II. Definitions

  1. Nevus Flammeus (Port-Wine Stain)
    1. Persistent vascular Birthmark

III. Epidemiology

  1. Incidence: 0.3% to 0.5% of newborns (1 in 300)
  2. Not genetic

IV. 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

V. 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)

VI. Differential Diagnosis

VII. 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

VIII. 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

IX. 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]

X. Course

  1. Persist and may become more prominent over time (contrast with Hemangiomas which fade)

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Related Studies

Ontology: Port-Wine Stain (C0235752)

Definition (NCI) A congenital vascular malformation in the skin (birthmark) characterized by the presence of dilated capillaries. The affected area of the skin is flat and reddish-purplish in color.
Definition (MSH) A vascular malformation of developmental origin characterized pathologically by ectasia of superficial dermal capillaries, and clinically by persistent macular erythema. In the past, port wine stains have frequently been termed capillary hemangiomas, which they are not; unfortunately this confusing practice persists: HEMANGIOMA, CAPILLARY is neoplastic, a port-wine stain is non-neoplastic. Port-wine stains vary in color from fairly pale pink to deep red or purple and in size from a few millimeters to many centimeters in diameter. The face is the most frequently affected site and they are most often unilateral. (From Rook et al., Textbook of Dermatology, 5th ed, p483)
Concepts Congenital Abnormality (T019)
MSH D019339
ICD10 Q82.5
SnomedCT 416377005, 67668002, 205560007, 157013001, 254211001
English PORT WINE STAIN, Nevus Flammeus, Port-Wine Stain, Port-Wine Stains, Stain, Port-Wine, Stains, Port-Wine, Port Wine Type Hemangioma, Salmon Patch Nevus, PORT-WINE STAIN, port wine nevus (diagnosis), nevus flammeus (diagnosis), nevus flammeus (port wine nevus) (physical finding), nevus flammeus (port wine nevus), nevus flammeus, port wine nevus, Port wine nevus, Port wine naevus, Port wine stain, congenital nevus flammeus, Flammeus Nevus, Portwine Nevus, Port-Wine Stain [Disease/Finding], Naevus flammeus, naevus flammeus, port wine birthmark, portwine stains, bites stork, port-wine birthmark, port-wine stain, port stain wine, portwine stain, flammeus nevus, port stains wine, stork bite, Port-wine stain (disorder), Port-Wine Stain of Skin, Salmon patch naevus, Salmon patch nevus, Stork bite, Unna's naevus, Unna's nevus, Salmon patch nevus (disorder), flammeus; nevus, nevus; flammeus, nevus; portwine, port wine; spot, spot; port wine, stain, portwine, Port-wine stain of skin, NOS, Port Wine Stain, Port Wine Birthmark, Port Wine Nevus, Port Wine Stain of Skin, Port Wine Stain of the Skin, Nevus flammeus, Portwine nevus, Portwine naevus, Port-wine birthmark, Port-wine naevus, Port-wine nevus, PWS - Port-wine stain, Port-wine stain, Port-wine stain of skin (disorder), Port-wine stain of skin, port wine stain
German NAEVUS FLAMMEUS, Storchenbiss, Portweinfleck, Portweinnaevus, Feuermal, Naevus flammeus, Port-Wine Stain, Portweinnävus
Swedish Portvinsfläckar
Dutch ooievaarsbeet, naevus flammeus, wijnvlek, wijnvlek-naevus, flammeus; naevus, naevus; flammeus, naevus; vinosus, vlek; wijn, wijn; vlek, Naevus flammeus, Vuurvlek
French Morsure de cigogne, Nevus flammeus, TACHES DE VIN, Naevus flammeus, Tache lie de vin, Angiome plan, Envie, Tache de vin congénitale, Tache de vin
Italian Nevus flammeus, Morso della cicogna, Macchia color vino porto, Nevo flammeo, Nevo vinoso, Nevus Flammeus, Nevo angiomatoso
Portuguese Nevus flammeus, Naevus flammeus, Picada de cegonha, Nevo cor de vinho, Nevo vinho-do-porto, Mancha vinho-do-porto, MANCHA COR DE VINHO DO PORTO, Mancha Vinho do Porto, Mancha de Vinho do Porto, Nevo Flâmeo, Mancha Vinho-do-Porto, Mancha de Vinho, Nevus Flammeus
Spanish Marca de la cigüeña, Nevus en vino de Oporto, Mancha de color vino de Oporto, MANCHA EN VINO DE OPORTO, Mancha Vino de Oporto, Nevus flámeo, Nevus de color vino de Oporto, mancha de vino oporto de la piel, nevo vino oporto, mancha de vino oporto de la piel (trastorno), Mancha de Vino de Oporto, Mancha Vino Oporto, Mancha Vino-de-Oporto, Mancha de Vino Oporto, nevo en mancha de vino de Oporto (trastorno), nevo en mancha de vino de Oporto, nevo flámeo, nevus flammeus, angioma plano (trastorno), angioma plano, mancha en vino de oporto en la piel, Nevo Flammeus
Japanese カエンジョウボハン, ウンナボハン, ウンナ母斑, ポートワインボハン, ポートワイン母斑, 火炎状母斑, ぶどう酒様血管腫, ポートワイン血管腫, ワイン様血管腫, 単純性血管腫, 成熟毛細血管腫, 赤酒しみ様血管腫, ブドウ酒様血管腫, 母斑-火炎状, 血管腫-ブドウ酒様, 血管腫-単純性
Czech névus barvy portského vína, névus vínový, naevus flammeus, Cévní névus (barvy portského vína), Nevus flammeus, Naevus flammeus, Kousnutí čápem
Finnish Portviiniluomi
Russian KOZHNO-SOSUDISTAIA ANOMALIIA, PORTVEINOVOE PIATNO, PORTVEINOVOE PIATNO KOZHNO-SOSUDISTOE, NEVUS FLAMMEUS, КОЖНО-СОСУДИСТАЯ АНОМАЛИЯ, ПОРТВЕЙНОВОЕ ПЯТНО, ПОРТВЕЙНОВОЕ ПЯТНО КОЖНО-СОСУДИСТОЕ
Croatian NAEVUS FLAMEUS
Polish Znamię płomieniste, Plama czerwonego wina
Hungarian Tűzfolt-anyajegy, Jóindulatú érdaganat a bőrön, Naevus flammeus, Haemangioma simplex cutis, Tűzfolt, Naevus haemangiomatosus
Norwegian Nevus flammeus, Portvinmerke