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首页> 外文期刊>Journal of cutaneous medicine and surgery >Remission of Refractory Benign Familial Chronic Pemphigus (Hailey-Hailey Disease) with the Addition of Systemic Cyclosporine
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Remission of Refractory Benign Familial Chronic Pemphigus (Hailey-Hailey Disease) with the Addition of Systemic Cyclosporine

机译:全身性环孢菌素治疗可缓解难治性良性家族性慢性天疱疮(Hailey-Hailey病)

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摘要

Background: Benign chronic familial pemphigus (BFCP) is an autosomal dominant dermatosis characterized by flares of painful and often debilitating blistering lesions in high friction areas of the body such as the groin, axillae, lateral neck, and intergluteal cleft. Limited knowledge of its pathophysiology has made treatment of BFCP a considerable challenge and efficacy with current first line therapies, topical corticosteroids and antibiotics, is variable.
机译:背景:良性慢性家族性天疱疮(BFCP)是一种常染色体显性皮肤病,其特征是在身体的高摩擦区域(如腹股沟,腋窝,颈外侧和臀间裂)爆发出疼痛且常常使人衰弱的水疱。对BFCP的病理生理学的有限​​了解使BFCP的治疗面临巨大挑战,而当前的一线疗法,局部皮质类固醇和抗生素的疗效却是可变的。

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