Berger Emily M, Galadari Hassan I, Gottlieb Alice B
Department of Dermatology, Tufts-New England Medical Center, Tufts University School of Medicine, Boston, MA 02111, USA.
J Drugs Dermatol. 2007 Jul;6(7):734-6.
Hailey-Hailey disease is an autosomal dominant skin condition characterized by waxing and waning painful and pruritic vesicles and plaques affecting the intertriginous areas. Its pathogenesis involves inherited abnormalities in a cutaneous calcium pump. Most patients are managed conservatively with topical corticosteroids as well as topical and oral anti-infective agents. Scarce reports in the literature describe the use of oral retinoid therapy to manage refractory cases. We present a case of Hailey-Hailey disease in a 64-year-old man who was refractory to conservative management but improved dramatically over 6 months of oral therapy with 25 mg of acitretin daily. The mechanism by which such therapy improves disease manifestations is unknown. A potential mechanism is based on the influence of retinoids on epidermal differentiation and may involve cutaneous calcium homeostasis. Hailey-Hailey disease is discussed and the use of oral retinoid treatment for Hailey-Hailey disease is reviewed.
黑利-黑利病是一种常染色体显性遗传性皮肤病，其特征为反复出现的、影响皮肤褶皱部位的疼痛性和瘙痒性水疱及斑块。其发病机制涉及皮肤钙泵的遗传性异常。大多数患者采用局部糖皮质激素以及局部和口服抗感染药物进行保守治疗。文献中关于使用口服维甲酸类药物治疗难治性病例的报道较少。我们报告一例64岁男性黑利-黑利病患者，该患者对保守治疗无效，但在每日口服25 mg阿维A进行6个月的治疗后有显著改善。这种治疗改善疾病表现的机制尚不清楚。一种潜在机制基于维甲酸类药物对表皮分化的影响，可能涉及皮肤钙稳态。本文对黑利-黑利病进行了讨论，并对口服维甲酸类药物治疗黑利-黑利病的应用进行了综述。
