Jemec G B, Moe A T
Department of Dermatology, Bispebjerg Hospital, University of Copenhagen, Denmark.
Pediatr Dermatol. 1996 Jan-Feb;13(1):58-60. doi: 10.1111/j.1525-1470.1996.tb01191.x.
Prolidase deficiency is a hereditary enzyme deficiency characterized dermatologically by chronic recurrent ulcers and scarring due to increased skin fragility. It has been speculated that the enzyme deficiency causes a relative deficiency of proline in the wounds of these patients and negatively affects clinical healing. Two ulcers in a 17-year-old girl with established prolidase deficiency were treated for 12 weeks with ointments containing amino acids in an open study comparing the effects of 5% proline and a combination of 5% proline plus 5% glycine. Both ointments caused significant reduction of the ulcer size (p < 0.02), but the 5% proline-5% glycine mixture caused a more rapid reduction (0.01 < p < 0.02). The results confirm previous findings in this rare inborn error of metabolism.
脯氨肽酶缺乏症是一种遗传性酶缺乏症,其皮肤学特征为由于皮肤脆性增加而导致慢性复发性溃疡和瘢痕形成。据推测,酶缺乏会导致这些患者伤口中脯氨酸相对缺乏,并对临床愈合产生负面影响。在一项开放性研究中,对一名已确诊脯氨肽酶缺乏症的17岁女孩的两处溃疡进行了为期12周的治疗,使用了含氨基酸的药膏,比较了5%脯氨酸和5%脯氨酸加5%甘氨酸组合的效果。两种药膏均使溃疡大小显著减小(p < 0.02),但5%脯氨酸 - 5%甘氨酸混合物导致溃疡缩小更快(0.01 < p < 0.02)。这些结果证实了此前在这种罕见的先天性代谢缺陷中的发现。