port wine stain


Summary: 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)

Top Publications

  1. Mehta M, Salas A, Fay A. Trigeminal dermatome distribution in patients with glaucoma and facial port wine stain. Dermatology. 2009;219:219-24 pubmed publisher
    The importance of glaucoma screening in patients with upper eyelid involvement of facial port wine stain (PWS) is well known...
  2. Sommer S, Seukeran D, Sheehan Dare R. Efficacy of pulsed dye laser treatment of port wine stain malformations of the lower limb. Br J Dermatol. 2003;149:770-5 pubmed
    ..5% are lower than frequently reported for other skin sites and adverse effects may be more frequent, patient satisfaction with treatment was generally high. Patients with PWS on the lower limb merit a trial of PDL treatment. ..
  3. Jasim Z, Woo W, Walsh M, Handley J. Multifocal basal cell carcinoma developing in a facial port wine stain treated with argon and pulsed dye laser: a possible role for previous radiotherapy. Dermatol Surg. 2004;30:1155-7 pubmed
    Basal cell carcinoma occurring in port wine stain is extremely rare but has been reported after radiotherapy for port wine stain...
  4. Wang H, Wang J, Jin H, Wen S, Jiang G. Flashlamp-pumped pulsed dye laser in treatment of port-wine stains. Chin Med Sci J. 2001;16:56-8 pubmed
    ..Pigmentary change (usually transient) occurred in 3.1% of patients. This study confirms the efficacy of the flashlamp-pumped pulsed dye laser in the treatment of port-wine stains in children and adults. ..
  5. Vázquez López F, Manjón Haces J, Vázquez López A, Perez Oliva N. The hand-held dermatoscope improves the clinical evaluation of port-wine stains. J Am Acad Dermatol. 2003;48:984-5 pubmed
  6. Seremet S, Benar E, Afsar F, Calli A, Ulusarac O. A case of acquired port wine stain: an association with repeated sunburn?. Int J Dermatol. 2016;55:e544-6 pubmed publisher
    Unlike congenital port wine stain (PWS), an acquired PWS is a rare vascular lesion that develops later in life...
  7. Piaserico S, Belloni Fortina A. Posttraumatic port-wine stain in a 4-year-old girl: Fegeler syndrome. Pediatr Dermatol. 2004;21:131-3 pubmed
    ..Including our patient, 25 instances of acquired port-wine stains have been reported in children. Ten (40%) of these were trauma related. The possible etiopathogenesis of acquired posttraumatic port-wine stains is discussed. ..
  8. Maari C, Frieden I. Klippel-Trénaunay syndrome: the importance of "geographic stains" in identifying lymphatic disease and risk of complications. J Am Acad Dermatol. 2004;51:391-8 pubmed
    ..Since these stains are present at birth, this clinical observation can help in identifying individuals with KTS at greatest risk for complications and in need of closer observation. ..
  9. Eerola I, Boon L, Watanabe S, Grynberg H, Mulliken J, Vikkula M. Locus for susceptibility for familial capillary malformation ('port-wine stain') maps to 5q. Eur J Hum Genet. 2002;10:375-80 pubmed
    ..22) between markers D5S1962 and D5S652 corresponding to 5q13-5q15. Interesting candidate genes implicated in vascular and neural development, such as MEF2C, RASA1, and THBS4, are in this locus. ..

More Information


  1. Lorenz S, Scherer K, Wimmershoff M, Landthaler M, Hohenleutner U. Variable pulse frequency-doubled Nd:YAG laser versus flashlamp-pumped pulsed dye laser in the treatment of port wine stains. Acta Derm Venereol. 2003;83:210-3 pubmed
    ..In port wine stains, the FPDL remains the therapy of choice because of the somewhat better results and a lower frequency of side effects, especially scarring. ..
  2. Svaasand L, Aguilar G, Viator J, Randeberg L, Kimel S, Nelson J. Increase of dermal blood volume fraction reduces the threshold for laser-induced purpura: implications for port wine stain laser treatment. Lasers Surg Med. 2004;34:182-8 pubmed
    The average success rate in achieving total blanching of port wine stain (PWS) lesions treated with laser-induced selective photothermolysis is below 25%, even after multiple treatments...
  3. Nortje C. General practitioner's radiology case 62. Sturge-Weber syndrome. SADJ. 2008;63:172 pubmed
  4. Mioc S, Mycek M. Selected laser-based therapies in otolaryngology. Otolaryngol Clin North Am. 2005;38:241-54 pubmed
    ..PDT has the potential to offer new, cost-effective treatment options, and possibly cure, to cancer patients who are not candidates for traditional surgery, chemotherapy, and radiotherapy. ..
  5. Bernstein E, Brown D. Efficacy of the 1.5 millisecond pulse-duration, 585 nm, pulsed-dye laser for treating port-wine stains. Lasers Surg Med. 2005;36:341-6 pubmed
    ..The 1.5-millisecond pulse-duration PDL is effective for treating PWSs and may offer improved efficacy over shorter pulse-duration lasers. ..
  6. Yung A, Sheehan Dare R. A comparative study of a 595-nm with a 585-nm pulsed dye laser in refractory port wine stains. Br J Dermatol. 2005;153:601-6 pubmed
    ..5 ms), test areas using 595-nm PDL (pulse width 1.5 ms and 6 ms) should be undertaken to ascertain if individual patients may benefit from the longer pulse width 595-nm PDL. ..
  7. Jung B, Kim C, Choi B, Kelly K, Nelson J. Use of erythema index imaging for systematic analysis of port wine stain skin response to laser therapy. Lasers Surg Med. 2005;37:186-91 pubmed
    Quantitative methods to assess port wine stain (PWS) skin response to laser therapy are needed to improve therapeutic outcome...
  8. Gerritsen M, Steijlen P, Brunner H, Rieu P. Cutis marmorata telangiectatica congenita: report of 18 cases. Br J Dermatol. 2000;142:366-9 pubmed
    ..All cases were sporadic. ..
  9. Hansen K, Kreiter C, Rosenbaum M, Whitaker D, Arpey C. Long-term psychological impact and perceived efficacy of pulsed-dye laser therapy for patients with port-wine stains. Dermatol Surg. 2003;29:49-55 pubmed
    ..A minority of patients was dissatisfied with treatment, and men were more likely to be dissatisfied. Additional long-term and prospective studies will be helpful in assessing the physical and psychosocial impact of PDL for PWS. ..
  10. Carrasco L, Pastor A, Fariña C, Martin L, Manzarbeitia F, Requena L. Acral arteriovenous tumor developed within a nevus flammeus in a patient with Sturge-Weber syndrome. Am J Dermatopathol. 2003;25:341-5 pubmed
    ..The development of acral arteriovenous tumor within the vascular malformation of a nevus flammeus in this patient with Sturge-Weber syndrome probably results from a vascular proliferation secondary to underlying arteriovenous shunts. ..
  11. Lopez Gutierrez J, Lapunzina P. Capillary malformation of the lower lip, lymphatic malformation of the face and neck, asymmetry and partial/generalized overgrowth (CLAPO): report of six cases of a new syndrome/association. Am J Med Genet A. 2008;146A:2583-8 pubmed publisher
    ..This constellation of findings has been observed in six unrelated patients, constituting a likely "new" syndrome/association of capillary-lymphatic-venous malformation and asymmetric overgrowth. ..
  12. Valdes F, Vadillo F, Martinez A. [Klippel-Trénaunay syndrome and Arnold-Chiari type I malformation]. Actas Dermosifiliogr. 2007;98:441-2 pubmed
  13. Sharma V, Khandpur S. Efficacy of pulsed dye laser in facial port-wine stains in Indian patients. Dermatol Surg. 2007;33:560-6 pubmed
    ..Seven patients developed hyperpigmentation while 1 patient showed hypopigmentation, but none had textural change or scarring. PDL offers significant cosmetic improvement in facial PWS in Indian patients. ..
  14. Ozluer S, Barlow R. Partial re-emergence of a port-wine stain following successful treatment with flashlamp-pumped dye laser. Clin Exp Dermatol. 2001;26:37-9 pubmed
    ..The latter would not be expected to respond to FPDL and may explain re-emergence of the lesion. ..
  15. Breugem C, Hennekam R, van Gemert M, van der Horst C. Are capillary malformations neurovenular or purely neural?. Plast Reconstr Surg. 2005;115:578-87 pubmed
  16. Lapidoth M, Ad El D, David M, Alcalay J, Azaria R. Basal cell carcinoma arising over facial port wine stain: a single-centre experience. J Eur Acad Dermatol Venereol. 2006;20:1066-9 pubmed
    A growing number of reports point to a possible connection between basal cell carcinoma (BCC) and port wine stain (PWS)...
  17. Low D. Invited discussion: on the efficacy of spot removers for port wine stains. Ann Plast Surg. 2008;60:431-2 pubmed publisher
  18. Syed S, Weibel L, Kennedy H, Harper J. A pilot study showing pulsed-dye laser treatment improves localized areas of chronic atopic dermatitis. Clin Exp Dermatol. 2008;33:243-8 pubmed publisher
    ..This may suggest that in AD dermal vasculature plays an important role or that PDL may have an effect on cutaneous immunological activation. ..
  19. Siewiera I, Wysocki M, Latkowski I. [Lasers in plastic surgery--vascular lasers]. Wiad Lek. 2007;60:178-84 pubmed
    ..In the paper we discuss the most common vascular lesions and available lasers used to treat them. Described indications and safety rules might be useful for many specialists for their patient therapy choice...
  20. Tennant L, Mulliken J, Perez Atayde A, Kozakewich H. Verrucous hemangioma revisited. Pediatr Dermatol. 2006;23:208-15 pubmed
    ..No firm conclusion seems possible as to whether verrucous hemangioma is a malformation or an indolent tumor, but clinical evidence favors the former category. ..
  21. Castori M, Rinaldi R, Angelo C, Zambruno G, Grammatico P, Happle R. Phacomatosis cesioflammea with unilateral lipohypoplasia. Am J Med Genet A. 2008;146A:492-5 pubmed publisher
    ..The patchy distribution of lipohypoplasia and its spatial relationship with vascular lesions strongly support the hypothesis of a postzygotic recombination event...
  22. Latkowski I, Wysocki M, Siewiera I. [Own clinical experience in treatment of port-wine stain with KTP 532 nm laser]. Wiad Lek. 2005;58:391-6 pubmed
    ..2. The treatment is especially effective in adults and in lesions localized to the face and neck area. 3. Port-wine stain localized on the trunk and extremities often appears to be KTP laser resistant. ..
  23. Zhao S, Gu Y, Xue P, Guo J, Shen T, Wang T, et al. Imaging port wine stains by fiber optical coherence tomography. J Biomed Opt. 2010;15:036020 pubmed publisher
    ..We demonstrate that OCT may be a useful tool for the noninvasive imaging of PWS. It may help determine the photosensitizer dose and laser parameters in photodynamic therapy for treating port wine stains. ..
  24. De Borgie C, Bossuyt P, van der Horst C, van Gemert M. Introduction of the flash-lamp pulsed-dye laser treatment of facial port-wine stains in childhood: A case of health care technology assessment. Lasers Surg Med. 2001;28:182-9 pubmed
    ..We believe that this nonadherence to the iterative HCTA model reflects the gradual way by which innovations find their way into clinical practice. ..
  25. Eerola I, Boon L, Mulliken J, Burrows P, Dompmartin A, Watanabe S, et al. Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am J Hum Genet. 2003;73:1240-9 pubmed
  26. Wharton S, Cole R. Basal cell carcinomata in port wine stains. Br J Plast Surg. 2001;54:156-8 pubmed
    The development of basal cell carcinoma (BCC) in a port wine stain is rare and the aetiology of these malignancies in this situation is the subject of some debate...
  27. Kimel S, Svaasand L, Hammer Wilson M, Nelson J. Influence of wavelength on response to laser photothermolysis of blood vessels: implications for port wine stain laser therapy. Lasers Surg Med. 2003;33:288-95 pubmed
    ..To ensure deposition of sufficient laser energy, irradiation at 585 nm, characterized by lower optical penetration depth in blood, may be preferable for PWS therapy. ..
  28. Verkruysse W, Choi B, Zhang J, Kim J, Nelson J. Thermal depth profiling of vascular lesions: automated regularization of reconstruction algorithms. Phys Med Biol. 2008;53:1463-74 pubmed publisher
    ..A second objective of this study was to depart from modeling port wine stain PWS, a vascular skin lesion frequently studied with PPTR, as strictly layered structures since this may ..
  29. Miller A, Pit ten Cate I, Watson H, Geronemus R. Stress and family satisfaction in parents of children with facial port-wine stains. Pediatr Dermatol. 1999;16:190-7 pubmed
    ..Health care providers should be prepared to screen for clinical levels of distress and to refer parents for psychological intervention when needed. ..
  30. Jia W, Aguilar G, Verkruysse W, Franco W, Nelson J. Improvement of port wine stain laser therapy by skin preheating prior to cryogen spray cooling: a numerical simulation. Lasers Surg Med. 2006;38:155-62 pubmed
    ..spray cooling (CSC) in conjunction with laser therapy has become the clinical standard for treatment of port wine stain (PWS) birthmarks, the current approach does not produce complete lesion blanching in the vast majority of ..
  31. Gómez Díez S, Losada A, Coto P, Soler T, Perez Oliva N. [Acquired flat angiomas in the form of a port-wine stain]. Actas Dermosifiliogr. 2006;97:62-4 pubmed
    ..Histopathology showed ectatic vessels in the papillary and reticular dermis, with no proliferation of endothelial cells. ..
  32. Sharan S, Swamy B, Taranath D, Jamieson R, Yu T, Wargon O, et al. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome. J AAPOS. 2009;13:374-8 pubmed publisher
    ..The aim of this study was to review clinical features and management of ocular complications of SWS and assess the effects of dermatological laser treatment on the incidence of glaucoma or ocular hypertension...
  33. Kono T, Ercocen A, Chan H, Kikuchi Y, Hori K, Uezono S, et al. Treatment of phacomatosis pigmentovascularis: a combined multiple laser approach. Dermatol Surg. 2003;29:642-6 pubmed
    ..Phacomatosis pigmentovascularis (PPV) consists of a capillary malformation with a variety of melanocytic lesions, which involve various regions of the body and are difficult to treat with conventional therapeutic tools...
  34. Childers M, Franco W, Nelson J, Aguilar G. Laser surgery of port wine stains using local vacuum pressure: changes in skin morphology and optical properties (Part I). Lasers Surg Med. 2007;39:108-17 pubmed
    In a recent case study, the use of a suction device to aid in port wine stain (PWS) laser treatments showed favorable results...
  35. Liu H, Dang Y, Chai X, Wang Z, Ma L, Ren Q. Treatment of port-wine stains with the 595-nm pulsed dye laser: a pilot study in Chinese patients. Clin Exp Dermatol. 2007;32:646-9 pubmed
    ..Prominent hyperpigmentation occurred in only 11 cases (6.0%) with poor improvement, and resolved within 6 months. The 595-nm PDL appears to be safe, effective and well tolerated in the treatment of PWS in Chinese patients. ..
  36. Azizkhan R. Laser surgery: new applications for pediatric skin and airway lesions. Curr Opin Pediatr. 2003;15:243-7 pubmed
  37. Woo W, Handley J. Does fluence matter in the laser treatment of port-wine stains?. Clin Exp Dermatol. 2003;28:556-7 pubmed
  38. Madlom M, Hoggard N, Griffiths P, Chan J. Facial naevus flammeus with choroidal haemangioma and without intracranial involvement. Dev Med Child Neurol. 2003;45:139 pubmed
  39. Reynolds N, Exley J, Hills S, Falder S, Duff C, Kenealy J. The role of the Lumina intense pulsed light system in the treatment of port wine stains--a case controlled study. Br J Plast Surg. 2005;58:968-80 pubmed
    ..The results showed that eight of the 12 subjects had some degree of fading of their port wine stain as measured on a percentage scoring system...
  40. Kaseka M, Bitton J, Decarie J, Major P. Predictive Factors for Epilepsy in Pediatric Patients With Sturge-Weber Syndrome. Pediatr Neurol. 2016;64:52-58 pubmed publisher
    ..Bilateral facial port-wine stain and cerebral developmental venous anomalies increase the risk of epilepsy in Sturge-Weber syndrome patients. Because they can be detected at birth, they might guide preventive management and follow-up. ..
  41. Al Dhalimi M, Al Janabi M. Split lesion randomized comparative study between long pulsed Nd:YAG laser 532 and 1,064?nm in treatment of facial port-wine stain. Lasers Surg Med. 2016;48:852-858 pubmed publisher
    ..Lasers Surg. Med. 48:852-858, 2016. © 2016 Wiley Periodicals, Inc. ..
  42. Rikihisa N, Tominaga M, Watanabe S, Mitsukawa N, Saito Y, Sakai H. Intravenous injection of artificial red cells and subsequent dye laser irradiation causes deep vessel impairment in an animal model of port-wine stain. Lasers Med Sci. 2018;33:1287-1293 pubmed publisher
    ..In Hb-Vs and RBC mixture, heat transfer to endothelial cells from absorbers/heater may increase. Hb-Vs function as photosensitizers to destroy deep vessels within a restricted distance that the photon can reach. ..
  43. Linwood J. On the mark. Interview by Dina Leifer. Nurs Stand. 2004;18:17 pubmed
  44. van Aalst J, Bhuller A, Sadove A. Pediatric vascular lesions. J Craniofac Surg. 2003;14:566-83 pubmed
    ..Understanding the clinical course of pediatric vascular lesions allows the surgeon to find an appropriate balance between watchful observation, providing reassurance when appropriate, and intervention when needed. ..
  45. Huang P, Chang C. Cryogen spray cooling in conjunction with pulse dye laser treatment of port wine stains of the head and neck. Chang Gung Med J. 2001;24:469-75 pubmed
    ..of cooling remains localized in the normal overlying epidermis, while leaving the temperature of deeper port wine stain (PWS) blood vessels unchanged...
  46. Chugani H, Juhasz C, Behen M, Ondersma R, Muzik O. Autism with facial port-wine stain: a new syndrome?. Pediatr Neurol. 2007;37:192-9 pubmed
    ..Unilateral facial port-wine stain and autism with no intracranial angioma on conventional imaging may represent a rare clinical entity distinct from both infantile autism and previously described variants of Sturge-Weber syndrome. ..
  47. Lanigan S. Reduction of pain in the treatment of vascular lesions with a pulsed dye laser and pneumatic skin flattening. Lasers Med Sci. 2009;24:617-20 pubmed publisher
    ..It was concluded that the PSF technology significantly reduced pain in the treatment of vascular lesions with a pulsed dye laser without affecting efficacy. ..
  48. Brodsky M. Circumpapillary choroidal "haemorrhoid" in Klippel-Trenauney-Weber syndrome. Br J Ophthalmol. 2007;91:394 pubmed
  49. Namba Y, Mae O, Ao M. The treatment of port wine stains with a dye laser: a study of 644 patients. Scand J Plast Reconstr Surg Hand Surg. 2001;35:197-202 pubmed
    ..Patients who had been given previous treatments such as argon laser required about two more laser treatments than those who had not, but there were no clear differences in the efficacy of dye laser treatment between the two groups. ..
  50. Jia W, Sun V, Tran N, Choi B, Liu S, Mihm M, et al. Long-term blood vessel removal with combined laser and topical rapamycin antiangiogenic therapy: implications for effective port wine stain treatment. Lasers Surg Med. 2010;42:105-12 pubmed publisher
    Complete blanching of port wine stain (PWS) birthmarks after laser therapy is rarely achieved for most patients...
  51. Tomson N, Lim S, Abdullah A, Lanigan S. The treatment of port-wine stains with the pulsed-dye laser at 2-week and 6-week intervals: a comparative study. Br J Dermatol. 2006;154:676-9 pubmed
    ..It also has the potential to reduce the total duration of a course of treatment significantly. ..
  52. Woo S, Ahn H, Kim S, Kye Y. Treatment of vascular skin lesions with the variable-pulse 595 nm pulsed dye laser. Dermatol Surg. 2006;32:41-8 pubmed
    ..The variable-pulse 595 nm PDL was found to be effective for treating several vascular skin lesions in dark-skinned patients. However, there were differences in treatment outcome owing to disease, age, and the location of the lesions. ..
  53. Silapunt S, Goldberg L, Thurber M, Friedman P. Basal cell carcinoma arising in a port-wine stain. Dermatol Surg. 2004;30:1241-5 pubmed
    ..The hematoma was drained and there was no further bleeding or evidence of recurrence of the tumor after 12 months. Basal cell carcinoma should be included in the differential diagnosis of a skin lesion occurring in a port-wine stain. ..