Significant Improvement of Persistent Hailey-Hailey Disease with Dupilumab: A Case Report

Malvika Ramesh, Brinda Chellappan, Lindy Ross
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Abstract

Hailey-Hailey disease is a rare, genetic blistering disease characterized by erythematous, scaly plaques in intertriginous regions such as the axillary, inframammary, and groin regions. The plaques cause discomfort and are difficult to treat. Treatment ranges from topical to systemic and surgical. Despite the variety of treatment options, Hailey-Hailey disease is notoriously difficult to treat with each patient responding uniquely to treatment. There has been evidence of patients with Hailey-Hailey disease experiencing improvement of symptoms with dupilumab injections. Dupilumab has several off-label uses, including allergic contact dermatitis, hand dermatitis, chronic spontaneous urticaria, and alopecia areata, showing promise for its use in dermatology outside of atopic dermatitis and prurigo nodularis. Due to its minimal side effects, dupilumab can be tried for Hailey-Hailey disease with little risk. We present the case of a 37-year-old female with recalcitrant Hailey-Hailey disease who noticed significant improvement on dupilumab.   
杜匹单抗显著改善顽固性海利-海利病:病例报告
海利-海利病是一种罕见的遗传性水疱病,其特征是在腋窝、乳房下和腹股沟等三叉神经间区域出现红斑、鳞屑性斑块。斑块会引起不适,而且难以治疗。治疗方法包括局部治疗、全身治疗和手术治疗。尽管治疗方法多种多样,但海利-海利病是出了名的难治病,每个患者对治疗的反应都不尽相同。有证据表明,海利-海利病患者在注射杜匹单抗后症状有所改善。杜比鲁单抗有几种标示外用途,包括过敏性接触性皮炎、手部皮炎、慢性自发性荨麻疹和斑秃,显示了它在特应性皮炎和结节性瘙痒症以外的皮肤科领域的应用前景。由于其副作用极小,杜比鲁单抗用于海利-海利病的风险很小。我们介绍了一例患有顽固性海利-海利病的 37 岁女性患者的病例。
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