eMedicine Specialties > Dermatology > Diseases of the Vessels

Capillary Malformation: Treatment & Medication

Author: Richard J Antaya, MD, Director of Pediatric Dermatology, Associate Professor, Departments of Dermatology and Pediatrics, Yale University
Contributor Information and Disclosures

Updated: Sep 25, 2009

Treatment

Medical Care

  • Affected areas can be tattooed with a skin-colored pigment; however, this is not routinely performed.
  • Use of a cosmetic cover-up is an alternative. Opaque makeup, sold commercially without a prescription under brand names like Dermablend and Covermark, disguises capillary malformations but does not treat them.
  • At least one report describes partial clearing of a capillary malformation on the arm of a 59-year-old woman after the use of topical imiquimod under plastic wrap occlusion 5 times per week for 4 weeks. The authors attribute the response to antiangiogenic properties of imiquimod through the induction of tissue inhibitor of matrix metalloproteinase-1, an inhibitor of vascular tumor growth.26

Surgical Care

  • Flashlamp-pumped pulsed-dye laser (PDL) surgery is the treatment of choice for capillary malformations. It uses selective photothermolysis with ultrashort pulses of monochromatic yellow light (585-600 nm), which are preferentially absorbed by oxyhemoglobin in the abnormally dilated superficial dermal blood vessels. Flashlamp-pumped PDL causes selective destruction of these superficial target blood vessels, inducing intravascular coagulation and rupture of some smaller vessels, which later become absorbed and replaced by collagen. The ultrashort laser pulses are shorter than the thermal relaxation time of the vessels, thereby limiting adjacent dermal and epidermal heating and preventing subsequent damage. Kono et al published a thorough review of PDL therapy for capillary malformations,27 which examines many of the idiosyncrasies of this treatment modality.
    • Skin treated with a PDL immediately develops edema and purpura, the latter of which generally last 1-2 weeks. Flashlamp-pumped PDL is relatively safe; major risks include pigmentation alterations (hyperpigmentation, which is always transient, and potentially permanent hypopigmentation), crusting, and, rarely, scarring. Immediate adverse events associated with PDL therapy are pain (a hot snapping sensation that can increase with repeated pulses) and combustion (especially of hair-bearing areas and in the presence of increased ambient oxygen). PDL therapy can be performed on patients with skin types V and VI, but with a potentially higher risk of pigmentary complications.
    • Some authorities advocate treatment beginning as early as 7-14 days of life, yet the age at which laser treatment achieves maximal response is unknown. Some studies indicate a better response if treatment is commenced before the patient is aged 1 year, while others reveal no difference. From a psychosocial perspective, treatment commencing in infancy is most beneficial.
    • The response rate depends on several variables. The first is anatomical location, with rates in descending order of location from best to worst as follows: neck, torso, face, and hand and arm; and with rates in descending order of location from best to worst for facial capillary malformations as follows: forehead, lateral part of the face, temple, and central part of the face. The second variable is size; smaller lesions appear to have higher response rates. These variables are influenced by the histologic features of capillary malformations. In 2004, videodermoscopy was used to demonstrate that capillary malformations with smaller vessels deep in the dermis were the most difficult to eradicate with PDL.28
    • Investigations are ongoing to explore methods to improve the outcome of laser surgery, including adjunctive use of exogenous heat,29 the topical application of the angiogenesis inhibitors rapamycin30 or imiquimod31 postoperatively, or intravenous porphyrin derivatives (photodynamic therapy)32 prior to laser surgery. While the early observations are promising, these interventions are strictly experimental at this time.
Capillary malformation on the left preauricular a...

Capillary malformation on the left preauricular aspect of the cheek, the ear, and the neck in a neonate (same patient as in Media Files 3-4).

[ CLOSE WINDOW ]
Capillary malformation on the left preauricular a...

Capillary malformation on the left preauricular aspect of the cheek, the ear, and the neck in a neonate (same patient as in Media Files 3-4).


Same patient as in Media Files 2 and 4 immediatel...

Same patient as in Media Files 2 and 4 immediately after test spots with the pulsed-dye laser at 585 nm. Note the purpuric macules where the laser impacted in a linear distribution on the preauricular aspect of the cheek.

[ CLOSE WINDOW ]
Same patient as in Media Files 2 and 4 immediatel...

Same patient as in Media Files 2 and 4 immediately after test spots with the pulsed-dye laser at 585 nm. Note the purpuric macules where the laser impacted in a linear distribution on the preauricular aspect of the cheek.


Same patient as in Media Files 2-3 after 4 treatm...

Same patient as in Media Files 2-3 after 4 treatments with the pulsed-dye laser. Treatments were given at 2-month intervals in an outpatient setting using topical anesthetic.

[ CLOSE WINDOW ]
Same patient as in Media Files 2-3 after 4 treatm...

Same patient as in Media Files 2-3 after 4 treatments with the pulsed-dye laser. Treatments were given at 2-month intervals in an outpatient setting using topical anesthetic.

  • Redarkening of capillary malformations can occur in patients treated by laser. The exact percentage of patients in which this occurs is unknown, with estimates from 11-50%. Currently, no characteristics help predict which lesions will redarken. No capillary malformations darken to the degree they were prior to embarking on laser treatment. Fifty-nine percent of patients are satisfied with the overall treatment result.33
  • Earlier laser therapies that caused an unacceptably high rate of scarring are not recommended; these therapies include carbon dioxide laser, copper vapor laser, and argon laser. Other modalities, such as intense pulsed light devices34 and the long-pulse35 or frequency-doubled Nd:YAG laser,36 are reported to be effective in certain instances, but they are generally considered to have higher complication rates than the PDL.
  • Previous nonlaser treatments determined unreliable or deleterious include surgical excision, radium implants, cryosurgery, electrocautery, sclerotherapy, and grenz ray radiotherapy.
  • Innovative methods such as laser speckle imaging37 and photoacoustic imaging38 to more accurately assess the vessel density, depth, and diameter and immediate efficacy of laser surgery are ongoing and represent the next frontiers toward improved treatment outcomes.

Consultations

More on Capillary Malformation

Overview: Capillary Malformation
Differential Diagnoses & Workup: Capillary Malformation
Treatment & Medication: Capillary Malformation
Follow-up: Capillary Malformation
Multimedia: Capillary Malformation
References
[ CLOSE WINDOW ]

References

  1. Happle R. What is a capillary malformation?. J Am Acad Dermatol. Dec 2008;59(6):1077-9. [Medline].

  2. Smoller BR, Rosen S. Port-wine stains. A disease of altered neural modulation of blood vessels?. Arch Dermatol. Feb 1986;122(2):177-9. [Medline].

  3. Chang CJ, Yu JS, Nelson JS. Confocal microscopy study of neurovascular distribution in facial port wine stains (capillary malformation). J Formos Med Assoc. Jul 2008;107(7):559-66. [Medline].

  4. Vural E, Ramakrishnan J, Cetin N, Buckmiller L, Suen JY, Fan CY. The expression of vascular endothelial growth factor and its receptors in port-wine stains. Otolaryngol Head Neck Surg. Oct 2008;139(4):560-4. [Medline].

  5. Kaji N, Nagase T, Nagase M, Koshima I. Changes in angiogenic gene expression in a case of expanded capillary malformation: does an expanded capillary malformation grow?. Ann Plast Surg. Jun 2005;54(6):645-50. [Medline].

  6. Boon LM, Mulliken JB, Vikkula M. RASA1: variable phenotype with capillary and arteriovenous malformations. Curr Opin Genet Dev. Jun 2005;15(3):265-9. [Medline].

  7. Hershkovitz D, Bergman R, Sprecher E. A novel mutation in RASA1 causes capillary malformation and limb enlargement. Arch Dermatol Res. Aug 2008;300(7):385-8. [Medline].

  8. Hershkovitz D, Bercovich D, Sprecher E, Lapidot M. RASA1 mutations may cause hereditary capillary malformations without arteriovenous malformations. Br J Dermatol. May 2008;158(5):1035-40. [Medline].

  9. Motley RJ, Lanigan SW, Katugampola GA. Videomicroscopy predicts outcome in treatment of port-wine stains. Arch Dermatol. Jul 1997;133(7):921-2. [Medline].

  10. Lanigan SW, Cotterill JA. Psychological disabilities amongst patients with port wine stains. Br J Dermatol. Aug 1989;121(2):209-15. [Medline].

  11. Sheehan DJ, Lesher JL Jr. Pyogenic granuloma arising within a port-wine stain. Cutis. Mar 2004;73(3):175-80. [Medline].

  12. Pratt AG. Birthmarks in infants. AMA Arch Derm Syphilol. Mar 1953;67(3):302-5. [Medline].

  13. Afsar FS, Ortac R. Acquired port-wine stain in a pediatric patient. J Cutan Med Surg. May-Jun 2006;10(3):151-3. [Medline].

  14. Geronemus RG, Ashinoff R. The medical necessity of evaluation and treatment of port-wine stains. J Dermatol Surg Oncol. Jan 1991;17(1):76-9. [Medline].

  15. Maari C, Frieden IJ. Klippel-Trénaunay syndrome: the importance of "geographic stains" in identifying lymphatic disease and risk of complications. J Am Acad Dermatol. Sep 2004;51(3):391-8. [Medline].

  16. Ch'ng S, Tan ST. Facial port-wine stains - clinical stratification and risks of neuro-ocular involvement. J Plast Reconstr Aesthet Surg. Aug 2008;61(8):889-93. [Medline].

  17. Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R, Viano J. Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci. Jul 2008;35(3):301-7. [Medline].

  18. Huiras EE, Barnes CJ, Eichenfield LF, Pelech AN, Drolet BA. Pulmonary thromboembolism associated with Klippel-Trenaunay syndrome. Pediatrics. Oct 2005;116(4):e596-600. [Medline].

  19. Samimi M, Maruani A, Bertrand P, Arbeille P, Lorette G. Arterial blood flow in limbs with port-wine stains can predict length discrepancy. Br J Dermatol. Jan 2009;160(1):219-20. [Medline].

  20. Toriello HV, Mulliken JB. Accurately renaming macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC) as macrocephaly-capillary malformation (M-CM). Am J Med Genet A. Dec 15 2007;143A(24):3009. [Medline].

  21. Guggisberg D, Hadj-Rabia S, Viney C, et al. Skin markers of occult spinal dysraphism in children: a review of 54 cases. Arch Dermatol. Sep 2004;140(9):1109-15. [Medline].

  22. Tubbs RS, Wellons JC 3rd, Iskandar BJ, Oakes WJ. Isolated flat capillary midline lumbosacral hemangiomas as indicators of occult spinal dysraphism. J Neurosurg. Feb 2004;100(2 Suppl Pediatrics):86-9. [Medline].

  23. Happle R. Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol. Mar 2005;141(3):385-8. [Medline].

  24. Lapidoth M, Ben Amitai D, Feinmesser M, Akerman L. Capillary malformation associated with angiolipoma: analysis of 127 consecutive clinic patients. Am J Clin Dermatol. 2008;9(6):389-92. [Medline].

  25. Happle R. Loss of heterozygosity in human skin. J Am Acad Dermatol. Aug 1999;41(2 Pt 1):143-64. [Medline].

  26. Kouba DJ, Yip D, Fincher EF, Moy RL. Topical imiquimod in the treatment of a long-standing capillary malformation. Br J Dermatol. Nov 2007;157(5):1071-2. [Medline].

  27. Kono T, Groff WF, Sakurai H. Treatment of port wine stains with the pulse dye laser. Ann Plast Surg. Apr 2006;56(4):460-3. [Medline].

  28. Sivarajan V, MacKay IR. The relationship between location, color, and vessel structure within capillary vascular malformations. Ann Plast Surg. Oct 2004;53(4):378-81. [Medline].

  29. Jia W, Aguilar G, Verkruysse W, Franco W, Nelson JS. Improvement of port wine stain laser therapy by skin preheating prior to cryogen spray cooling: a numerical simulation. Lasers Surg Med. Feb 2006;38(2):155-62. [Medline].

  30. Phung TL, Oble DA, Jia W, Benjamin LE, Mihm MC Jr, Nelson JS. Can the wound healing response of human skin be modulated after laser treatment and the effects of exposure extended? Implications on the combined use of the pulsed dye laser and a topical angiogenesis inhibitor for treatment of port wine stain birthmarks. Lasers Surg Med. Jan 2008;40(1):1-5. [Medline].

  31. Chang CJ, Hsiao YC, Mihm MC Jr, Nelson JS. Pilot study examining the combined use of pulsed dye laser and topical Imiquimod versus laser alone for treatment of port wine stain birthmarks. Lasers Surg Med. Nov 2008;40(9):605-10. [Medline].

  32. Gu Y, Huang NY, Liang J, Pan YM, Liu FG. [Clinical study of 1949 cases of port wine stains treated with vascular photodynamic therapy (Gu's PDT)]. Ann Dermatol Venereol. Mar 2007;134(3 Pt 1):241-4. [Medline].

  33. Huikeshoven M, Koster PH, de Borgie CA, Beek JF, van Gemert MJ, van der Horst CM. Redarkening of port-wine stains 10 years after pulsed-dye-laser treatment. N Engl J Med. Mar 22 2007;356(12):1235-40. [Medline].

  34. Ozdemir M, Engin B, Mevlitoglu I. Treatment of facial port-wine stains with intense pulsed light: a prospective study. J Cosmet Dermatol. Jun 2008;7(2):127-31. [Medline].

  35. Yang MU, Yaroslavsky AN, Farinelli WA, et al. Long-pulsed neodymium:yttrium-aluminum-garnet laser treatment for port-wine stains. J Am Acad Dermatol. Mar 2005;52(3 Pt 1):480-90. [Medline].

  36. Pence B, Aybey B, Ergenekon G. Outcomes of 532 nm frequency-doubled Nd:YAG laser use in the treatment of port-wine stains. Dermatol Surg. May 2005;31(5):509-17. [Medline].

  37. Huang YC, Ringold TL, Nelson JS, Choi B. Noninvasive blood flow imaging for real-time feedback during laser therapy of port wine stain birthmarks. Lasers Surg Med. Mar 2008;40(3):167-73. [Medline].

  38. Kolkman RG, Mulder MJ, Glade CP, Steenbergen W, van Leeuwen TG. Photoacoustic imaging of port-wine stains. Lasers Surg Med. Mar 2008;40(3):178-82. [Medline].

  39. [Guideline] Shevell M, Ashwal S, Donley D, et al. Practice parameter: evaluation of the child with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society. Neurology. Feb 11 2003;60(3):367-80. [Medline].

  40. Fonder MA, Mamelak AJ, Kazin RA, Cohen BA. Port-wine-stain-associated dermatitis: implications for cutaneous vascular laser therapy. Pediatr Dermatol. Jul-Aug 2007;24(4):376-9. [Medline].

  41. Boukobza M, Enjolras O, Cambra M, Merland J. [Sturge-Weber syndrome. The current neuroradiologic data]. J Radiol. Jul 2000;81(7):765-71. [Medline].

  42. Burrows PE, Laor T, Paltiel H, Robertson RL. Diagnostic imaging in the evaluation of vascular birthmarks. Dermatol Clin. Jul 1998;16(3):455-88. [Medline].

  43. Eerola I, Boon LM, Mulliken JB, et al. Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am J Hum Genet. Dec 2003;73(6):1240-9. [Medline].

  44. Eubanks LE, McBurney EI. Videomicroscopy of port-wine stains: correlation of location and depth of lesion. J Am Acad Dermatol. Jun 2001;44(6):948-51. [Medline].

  45. Garzon MC, Huang JT, Enjolras O, Frieden IJ. Vascular malformations: Part I. J Am Acad Dermatol. Mar 2007;56(3):353-70; quiz 371-4. [Medline].

  46. Goldman MP, Fitzpatrick RE. Laser treatment of cutaneous vascular lesions. In: Goldman MP, Fitzpatrick RE, eds. Cutaneous Laser Surgery: The Art and Science of Selective Photothermolysis. 2nd ed. Mosby-Year Book: St. Louis, Mo; 1999:37-74.

  47. Goldman MP, Fitzpatrick RE, Ruiz-Esparza J. Treatment of port-wine stains (capillary malformation) with the flashlamp-pumped pulsed dye laser. J Pediatr. Jan 1993;122(1):71-7. [Medline].

  48. Huikeshoven M, Koster PH, de Borgie CA, Beek JF, van Gemert MJ, van der Horst CM. Redarkening of port-wine stains 10 years after pulsed-dye-laser treatment. N Engl J Med. Mar 22 2007;356(12):1235-40. [Medline].

  49. Mazereeuw-Hautier J, Syed S, Harper JI. Bilateral facial capillary malformation associated with eye and brain abnormalities. Arch Dermatol. Aug 2006;142(8):994-8. [Medline].

  50. Mulliken JB. Capillary (port-wine) and other telangiectatic stains. In: Vascular Birthmarks. Portland, Ore: Book News; 1988:170-83.

  51. Requena L, Sangueza OP. Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilation of preexisting vessels. J Am Acad Dermatol. Oct 1997;37(4):523-49; quiz 549-52. [Medline].

  52. Selim MM, Kelly KM, Nelson JS, Wendelschafer-Crabb G, Kennedy WR, Zelickson BD. Confocal microscopy study of nerves and blood vessels in untreated and treated port wine stains: preliminary observations. Dermatol Surg. Jun 2004;30(6):892-7. [Medline].

  53. Sivarajan V, Maclaren WM, Mackay IR. The effect of varying pulse duration, wavelength, spot size, and fluence on the response of previously treated capillary vascular malformations to pulsed-dye laser treatment. Ann Plast Surg. Jul 2006;57(1):25-32. [Medline].

  54. Waner M, Suen JY. The treatment of vascular malformations. In: Hemangiomas and Vascular Malformations of the Head and Neck. New York, NY: John Wiley & Sons; 1999:353-66.

  55. Waner M, Suen JY. The natural history of vascular malformations. In: Hemangiomas and Vascular Malformations of the Head and Neck. New York, NY: John Wiley & Sons; 1999:52-62.

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

capillary malformation, nevus flammeus, naeuvus flammeus, NF, port-wine stain, port-wine mark, portwine stain, port wine stain, strawberry patch, naevus maternus, venular malformation, venulocapillary malformation, vascular malformation (not capillary hemangioma), birthmark, birth mark

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Richard J Antaya, MD, Director of Pediatric Dermatology, Associate Professor, Departments of Dermatology and Pediatrics, Yale University
Richard J Antaya, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Mark W Cobb, MD, Consulting Staff, WNC Dermatological Associates
Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society of Dermatopathology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.