Asymmetric nevus flammeus

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: -

Lateral nevus, nevus flammeus, port-wine stain, nevus vinosus.

Congenital or early-developing malformation of the capillaries and postcapillary venules, localised laterally of the midline.

Vascular innervation is not present, thus vascular tone is reduced, resulting in vasodilatation.

  • Red wine-coloured, blue-red, sharply demarcated macules. Compression during glass spatula printing causes the redness to disappear.
  • Associated malformations
    • Phakomatosis pigmentovascularis
    • Congenital angiodysplasia syndromes

In case of periorbital localisation, an ophthalmological examination (glaucoma, retinal damage) is indicated.

Subepidermal capillary dilatation.

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  2. Achtelik W, Tronnier M, Wolff HH. [Combined naevus flammeus and naevus fuscocoeruleus: phacomatosis pigmentovascularis type IIa]. Hautarzt 1997;48:653-6.
  3. Taute BM, Podhaisky H, Wiegand E, Eifert S, Behrmann C. [Unilateral giant extremity growth, naevus flammeus and chronic venous insufficiency in a 48-year-old patient]. Internist (Berl) 1998;39:308-11.
  4. Johnson SC, Hanke CW. Unilateral acquired nevus flammeus in women. Cutis 2001;67:225-8.