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Open Access Macedonian Journal of... Jan 2019To assess the efficacy in the treatment of port wine stain in the head and neck by using (Vbeam perfecta®).
AIM
To assess the efficacy in the treatment of port wine stain in the head and neck by using (Vbeam perfecta®).
METHODS
Forty-two port wine stain patients were recruited at the National Hospital of Dermatology and Venereology, Hanoi, Vietnam.
RESULTS
We reported an excellent response (43.8%) (76%-100% lightening), a good response (18.8%) (51%-75% lightening), fair improvement (18.8%) (26%-50% lightening), and no response (18.8%) (0%-25% lightening).
CONCLUSION
In conclusion, pulsed dye laser is an excellent technique to remove port wine stains on the face and neck.
PubMed: 30745960
DOI: 10.3889/oamjms.2019.052 -
Skin Health and Disease Feb 2024Port-wine stains (PWS) are capillary vascular anomalies that are often treated with pulsed-dye laser (PDL). Revascularization limits persistent clearance; however, the... (Review)
Review
Port-wine stains (PWS) are capillary vascular anomalies that are often treated with pulsed-dye laser (PDL). Revascularization limits persistent clearance; however, the anti-angiogenic effects of sirolimus (SIRO) may inhibit revascularization. This review aims to determine differences in PWS outcomes when treated with PDL monotherapy or in combination with SIRO. A systematic review was conducted using PubMed, Cochrane, and Embase databases. The following search terms were used: 'port wine stain PDL SIRO', 'port wine stain PDL', and 'port wine stain PDL and topical treatment' with (MeSH) and (Title/Abstract) limits. The search was limited to the English language and human-subject studies conducted between 1 January 2000 and 1 June 2023. Inclusion criteria included studies evaluating SIRO as an adjunct to PDL in patients with PWS. Data extraction and quality assessment were performed by two independent reviewers. A total of nine studies met the inclusion criteria, which included randomized controlled trials (3), case series (2), case reports (3), and a prospective intrapatient study (1), which represented a total of 58 patients. Five studies showed improvement of a measured post-treatment PDL parameter including shortening treatment time and less frequent dosing. A subset of studies (4/9) which did not demonstrate significant clinical improvements exhibited significant photographic evidence of improvement. Heterogeneity among the studies highlights the need for further research and standardization. While adjunctive SIRO shows promise, larger studies and comprehensive evaluation methods are required to establish conclusive safety and efficacy guidelines to shape clinical decision-making.
PubMed: 38312256
DOI: 10.1002/ski2.333 -
Cureus Aug 2022Klippel-Trenaunay Syndrome (KTS) is a rare congenital capillary/venous malformation (CVM) disorder associated with deep soft-tissue swelling. A seven-year-old Caucasian...
Klippel-Trenaunay Syndrome (KTS) is a rare congenital capillary/venous malformation (CVM) disorder associated with deep soft-tissue swelling. A seven-year-old Caucasian male with a history of Klippel-Trenaunay Syndrome presented to the dermatology clinic for evaluation and treatment of mildly edematous right lower extremity and varicose veins with multiple, small port-wine stains and nodules. Typically, cases of KTS report large port-wine stains, where a multidisciplinary approach is the mainstay for management. However, this case involves a clinical presentation of mild KTS with atypical, port-wine stains on the affected limb.
PubMed: 36158438
DOI: 10.7759/cureus.28303 -
Cureus Jan 2021Benign vascular neoplasms are common clinical problems encountered in the practice of primary care. Pyogenic granulomas are one of the most common benign vascular...
Benign vascular neoplasms are common clinical problems encountered in the practice of primary care. Pyogenic granulomas are one of the most common benign vascular lesions in young adults. Although the physiopathological mechanism for the development of this condition is still not well understood, it has been commonly associated with several triggers such as treatment with retinoids, biological agents, invasive cutaneous therapies and trauma. The development of pyogenic granulomas on sites of vascular malformations like port wine stains has been described in the literature to occur rarely. Most of these types of cases have been studied to occur in the setting of pregnancy and after cryotherapy or pulsated laser therapy. The aim of this article is to present the case of a 21-year-old man with a recent appearance of a pyogenic granuloma within an underlying port wine stain in the posterior cervical region without any history of triggers or risk factors. Excision of the vascular lesion was done, and histopathological report confirmed the diagnosis. The objective of this manuscript is to discuss the possible mechanisms involved in the development of this uncommon presentation and to summarize the current literature related to this clinical scenario.
PubMed: 33564515
DOI: 10.7759/cureus.12509 -
Cureus Oct 2022The Klippel-Trénaunay syndrome (KTS) is a rare form of a birth disorder that includes capillary malformation, hypertrophy of bones and soft tissues, and tortuous...
The Klippel-Trénaunay syndrome (KTS) is a rare form of a birth disorder that includes capillary malformation, hypertrophy of bones and soft tissues, and tortuous varicosities, as well as hypertrophy of the capillaries resulting in hemangiomas and port wine discoloration. KTS is also known as angio-osteohypertrophy syndrome and dysplastic angiopathy. In this case report, we describe the case of a 13-year-old female with multiple superficial varicosities on the medial aspect of her left leg since birth. Computed tomography angiogram assessed and identified abnormal venous drainage in the lower limb. Klippel-Trénaunay-Weber syndrome (KTWS) differs from KTS in that KTWS involves arteriovenous malformations.
PubMed: 36381931
DOI: 10.7759/cureus.30128 -
Actas Dermo-sifiliograficas Sep 2006Laser treatment of vascular skin lesions is rapidly improving. Port wine stain (PWS) is one of the first lesions successfully treated with laser. The pulsed dye laser... (Review)
Review
Laser treatment of vascular skin lesions is rapidly improving. Port wine stain (PWS) is one of the first lesions successfully treated with laser. The pulsed dye laser (PDL) has been developed on the concept of selective photothermolysis and has revolutionized PWS treatment. Even though the response is variable, substantial blanching can be obtained after several sessions. Greater selectivity and better response have been achieved by modifying PDL parameters such as longer pulse width, longer wavelength, or higher energy fluencies with cooling skin devices. It is known that thick, deep or raised lesions present poor response to conventional treatments. The aim of this study is to review the different lasers and other light sources which are developing for the treatment of PWS and other vascular lesions.
Topics: Humans; Laser Therapy; Port-Wine Stain
PubMed: 16978541
DOI: 10.1016/s0001-7310(06)73434-2 -
The Journal of Investigative Dermatology Jun 2024Capillary malformations (CM) (port-wine stains) are congenital skin lesions that are characterized by dilated capillaries and postcapillary venules. CMs are caused by...
Capillary malformations (CM) (port-wine stains) are congenital skin lesions that are characterized by dilated capillaries and postcapillary venules. CMs are caused by altered functioning of the vascular endothelium. Somatic genetic mutations have predominantly been identified in the endothelial cells of CMs, providing an opportunity for the development of targeted therapies. However, there is currently limited in-depth mechanistic insight into the pathophysiology and a lack of preclinical research approaches. In a monocenter exploratory study of 17 adult patients with CMs, we found somatic sequence variants in the GNAQ (p.R183Q, p.R183G, or p.Q209R) or GNA11 (p.R183C) genes. We applied an endothelial-selective cell isolation protocol to culture primary endothelial cells from skin biopsies from these patients. We successfully expanded patient-derived cells in culture in 3 of the 17 cases while maintaining endothelial specificity as demonstrated by vascular endothelial-cadherin immunostainings. In addition, we tested the angiogenic capacity of endothelial cells from a patient with a GNAQ (p.R183G) sequence substitution. These proof-of-principle results reveal that primary cells isolated from CMs may represent a functional research model to investigate the role of endothelial somatic mutations in the etiology of CMs, but improved isolation and culture methodologies are urgently needed to advance the field.
Topics: Humans; GTP-Binding Protein alpha Subunits, Gq-G11; Endothelial Cells; Capillaries; Male; Female; Mutation; Adult; Port-Wine Stain; Cells, Cultured; Skin; Vascular Malformations; GTP-Binding Protein alpha Subunits; Middle Aged; Biopsy; Young Adult
PubMed: 38013159
DOI: 10.1016/j.jid.2023.10.033 -
Sao Paulo Medical Journal = Revista... 2022Congenital vascular anomalies and hemangiomas (CVAH) such as infantile hemangiomas, port-wine stains and brain arteriovenous malformations (AVMs) impair patients' lives... (Review)
Review
BACKGROUND
Congenital vascular anomalies and hemangiomas (CVAH) such as infantile hemangiomas, port-wine stains and brain arteriovenous malformations (AVMs) impair patients' lives and may require treatment if complications occur. However, a great variety of treatments for those conditions exist and the best interventions remain under discussion.
OBJECTIVE
To summarize Cochrane systematic review (SR) evidence on treatments for CVAH.
DESIGN AND SETTING
Review of SRs conducted in the Division of Vascular and Endovascular Surgery of Universidade Federal de São Paulo, Brazil.
METHODS
A broad search was conducted on March 9, 2021, in the Cochrane Database of Systematic Reviews to retrieve any Cochrane SRs that assessed treatments for CVAH. The key characteristics and results of all SRs included were summarized and discussed.
RESULTS
A total of three SRs fulfilled the inclusion criteria and were presented as a qualitative synthesis. One SR reported a significant clinical reduction of skin redness by at least 20%, with more pain, among 103 participants with port-wine stains. One SR reported that propranolol improved the likelihood of clearance 13 to 16-fold among 312 children with hemangiomas. One SR reported that the relative risk of death or dependence was 2.53 times greater in the intervention arm than with conservative management, among 218 participants with brain AVMs.
CONCLUSION
Cochrane reviews suggest that treatment of port-wine stains with pulsed-dye laser improves redness; propranolol remains the best option for infantile hemangiomas; and conservative management seems to be superior to surgical intervention for treating brain AVMs.
Topics: Arteriovenous Malformations; Brazil; Child; Hemangioma; Humans; Port-Wine Stain; Systematic Reviews as Topic
PubMed: 35293939
DOI: 10.1590/1516-3180.2021.0374.R2.15092021 -
Hereditas Jul 2020Capillary malformation-arteriovenous malformations (CM-AVMs) caused by a RASA-1 or EPHB4 mutation are characterized as hereditary sporadic or multifocal capillary...
Capillary malformation-arteriovenous malformations (CM-AVMs) caused by a RASA-1 or EPHB4 mutation are characterized as hereditary sporadic or multifocal capillary malformations (CMs), associated with potential fast-flow vascular anomalies underlying erythema lesions. Because of the similar phenotype, CM-AVMs should be considered in the differential diagnosis of isolated CMs as well as other disorders with an erythema phenotype, such as hereditary hemorrhagic telangiectasia (HHT).Herein, we report a male patient with facial erythema. Red lesions were located in the V1 region of his left face, the V2 and V3 regions on his right side, and the nasal back. The patient was initially thought to have PWSs because of the unilateral and segmental distribution of his red facial lesions. In contrast to a previous diagnosis, we diagnosed the child with capillary malformation-arteriovenous malformation type 2 (CM-AVM2) based on a family history of erythema, the results of physical examination and ultrasound raising potential fast-flow lesions, and a genetic study revealing a germline EPHB4 mutation. This study emphasizes the importance of differential diagnosis for PWS and CM-AVM. A single clinical diagnosis can be limited, and molecular diagnosis is recommended to provide more information for the evaluation of the potential risk of fast-flow lesions underlying erythema lesions if necessary.
Topics: Arteriovenous Malformations; Capillaries; Child, Preschool; DNA Mutational Analysis; Diagnosis, Differential; Erythema; Face; High-Throughput Nucleotide Sequencing; Humans; Male; Mutation; Phenotype; Port-Wine Stain; Receptor, EphB4
PubMed: 32635943
DOI: 10.1186/s41065-020-00143-z -
Photodiagnosis and Photodynamic Therapy 2009Pulsed dye laser (PDL) is a commonly utilized treatment for port wine stain birthmarks (PWS) in the United States; however, results are variable and few patients achieve... (Clinical Trial)
Clinical Trial
BACKGROUND
Pulsed dye laser (PDL) is a commonly utilized treatment for port wine stain birthmarks (PWS) in the United States; however, results are variable and few patients achieve complete removal. Photodynamic therapy (PDT) is commonly used in China, but treatment associated photosensitivity lasts several weeks and scarring may occur. We propose an alternative treatment option, combined PDT+PDL and performed a proof-of-concept preliminary clinical trial.
METHODS
Subjects with non-facial PWS were studied. Each subject had four test sites: control, PDL alone, PDT alone (benzoporphyrin derivative monoacid ring A photosensitizer with 576 nm light), and PDT+PDL. Radiant exposure time for PDT was increased in increments of 15 J/cm(2). Authors evaluated photographs and chromametric measurements before and 12 weeks post-treatment.
RESULTS
No serious adverse events were reported; epidermal changes were mild and self-limited. No clinical blanching was noted in control or PDT-alone sites. At PDT radiant exposures of 15 and 30 J/cm(2), equivalent purpura and blanching was observed at PDL and PDT+PDL sites. At PDT radiant exposures over 30 J/cm(2), greater purpura was noted at PDT+PDL sites as compared to PDL alone. Starting at 75 J/cm(2), improved blanching was noted at PDT+PDL sites.
CONCLUSIONS
Preliminary results indicate that PDT+PDL is safe and may offer improved PWS treatment efficacy. Additional studies are warranted.
Topics: Adult; Female; Humans; Lasers, Dye; Male; Middle Aged; Photochemotherapy; Photosensitizing Agents; Porphyrins; Port-Wine Stain; Treatment Outcome; Verteporfin; Young Adult
PubMed: 19932451
DOI: 10.1016/j.pdpdt.2009.10.002