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LC50.1
3.1.4 Nevus Flammeus
Grading & Level of Importance: B
ICD-11
Synonyms
Nevus vinosus, Portwine nevus, Naevus teleangiectaticus.
Epidemiology
0.3-1% of the population; F 2x M.
Definition
Benign vascular malformation since birth. Sharply demarcated red spots, due to dilatation and augmentation of small superficial vessels in the affected area.
Aetiology & Pathogenesis
Inherited. May be in the nosologic context of angiodysplasia syndromes (Sturge-Weber-Syndrom, von Hippel-Lindau-syndrome, Klippel-Trénaunay / Parkes-Weber-syndrome).
Signs & Symptoms
Sharply demarcated red spots and patches without augmentation of tissue.
Localisation
- Axial nevi flammei: (Unna-nevus; angel’s kiss of the forehead; Salmon Patch); never associated with syndromatic angidystrophy.
- Lateral nevi flammei, syndrome associated quadrant nevus
- Trigeminus-area: unilateral Portwine-nevus; potential association with meningeal or central nervous angiomas of eyes, meninges and brain (Sturge Weber syndrome)
- Extremities: possible association with venous malformation and increased growth of soft tissue and bones of the respective limb (Klippel-Trénaunay syndrome).
Classification
- True nevoid not self-regressing capillary malformations
- Temporary and regressing capillary malformation «Salmon Patches» (neck, forehead)
Laboratory & other workups
None. Ultrasound for the determination of depth.
Dermatopathology
Not needed. Dilated vessels in the dermis.
Course
In contrast to axial nevi, the lateral nevi do not regress spontaneously, but enlarge with body growth, change color to deep red, and can show tuberous transformation (i.e., macrocheily) and pyogenic granuloma.
Complications
Exclude involvement of eye (glaucoma) in periorbital nevus flammeus. Additional complications may be found in conjunction with angiodysplastic syndromes (CNS-involvement; hemorrhage; thromboses; limb deformities).
Diagnosis
Typical clinical features.
Differential diagnosis
Infantile hemangioma: red-blue vascular tumor (not nevus!), developing in the first week of life, grows proportionally and regresses spontaneously after months.
Prevention & Therapy
Laser, intense pulse light, cosmetic camouflage.
Ophthalmologic control for nevi covered by trigeminus I and II.
Differential Diagnosis
Podcasts
Tests
- Nevus flammeus can change their intensity of color
- Aussage 1 Teleangiectasias are usually acquired
- Statement 1: Nevus flammeus are already present at birth,
- Hemangiomas generally do not require treatment
- Nevus flammeus is usally a harmless skin lesion
- Statement 1 Nevus flammeus can change their intensity of color
- Statement 1 Nevus flammeus can pose considerable cosmetic problems
- Statement 1 A nevus flammeus can be identified with the naked eye
- Statement 1 Telangiectases are usually acquired
- Statement 1 A nevus flammeus is a malformation
- Statement 1 Nevus flammeus is present at birth
- Statement 1 Hemangiomas generally do not require treatment
- Statement 1 Nevus flammeus is a common and usually harmless lesion
- One of the most common malformations?
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