Port-Wine Stain

Aka: Port-Wine Stain, Nevus Flammeus

1. See Also
   1. Newborn Skin Exam
2. Definition
   1. Persistent vascular Birthmark
3. Epidemiology
   1. Incidence: 0.3% to 0.5% of newborns
4. Pathophysiology
   1. Vascular Malformation of mature ectatic capillaries
      1. Superficial Capillaries (seen in Salmon Patch)
      2. Deeper vessels of Dermis and subcutaneous tissue
5. Signs
   1. Flat, unilateral Macules on face or extremities
   2. Present at birth and may deepen in color with time
   3. Varied coloration
      1. Pale pink to reddish-blue or purple vascular patch
6. Differential Diagnosis
   1. Hemangioma
7. Evaluation
   1. Lesion located near eye requires pediatric ophthalmology evaluation
      1. Sturge-Weber Syndrome (5-8%)
      2. Neonatal Glaucoma
   2. Lesion associated with extremity hypertrophy
      1. Klippel-Trenaunay-Weber Syndrome
8. Associated Conditions
   1. Klippel-Trenaunay-Weber Syndrome
   2. Sturge-Weber Syndrome
   3. Neonatal Glaucoma (when ophthalmic branch of the Trigeminal Nerve is involved)
9. Management
   1. Treatment is cosmetic only (to lighten lesions)
   2. Compression garment if on extremities
   3. Pulsed-dye laser therapy
      1. Optimally treated before age 1 year
      2. Protocol: Total of 5 sessions each spaced 2 weeks apart
      3. Tomson (2006) Br J Dermatol 154:676-9 [PubMed]
10. Course
    1. Persist and may become more prominent over time (contrast with Hemangiomas which fade)
11. References
    1. Nguyen (1998) Br J Dermatol 138:821-5 [PubMed]
    2. McLaughlin (2008) Am Fam Physician 77:56-60 [PubMed]