Port-Wine Stain (Nevus Flammeus)

A vascular malformation of developmental origin characterized pathologically by ectasia of superficial dermal capillaries, and clinically by persistent macular erythema. In the past, port wine stains have frequently been termed capillary hemangiomas, which they are not; unfortunately this confusing practice persists: HEMANGIOMA, CAPILLARY is neoplastic, a port-wine stain is non-neoplastic. Port-wine stains vary in color from fairly pale pink to deep red or purple and in size from a few millimeters to many centimeters in diameter. The face is the most frequently affected site and they are most often unilateral. (From Rook et al., Textbook of Dermatology, 5th ed, p483)

Also Known As:

Networked: 893 relevant articles (145 outcomes, 87 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hemangioma (Angioma)
2.	Telangiectasis (Telangiectasia)
3.	Cicatrix (Scar)
4.	Pain (Aches)
5.	Vascular Malformations

Experts

1.	Nelson, J Stuart: 42 articles (12/2019 - 01/2002)
2.	Gu, Ying: 25 articles (02/2021 - 06/2007)
3.	Choi, Bernard: 21 articles (10/2017 - 06/2003)
4.	Lin, Xiaoxi: 18 articles (03/2022 - 03/2015)
5.	Ma, Gang: 17 articles (03/2022 - 10/2011)
6.	Yu, Wenxin: 17 articles (03/2022 - 03/2015)
7.	Kelly, Kristen M: 17 articles (10/2017 - 01/2002)
8.	Wang, Ying: 16 articles (02/2021 - 06/2008)
9.	Chen, Bin: 13 articles (04/2022 - 01/2015)
10.	Li, Dong: 13 articles (04/2022 - 01/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Port-Wine Stain:

1.	1-phenyl-3,3-dimethyltriazene (PDT)IBA 07/17/2023 - "This study intends to observe the influential factors for the efficacy of HMME-PDT in the treatment of port-wine stains (PWS). " 07/01/2022 - "The Relationship Between the Effectiveness of HMME-PDT and the Dermoscopic Features of Port-wine Stains in Chinese Pediatric Patients: A Retrospective Study." 03/01/2020 - "Efficacy and related factors analysis in HMME-PDT in the treatment of port wine stains." 03/01/2019 - "Application of different noninvasive diagnostic techniques used in HMME-PDT in the treatment of port wine stains." 11/01/2011 - "[The change of reflection spectra and fluorescence spectra of port wine stains during PDT]."
2.	hematoporphyrin monomethyl etherIBA 12/01/2020 - "Correlation between the hemoporfin-mediated photodynamic treatment response and the dermoscopy vascular pattern in patients with a port-wine stain: a prospective study." 12/01/2011 - "To investigate the safety, tolerability and pharmacokinetics of intravenous hemoporfin, a novel photosensitive drug for the treatment of port-wine stain (PWS), in healthy Chinese volunteers following single-dose administration. " 12/01/2021 - "Hemoporfin photodynamic therapy was well tolerated and effective in paediatric Chinese patients with port-wine stains. " 12/01/2021 - "Although hemoporfin photodynamic therapy is a promising treatment approach for port-wine stains, its efficacy in children has not been sufficiently assessed. " 07/17/2023 - "Influential Factors in the Efficacy of Hemoporfin-Mediated Photodynamic Therapy for Port-wine Stains."
3.	ArgonIBA 04/01/1985 - "Argon laser therapy appears to be most effective for purple port wine stains on the faces of adults and telangiectatic lesions involving the nose and forehead. " 07/01/1990 - "International standard of argon laser therapy of port wine stains are 60 to 70% good results in adult patients afflicted with red and purple lesions. " 09/01/1986 - "Although the blue-green argon laser light has been used successfully to treat port-wine stains (PWSs) for many years, it produces substantial epidermal damage. " 01/01/1991 - "Argon laser surgery is an effective treatment for ectasias and congenital port-wine stains; however, its use in children under the age of 13 is controversial. " 11/01/1985 - "Argon laser therapy is effective for removing port-wine stains and for reducing cutaneous vascular and pigmented lesions. "
4.	Hemoglobins (Hemoglobin)IBA 10/01/2015 - "Previous studies suggest that IPL can be more effective in treatment of port wine stain by utilizing multiple wavelengths to selectively target different peaks in oxy- and deoxy-hemoglobin. " 07/01/2004 - "The treatment of cutaneous vascular lesions (port wine stains etc.) using lasers has been guided by theories based on the "cold" or room-temperature optical properties of the hemoglobin target chromophore. " 03/01/2017 - "Based on the principle of treating port-wine stains with a dye laser, intravenous injection of artificial red cells (hemoglobin vesicles) immediately before laser treatment might improve the clinical outcome of the therapy. " 01/01/2023 - "Artificial red blood cells [i.e., hemoglobin vesicles (HbVs)] can be used as photosensitizers in pulsed-dye laser (PDL) treatment for port wine stains in animal models. " 05/01/2021 - "hemoglobin [Hb] vesicles [Hb-Vs]) function effectively as photosensitizers in flashlamp-pumped pulsed-dye laser (PDL) treatment for port-wine stains in animal models. "
5.	Melanins (Melanin)IBA 01/01/2022 - "Competitive epidermal melanin absorption decreases the light energy reaching dysplastic vessels in a port-wine stain (PWS), preventing the vessel from achieving the temperature of the desired clinical result. " 01/01/1996 - "The object of this study is to characterize the effects of epidermal melanin in brown skin on selective vessel damage by copper vapor laser radiation in port wine stain (PWS). " 08/01/2017 - "Port-wine stains (PWSs) usually respond poorly to pulsed dye laser treatment because of the shallow penetration and light absorption of melanin in the epidermis. " 01/01/2001 - "Estimated values of heat removed from the epidermis suggest that a cryogen spurt as long as 200 milliseconds is required to counteract heat generated by high laser fluences (e.g., in treatment of port wine stains) in patients with high concentration of epidermal melanin. " 05/01/1993 - "The heat deposited by the laser pulse in the superficial epidermis causes an immediate temperature jump, and the heat deposited in basal epidermal melanin and deep port wine stains diffuses to the surface before detection. "
6.	AcidsIBA 08/01/2012 - "Topical application of 5-aminolevulinic acid followed by 595-nm pulsed dye laser irradiation for the treatment of recalcitrant port-wine stains: a primary study." 08/01/2012 - "To evaluate the effectiveness and safety of topical application of 5-aminolevulinic acid (ALA) followed by pulsed dye laser (PDL) irradiation for the treatment of recalcitrant port-wine stain (PWS). " 07/01/2010 - "Photodynamic therapy with intradermal administration of 5-aminolevulinic acid for port-wine stains." 07/01/2010 - "To investigate whether intradermal administration of 5-aminolevulinic acid (5-ALA) could achieve a sufficient amount of protoporphyrin IX (PpIX) to induce photochemical reaction in chicken comb, the animal model of port wine stains (PWSs). " 04/01/2005 - "The efficacy of PDT using systemic 5-aminolaevulinic acid (5-ALA) as the photosensitiser and pulsed dye laser (PDL) as the light source in port wine stains (PWS) is unknown. "
7.	Indocyanine Green (Cardio-Green)FDA LinkGeneric 11/01/2018 - "Photothermal treatment of port-wine stains using erythrocyte-derived particles doped with indocyanine green: a theoretical study." 08/01/2012 - "Indocyanine green-augmented diode laser treatment of port-wine stains: clinical and histological evidence for a new treatment option from a randomized controlled trial."
8.	Latanoprost (Xalatan)FDA Link 09/01/2003 - "Latanoprost in port wine stain related paediatric glaucoma." 09/01/2003 - "To evaluate the efficacy of latanoprost (Xalatan) as adjunctive therapy in port wine stain related paediatric glaucoma. " 09/01/2003 - "A trial of latanoprost as adjunctive therapy in patients with port wine stain related glaucoma may temporise the need for surgery; with 50% of patients being controlled at 1 year follow up. "
9.	Coloring Agents (Dyes)IBA 03/01/2021 - "Heterogeneity of stain color saturation, warmth to touch, peripheral pallor, and overgrowth/soft tissue swelling help distinguish HFVS from port wine stains. " 06/01/2018 - "Acquired Port-Wine Stain: Not a simple stain!" 12/01/2003 - "Capillary malformation (CM), or "port-wine stain," is a common cutaneous vascular anomaly that initially appears as a red macular stain that darkens over years. " 04/09/1998 - "The outcome measure was lightening of the port-wine stain (reduction in the difference in color between the skin with the stain and contralateral healthy skin) as measured with a colorimeter after an average of five treatments (range, three to seven) of the entire lesion. " 02/01/2004 - "Evaluating the efficacy of treatment of resistant port-wine stains with variable-pulse 595-nm pulsed dye and 532-nm Nd:YAG lasers."
10.	CollagenIBA 12/01/2003 - "Because of its low fluence and its shorter pulse duration, the 585 nm collagen remodelling pulsed dye laser has limited efficacy for the treatment of port wine stains. " 06/01/2003 - "Increasing radiant exposure offers a means to increase treatment efficacy during laser-mediated treatment of vascular lesions, such as port-wine stains; however, excessive radiant exposure decreases selective vascular injury due to increased heat generation within the epidermis and collateral damage to perivascular collagen. "

Therapies and Procedures

1.	Dye Lasers 12/01/2012 - "Pulsed dye laser (PDL) is the most effective treatment for port wine stain (PWS) birthmarks. " 01/01/1998 - "Port wine stains can be treated successfully with the dye laser in most cases. " 01/01/2021 - "Although pulsed-dye laser (PDL) 595 nm is known as the gold standard for treatment of port-wine stains (PWS), complete clearance of lesions occurs in a minority of cases. " 08/01/2014 - "Pulsed dye laser is effective for treating port-wine stains, although the optimal timing is controversial. " 04/01/2012 - "Pulsed dye laser (PDL) is the gold standard for treatment of port-wine stain (PWS) birthmarks but multiple treatments are required and complete resolution is often not achieved. "
2.	Lasers (Laser) 01/01/1999 - "The efficacy of laser treatment of port wine stains (PWS) has been shown to be highly dependent on patient-specific vasculature. " 08/01/2012 - "Complete clearance of port-wine stains (PWS) is difficult to achieve, mainly because of the resistance of small blood vessels to laser irradiation. " 11/01/1996 - "These data indicate that the therapeutic outcome of port-wine stains can be improved by using the lesional vessel parameters to select the optimal laser wavelength, pulse duration, and dose." 04/01/1991 - "The choice of suitable laser parameters is important for effective treatment of port-wine stains. " 10/01/1987 - "This study has shown that laser treatment can offer a moderately effective treatment for port-wine stains. "
3.	Photochemotherapy (Photodynamic Therapy) 01/01/2023 - "Efficacy of photodynamic therapy in the treatment of port wine stains: A systematic review and meta-analysis." 03/01/2023 - "General anaesthesia for photodynamic therapy of port-wine stain in children: A retrospective study." 03/01/2022 - "Histological Characteristics of Port-Wine Stains with Complete Regression After Photodynamic Therapy Treatment: A 7-Year Follow-Up." 12/01/2021 - "Recurrence of Port-Wine Stain After Photodynamic Therapy: A Case Report with a 22-Year Follow-Up." 02/01/2021 - "Monitoring perfusion and oxygen saturation in port-wine stains during vascular targeted photodynamic therapy."
4.	Therapeutics 11/01/1987 - "The worst response to the therapy was seen in pink port wine stains in children and juvenile patients (20% good results), in contrast to red and purple lesions in adult patients (80% excellent and good results). " 06/01/1999 - "We investigated whether a noncoherent intense pulsed light source (IPLS) would be effective in therapy of port-wine stains (PWSs). " 01/01/1997 - "The new intense pulsed light source Photoderm VL seems to be a promising treatment alternative for the therapy of otherwise nonresponding port-wine stains due to its special technical features." 01/01/2003 - "To study ocular complication of photoDerm VL therapy for facial port-wine stain. " 10/12/2022 - "Pathogenesis of Port-Wine Stains: Directions for Future Therapies."
5.	Laser Therapy (Surgery, Laser) 12/27/2022 - "Laser therapy has become the golden standard of port wine stain (PWS), but complete clearance of resistant PWSs is still difficult. " 09/08/2019 - "Site-specific pharmaco-laser therapy: A novel treatment modality for refractory port wine stains." 09/08/2019 - "Despite extensive efforts to optimize laser therapy, i.e., the current gold standard treatment, a majority of port wine stain (PWS) patients responds suboptimally to laser therapy. " 11/01/2008 - "The objective of this study was to improve port wine stain (PWS) therapeutic outcome in response to laser therapy. " 04/01/2022 - "Triple pass laser therapy for recalcitrant facial port wine stains."