3.1.4 Nevus Flammeus

Grading & Level of Importance: B

Review:
2021

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

ICD-11

LC50.1

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.

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