Peds

Port-Wine Stain

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Port-Wine Stain, Nevus Flammeus

  • See Also
  1. Newborn Skin Exam
  • Definitions
  1. Nevus Flammeus (Port-Wine Stain)
    1. Persistent vascular Birthmark
  • Epidemiology
  1. Incidence: 0.3% to 0.5% of newborns
  • Pathophysiology
  1. Vascular Malformation of mature ectatic capillaries
    1. Superficial Capillaries (seen in Salmon Patch)
    2. Deeper vessels of Dermis and subcutaneous tissue
  • Signs
  1. Flat, unilateral, well-demarcated 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
  4. Lesions may progress
    1. Nodules
    2. Pyogenic Granuloma
    3. Soft tissue hypertrophy (e.g. Gingiva)
  • Differential Diagnosis
  1. Hemangioma
  • 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
  • Associated Conditions
  1. Klippel-Trenaunay-Weber Syndrome
  2. Sturge-Weber Syndrome
    1. Congenital Neurocutaneous condition
    2. Associated with CNS and eye Vascular Malformations
  3. Neonatal Glaucoma
    1. When ophthalmic branch of the Trigeminal Nerve is involved
  • 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. Transient Purpura, edema, crusting or Blistering
    4. Rare longterm complications (e.g. scarring, pigment changes)
    5. Tomson (2006) Br J Dermatol 154:676-9 [PubMed]
  • Course
  1. Persist and may become more prominent over time (contrast with Hemangiomas which fade)
  • References
  1. Nguyen (1998) Br J Dermatol 138:821-5 [PubMed]
  2. McLaughlin (2008) Am Fam Physician 77:56-60 [PubMed]
  3. Snyder (2024) Am Fam Physician 109(3): 212-6 [PubMed]