Anolik J H, Di Giovanna J J, Gaspari A A
Department of Dermatology, National Institute of Arthritis, Musculoskeletal and Skin Diseases, National Institutes of Health, USA.
Br J Dermatol. 1998 Feb;138(2):236-41. doi: 10.1046/j.1365-2133.1998.02067.x.
Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder characterized by sun sensitivity, defective DNA repair, markedly increased susceptibility to skin cancer, and a variety of immunological defects, including defective natural killer (NK) cell activity. Retinoid therapy has been demonstrated to protect effectively against the development of skin cancers in patients with XP, although its mechanism of action is unknown. We describe a series of eight XP patients, six of whom were given oral isotretinoin. The NK cell activity was not affected by low-dose isotretinoin, i.e. 0.5 mg/kg per day. However, higher doses of isotretinoin, e.g. 1.0 mg/kg per day, produced a significant decrease in NK cell function, at the same time as producing a reduction in the frequency of development of skin cancers. Retinoid therapy may have a skin cancer preventing effect by enhancing other immune effector mechanisms or via epithelial cell differentiation.
着色性干皮病（XP）是一种罕见的常染色体隐性疾病，其特征为对阳光敏感、DNA修复缺陷、患皮肤癌的易感性显著增加以及多种免疫缺陷，包括自然杀伤（NK）细胞活性缺陷。维甲酸疗法已被证明可有效预防XP患者皮肤癌的发生，但其作用机制尚不清楚。我们描述了一组8例XP患者，其中6例接受了口服异维甲酸治疗。低剂量异维甲酸（即每天0.5mg/kg）对NK细胞活性没有影响。然而，较高剂量的异维甲酸（如每天1.0mg/kg）会使NK细胞功能显著下降，同时皮肤癌的发生频率也会降低。维甲酸疗法可能通过增强其他免疫效应机制或通过上皮细胞分化来发挥预防皮肤癌的作用。
