The Role of Angiogenesis-related Pathways in the Development of Port Wine Stains

Air Force General Hospital of the PLA, First Hospital of China Medical University, Chinese Academy of Medical Sciences

Port wine stain (PWS) is a congenital, progressive vascular malformation of human skin involving the superficial vascular plexus that occurs in estimated 3-5 children per 1,000 live births. In childhood, PWS are flat red macules, but lesions tend to darken progressively to purple and, by middle age, often become raised as a result of the development of vascular nodules. Because most malformations occur on the face, PWS is a clinically significant problem in the majority of patients. The late-stage cobblestoning appearance of PWS subjects is comprised by not only pronounced vascular ectasia with proliferation of thin and/or thick-walled vessels and their stroma, but also numerous epithelial, neural and mesenchymal hamartomatous abnormalities. Despite these histologic observations, the specific mechanisms involved in PWS nodular formation remains unclear. In one nodular PWS subject, we found that phosphatidylinositol 3-kinases (PI3K)/protein kinase B (AKT) and phosphoinositide phospholipase C g subunit (PLC-g) were activated in both hypertrophic areas and nodules within the lesion. These observations led us to hypothesize that the PI3K pathway may play an important role in nodular formation.

Patients with port wine stains receive PDL (1.5-10ms, 11-12.5J/cm2) with a treatment range of approximately 10*10cm2

Pulsed dye laser (PDL, 595nm) is effective for vasodilatory diseases, especially for the superficial to middle layers of the dermis

In patients with nodular port wine stains, the exon mutation sequence and mutation frequency were compared between normal skin tissue (or blood), erythema and nodular tissue of the same patient.

Changes in the levels of VEGF, FGF, HGF, PDGF, TGF-beta and other factors in serum and skin tissues before and after laser treatment

Inclusion Criteria: Age 1 month - 70 years old, male or female; The patient and the family of the child agreed to participate in the experiment and signed an informed consent form; Clinical diagnosis of refractory port wine stains with thickened nodular or PDL resistance; .There is no bleeding, ulceration, infection, etc. affecting the visual field of laser surgery operation. Exclusion Criteria: Patients with severe infectious diseases; Heart disease patients; Epilepsy patient; Pregnant patient; Researchers believe that patients who are not suitable for this experiment