Port-wine stain

From Wikipedia, the free encyclopedia - View original article

Nevus flammeus
Classification and external resources

Mikhail Gorbachev has a prominent port-wine stain on his forehead.
ICD-10Q82.5 (ILDS Q82.510)
ICD-9757.32
OMIM163000
DiseasesDB10384
MedlinePlus001475
eMedicinederm/295
MeSHD019339
 
Jump to: navigation, search
Nevus flammeus
Classification and external resources

Mikhail Gorbachev has a prominent port-wine stain on his forehead.
ICD-10Q82.5 (ILDS Q82.510)
ICD-9757.32
OMIM163000
DiseasesDB10384
MedlinePlus001475
eMedicinederm/295
MeSHD019339

A port-wine stain or nevus flammeus is a vascular anomaly consisting of superficial and deep dilated capillaries in the skin which produce a reddish to purplish discoloration of the skin. They are so called for their colour, resembling that of port wine. It is part of the family of disorders known as vascular malformations, specifically an arteriovenous malformation.

The two terms are not always equated. Sometimes the term "nevus flammeus" is divided into two categories: port-wine stain and salmon patch.[1][2]

Port-wine stains are present at birth and persist throughout life; they are birth marks. The area of skin affected grows in proportion to general growth. Port-wine stains occur most often on the face but can appear anywhere on the body. Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish colour. In adulthood, thickening of the lesion or the development of small lumps may occur.[3]

Port-wine stains may be part of a syndrome such as Sturge–Weber syndrome or Klippel–Trénaunay–Weber syndrome.

Contents

Types

A hand with a red port-wine stain

Nevus flammeus may be divided as follows:[4]

Genetics

An association with RASA1 has been described.[5]

Sturge–Weber syndrome is the association of a facial port-wine stain with a corresponding haemangioma in the brain, leading to contralateral focal fits.

Diagnosis

A physician can usually diagnose a port wine stain based entirely upon the history and appearance. In unusual cases, a skin biopsy may be needed to confirm the diagnosis. Depending on the location of the birthmark and other associated symptoms, a physician may choose to order a measurement of intraocular pressure or X-ray of the skull.

A MRI scan of the brain may be performed (under anesthesia) on infants who have a Port-wine stain in the head area. This is done to check for signs of Sturge-Weber syndrome. The brain can also in addition be examined with an ultrasound.

If the Port-wine stain exists inside the mouth, a physician may check the insides of a newborn baby's throat with a scope to see if there are any changes (growths) other than just the color.

Treatment

Many treatments have been tried for port-wine stains including freezing, surgery, radiation, and tattooing; port-wine stains can also be covered with cosmetics. Lasers have made the biggest impact on treatment, because they are the sole method of destroying the cutaneous capillaries without significant damage to the overlying skin.

The flashlamp pumped dye laser, a yellow light laser, has been the most successful at destroying stains in infants and young children.[6] The neodymium YAG laser is used to treat the nodules that may develop in some adult port-wine stains. The use of YAG laser is not recommended on infants, as it may leave scars.

Treatment of infants with the flashlamp pumped dye laser generally produces marked improvement in appearance.[5] Usually a lighter shade Port-wine stain requires 4 laser treatments, whereas the darker ones require 7 or more.

However, complete disappearance is rare. In approximately 20%[7] of cases, there may be no improvement at all. Stains on the face respond better than those on the trunk or limbs. Older stains may be more difficult to treat.

The laser treatment can be done in 6 week intervals. However, as it is recommended to be performed under anesthesia (15 minutes) on small children, it is not always possible to get the treatment that often. For example in Finland a child gets treated 2-3 times per year, resulting in a target of "being ready before school age" (7 years).

After the laser treatment the skin is filled with black marks, the size of a pen. This is due to the laser instrument's size. The black marks will disappear within 1-3 weeks. The treated area can be sore and swollen for a couple of days.

Prognosis

In the absence of successful treatment, hypertrophy (increased tissue mass) of the stains may produce deformity, loss of function (especially near the eye or mouth), bleeding, and increasing disfigurement. These complications are usually seen later in life. If the port-wine stain is on the face or other highly visible part of the body, its presence can also cause emotional and social problems for the affected person.

Epidemiology

The incidence is 3–5 out of 1000 persons.[8][9]

References

  1. ^ "nevus flammeus" at Dorland's Medical Dictionary
  2. ^ "port-wine stain" at Dorland's Medical Dictionary
  3. ^ Minkis, K; Geronemus, RG; Hale, EK (2009). "Port wine stain progression: A potential consequence of delayed and inadequate treatment?". Lasers in surgery and medicine 41 (6): 423–6. doi:10.1002/lsm.20788. PMID 19588535. 
  4. ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 582–3. ISBN 0-7216-2921-0.
  5. ^ a b Eerola I, Boon LM, Mulliken JB et al. (December 2003). "Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations". Am. J. Hum. Genet. 73 (6): 1240–9. doi:10.1086/379793. PMC 1180390. PMID 14639529. 
  6. ^ Chapas, AM; Eickhorst, K; Geronemus, RG (2007). "Efficacy of early treatment of facial port wine stains in newborns: A review of 49 cases". Lasers in surgery and medicine 39 (7): 563–8. doi:10.1002/lsm.20529. PMID 17868100. 
  7. ^ Jasim ZF, Handley JM (2007). "Treatment of pulsed dye laser-resistant port wine stain birthmarks". J. Am. Acad. Dermatol. 57 (4): 677–82. doi:10.1016/j.jaad.2007.01.019. PMID 17658196. 
  8. ^ Jacobs, AH; Walton, RG (1976). "The incidence of birthmarks in the neonate". Pediatrics 58 (2): 218–22. PMID 951136. 
  9. ^ Alper, JC; Holmes, LB (1983). "The incidence and significance of birthmarks in a cohort of 4,641 newborns". Pediatric dermatology 1 (1): 58–68. doi:10.1111/j.1525-1470.1983.tb01093.x. PMID 6679890. 

External links