review
Bullous pemphigoid is a blistering disorder which mainly affects the geriatric population predominantly older than 70 years. It is caused by an autoimmune reaction to the hemidesmosomal proteins in basal keratinocytes, causing an inflammatory cascade and subsequent bullae formation. It is rarely encountered in infants, children, and middle-aged adults. Herein, a case of Bullous pemphigoid in a 37-year old female patient is reported. The patient presented with a three-month history of multiple serous fluid-filled tense blisters on the face, neck, trunk, flexor and extensor surfaces of the extremities up to the lower thigh, with areas of excoriation, peeling, erosion and crusting. No involvement of the mucous membranes noted. The lesions were associated with intense pruritus and pain upon rupture. Patient had no other subjective complaints and had an unremarkable past medical history. Patient was initially treated with antibiotics but noted no improvement in her condition. Histologic evaluation of the skin was done and revealed a subepidermal vesicular dermatitis with prominent neutrophilic infiltrates. No hyphae or spores were seen. With these findings, patient was admitted and treated with systemic steroids, antibiotics, and antipruritic medications. Direct Immunofluorescence was done and yielded findings consistent with the diagnosis of Bullous Pemphigoid. The overall response of the patient to therapy was satisfactory. The differentiation of Bullous pemphigoid from other subepidermal bullous diseases is important due to the potential of systemic manifestations and complications of the other diseases. The importance of clinical, histopathologic and immunologic findings in confirming the diagnosis of Bullous Pemphigoid is highlighted in this case report.
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