Vanderbeck Kaitlin A, Giroux Lyne, Murugan Nirosha J, Karbowski Lukasz M
Department of Medicine, Northern Ontario School of Medicine , Greater Sudbury, Canada.
Department of Medicine, University of Ottawa , Canada.
Dermatol Reports. 2014 Nov 3;6(1):5604. doi: 10.4081/dr.2014.5604. eCollection 2014 Feb 17.
Hailey-Hailey disease (HHD) is a chronic familial bullous disease characterized by recurrent blisters and erosions typically at friction-prone areas of the body accompanied by acantholysis upon histologic examination. There are a number of therapies used in the management of HHD. Its symptoms have been effectively treated with antimicrobial therapies, corticosteroids and other agents such as cyclosporine and prednisone. However, such treatments are not always effective. Therefore, there is a need for new treatments for the management of HHD. In this report, a patient with long-standing HHD responsive only to high levels of prednisone is described. After the successful tapering and cessation of oral prednisone the patient began a new combination therapy of complementary doses of oral alitretinoin, and narrowband UVB therapy, which yielded a favorable response within 2-3 weeks. After 6 weeks, a mono-therapy of daily (30 mg) oral alitretinoin was sufficient to maintain successful near-complete remission of the disease.
黑利-黑利病（HHD）是一种慢性家族性大疱性疾病，其特征为身体易摩擦部位反复出现水疱和糜烂，组织学检查可见棘层松解。治疗HHD有多种疗法。抗菌疗法、皮质类固醇以及环孢素和泼尼松等其他药物已有效治疗其症状。然而，此类治疗并非总是有效。因此，需要新的治疗方法来管理HHD。在本报告中，描述了一名长期患有HHD且仅对高剂量泼尼松有反应的患者。在成功逐渐减少并停用口服泼尼松后，该患者开始采用口服阿利维A酸补充剂量与窄谱中波紫外线（UVB）疗法的新联合治疗，在2至3周内产生了良好反应。6周后，每日口服阿利维A酸（30毫克）的单一疗法足以维持疾病几乎完全缓解的成功状态。
