Port Wine Stain
Conditions
Keywords
port wine stain, PI3K, vascular malformation,PDL
Brief summary
1. 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. 2. 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. 3. 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.
Interventions
RADIATIONPulsed dye laser (PDL)
Pulsed dye laser (PDL, 595nm) is effective for vasodilatory diseases, especially for the superficial to middle layers of the dermis
Sponsors
Air Force General Hospital of the PLA
First Hospital of China Medical University
Chinese Academy of Medical Sciences
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Study design
Allocation
RANDOMIZED
Intervention model
SINGLE_GROUP
Primary purpose
BASIC_SCIENCE
Masking
DOUBLE (Subject, Investigator)
Eligibility
Sex/Gender
ALL
Age
1 Months to 70 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Exon mutation sequences and mutation frequencies | Baseline (before treatment) | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Changes in the levels of cytokines in serum and skin tissues | baseline (before treatment) and 1, 3, 7 days after treatment | Changes in the levels of VEGF, FGF, HGF, PDGF, TGF-beta and other factors in serum and skin tissues before and after laser treatment |
Outcome results
None listed