Contents

Search

nevus flammeus (port-wine stain)

Irregularly-shaped, red or violaceous, macular, vascular malformation (capillary angioma) of dermal blood vessels present at birth. Etiology: - most port-wine stains are isolated findings in otherwise healthy children - Sturge-Weber syndrome - Klippel-Trenaunay syndrome Epidemiology: incidence 0.3% Pathology: 1) ectasia of capillaries 2) no endothelial proliferation Clinical manifestations: 1) irregularly-shaped, pink to violaceous, macule present at birth - described as blanchable erythema in ref [3] 2) present for life, except for nevus flammeus nuchae (salmon patch) 3) with increasing age, papules or nodules often develop leading to significant disfigurement 4) 85% are unilateral following a dermatomal distribution & rarely crossing midline 5) most commonly involves face, but may involve any skin site 6) asymptomatic 7) Sturge-Weber syndrome with port-wine-stains in the V1 (ophthalmic nerve) or V2 (maxillary nerve) ditributions of the trigeminal nerve 8) Klippel-Trenaunay syndrome with with port-wine-stains on the extremities rather than the face Radiology: CT scan to rule out Sturge-Weber syndrome Management: 1) screen all patients for glaucoma & CNS involvement 2) during macular phase, make-up will cover stained skin 3) laser therapy with tunable dye or copper vapor laser

Related

Sturge-Weber syndrome (encephalotrigeminal angiomatosis)

Specific

nevus flammeus nuchae (salmon patch, erythema nuche, stork bite)

General

nevus cutaneous hemangioma

References

  1. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 151-53
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 175
  3. NEJM Knowledge+ Question of the Week. Jan 10, 2017 http://knowledgeplus.nejm.org/question-of-week/4789/