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 (1 in 300)
  2. Not genetic
  • Pathophysiology
  1. Vascular Malformation of mature ectatic capillaries
    1. Superficial Capillaries (seen in Salmon Patch)
    2. Deeper vessels of Dermis and subcutaneous tissue
    3. Associated with decreased Sympathetic Nerve fibers in the region of the lesions
  • Signs
  1. Flat, well-demarcated Macules on face or extremities
    1. Unilateral (rarely cross the midline)
  2. Present at birth and may deepen in color with time
  3. Varied coloration and borders
    1. Blanchable, pale pink to reddish-blue or purple vascular patch
    2. Irregular borders
  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 Eyelid requires pediatric ophthalmology evaluation
    1. Sturge-Weber Syndrome (5-8%)
    2. Neonatal Glaucoma (10% of lesions that involve the Eyelid)
  2. Lesion associated with extremity hypertrophy
    1. Klippel-Trenaunay-Weber Syndrome
  • Associated Conditions
  1. Klippel-Trenaunay-Weber Syndrome
  2. Sturge-Weber Syndrome
    1. Overall 1 in 20,000 to 1 in 50,000 children (more common with Eyelid involvement)
    2. Congenital Neurocutaneous condition
    3. Associated with CNS and eye Vascular Malformations
    4. Risk of blindness, focal Epilepsy, Hemiplegia and Learning Disorders
  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. Poliner (2022) Pediatr Rev 43(9): 507-16 [PubMed]
  4. Snyder (2024) Am Fam Physician 109(3): 212-6 [PubMed]