3.1.4 Nevus Flammeus

Grading & Level of Importance: B

Review:

2026

W. Burgdorf, Munich; A. Salam, J. McGrath, London;
Revised by M. Bagot, Paris; G. Dobos, Paris; A. de Masson, Paris

Overview:

ICD-11
Synonyms
Epidemiology
Definition
Aetiology & Pathogenesis
Signs & Symptoms
Localisation
Classification
Laboratory & other workups
Dermatopathology
Course
Complications
Diagnosis
Differential Diagnosis
Prevention & Therapy
Special

ICD-11

LC50.1

Synonyms

Nevus vinosus, Portwine nevus, Naevus teleangiectaticus.

Epidemiology

0.3-1% of the population; F 2x M.

Naevus flammeus is frequent. It affects 0.3-1% of the population, with women being twice as likely to be affected as men. Cases are usually sporadic, but a 10% familial incidence and an autosomal dominant inheritance have been described.

Definition

Benign vascular malformation since birth. Sharply demarcated red spots, due to dilatation and augmentation of small superficial vessels in the affected area.

Nevus flammeus is a sharply circumscribed red to bluish (port-wine coloured) patch present since birth. This is a benign vascular malformation consisting of increased numbers of dilated dermal vessels.

Aetiology & Pathogenesis

Inherited. May be in the nosologic context of angiodysplasia syndromes (Sturge-Weber-Syndrom, von Hippel-Lindau-syndrome, Klippel-Trénaunay / Parkes-Weber-syndrome).

This is a benign skin condition, present since birth.

It is rarely associated with genetic angiodysplastic syndromes (Sturge-Weber syndrome (encephalotrigeminal angiomatosis) associated with GNAQ mutations, or Klippel-Trénaunay syndrome associated with PIK3CA mutations).

Signs & Symptoms

Sharply demarcated red spots and patches without augmentation of tissue.

Lesions are sharply circumscribed red or violaceus macules or patches without palpable features. In rare, unilateral cases, papules or nodules may appear over time.

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)

Nevus flammeus comprise capillary malformations representing real naevi, and capillary malformations that do not represent nevi, such as the frequent median and regressive lesions now called salmon patches.

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.

Axial lesions tend to regress spontaneously but may not disappear completely. Lateral ones grow with body growth, get darker with age and may develop nodular changes (e.g. soft tissue enlargement of lips) and pyogenic granulomas.

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).

In periorbital nevi flammei ocular involvement (glaucoma, which can lead to blindness) should be excluded by an ophthalmologist, and brain magnetic resonance imaging (MRI) should be performed to exclude meningeal or central nervous system angioma (Sturge-Weber syndrome), which can be complicated with seizures, stroke, or mental retardation.

Klippel-Trénaunay syndrome may be associated with complications (bleeding, thrombosis, soft tissue 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.

Nevus flammeus may spontaneously disappear in the first 6 months of life (especially the stork bite and angel’s kiss types). A treatment is generally not needed and only indicated in cosmetically or functionally severe cases. Cosmetic camouflage, laser treatment, intense pulsed light, may be used.