nevus flammeus (port-wine stain)
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Introduction
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
Clinical manifestations
- irregularly-shaped, pink to violaceous, macule present at birth
- described as blanchable erythema in ref[3]
- present for life, except for nevus flammeus nuchae (salmon patch)
- with increasing age, papules or nodules often develop leading to significant disfigurement
- 85% are unilateral following a dermatomal distribution & rarely crossing midline
- most commonly involves face, but may involve any skin site
- asymptomatic
- Sturge-Weber syndrome with port-wine-stains in the V1 (ophthalmic nerve) or V2 (maxillary nerve) ditributions of the trigeminal nerve
- 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
- screen all patients for glaucoma & CNS involvement
- during macular phase, make-up will cover stained skin
- laser therapy with tunable dye or copper vapor laser
More general terms
More specific terms
Additional terms
References
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 151-53
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 175
- ↑ 3.0 3.1 NEJM Knowledge+ Question of the Week. Jan 10, 2017 http://knowledgeplus.nejm.org/question-of-week/4789/