Giustini Sandra, Miraglia Emanuele, Berardesca Enzo, Milani Massimo, Calvieri Stefano
Genodermatosis Service, Dermatology Clinic, University 'La Sapienza', Rome, Italy.
San Gallicano Dermatological Institute, Rome, Italy.
Case Rep Dermatol. 2014 Sep 19;6(3):222-6. doi: 10.1159/000368182. eCollection 2014 Sep.
Skin cancer is common in xeroderma pigmentosum (XP) due to a DNA repair mechanisms genetic defect. Ultraviolet (UV) exposure is the main cause of increased incidence of actinic keratosis (AK), basal cell carcinoma (BCC) and squamous cell carcinoma (SCC) observed in XP subjects. Photoprotection is therefore a mandatory strategy in order to reduce skin damage. A topical DNA repair enzyme has been shown to slow down the development of skin lesions in XP. However, there are no data regarding the effects of photoprotection combined with DNA repair strategies in this clinical setting. A film-forming medical device containing the DNA repair enzyme photolyase and very high-protection UV filters (Eryfotona AK-NMSC, Ery) is currently available. We report retrospective data regarding the use of Ery in 8 patients (5 women, 3 men) with a diagnosis of XP treated for at least 12 consecutive months, comparing the rate of new skin lesions (AK, BCC and SCC) during active treatment with Ery and during 12 months just before the use of the product. New AK, BCC and SCC mean lesion numbers during the 1-year Ery treatment were 5, 3 and 0, respectively in comparison with 14, 6.8 and 3 lesions, respectively during the 1-year pre-treatment period. Ery use was associated with a 65% reduction in appearance of new AK lesions and with 56 and 100% reductions in the incidence of new BCC and SCC lesions, respectively. These data suggest that topical use of photoprotection and DNA repair enzyme could help lower skin cancer lesions in XP. Control prospective trials are advisable in this clinical setting.
由于DNA修复机制的基因缺陷，皮肤癌在着色性干皮病（XP）中很常见。紫外线（UV）暴露是XP患者中光化性角化病（AK）、基底细胞癌（BCC）和鳞状细胞癌（SCC）发病率增加的主要原因。因此，光防护是减少皮肤损伤的一项必要策略。一种局部DNA修复酶已被证明可减缓XP患者皮肤病变的发展。然而，在这种临床情况下，关于光防护与DNA修复策略联合使用的效果尚无数据。目前有一种含有DNA修复酶光解酶和极高防护紫外线滤光片的成膜医疗器械（Eryfotona AK-NMSC，Ery）。我们报告了8例（5名女性，3名男性）诊断为XP且连续治疗至少12个月的患者使用Ery的回顾性数据，比较了使用Ery进行积极治疗期间和使用该产品前12个月内新皮肤病变（AK、BCC和SCC）的发生率。在为期1年的Ery治疗期间，新的AK、BCC和SCC平均病变数分别为5、3和0，而在为期1年的治疗前期分别为14、6.8和3个病变。使用Ery可使新AK病变的出现减少65%，新BCC和SCC病变的发生率分别降低56%和100%。这些数据表明，局部使用光防护和DNA修复酶可能有助于减少XP患者的皮肤癌病变。在这种临床情况下，建议进行对照前瞻性试验。
