Pathogenesis of Port-Wine Stains: Directions for Future Therapies.

Pathogenesis of Port-Wine Stains: Directions for Future Therapies.
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DOI:
10.3390/ijms232012139
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发表时间:
2022-10-12
影响因子:
5.6
通讯作者:
--
中科院分区:
生物学2区
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--
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葡萄酒斑是一种涉及皮肤和粘膜的先天性血管畸形。迄今为止,pws发病和进展的机制尚未清楚阐明。血管扩张的可能原因有:(1)体细胞GNAQ (R183Q)突变,通过血管生成素-2形成毛细血管畸形样血管增大;(2)血管周围神经元素减少;(3)Eph受体B1和ephrin B2共存;(4)周细胞αSMA表达不足。此外,ERK、c-JNK、P70S6K、AKT、PI3K和PKC被认为参与了PWS的发展。虽然脉冲染料激光(PDL)仍然是治疗PWSs的金标准,但复发率很高。局部药物,包括咪喹莫特、阿西替尼和雷帕霉素,联合PDL治疗,有望改变pws的复发率并减少PDL的次数。对于深血管丛,纳米载体包封表面标记物(CD133/CD166/VEGFR-2)的光敏剂或光热转导剂在光动力或光热治疗pss方面具有广阔的应用前景。pws的发病机制、进展和治疗应进行广泛的研究。
Port-wine stains (PWSs) are congenital vascular malformations that involve the skin and mucosa. To date, the mechanisms underlying the pathogenesis and progression of PWSs are yet to be clearly elucidated. The potential reasons for dilated vessels are as follows: (1) somatic GNAQ (R183Q) mutations that form enlarged capillary malformation-like vessels through angiopoietin-2, (2) decreased perivascular nerve elements, (3) the coexistence of Eph receptor B1 and ephrin B2, and (4) the deficiency of αSMA expression in pericytes. In addition, ERK, c-JNK, P70S6K, AKT, PI3K, and PKC are assumed to be involved in PWS development. Although pulsed-dye laser (PDL) remains the gold standard for treating PWSs, the recurrence rate is high. Topical drugs, including imiquimod, axitinib, and rapamycin, combined with PDL treatments, are expected to alter the recurrence rate and reduce the number of PDL sessions for PWSs. For the deep vascular plexus, photosensitizers or photothermal transduction agents encapsulated by nanocarriers conjugated to surface markers (CD133/CD166/VEGFR-2) possess a promising therapeutic potential in photodynamic therapy or photothermal therapy for PWSs. The pathogenesis, progression, and treatment of PWSs should be extensively investigated.
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