Asymmetrical nevus flammeus (overview) Q82.5

Non-recoverable vascular, primarily capillary malformation (CM), which is visible at birth or manifests itself at a later age, but can also affect venous and/or arterial vascular systems of the skin and/or other organs. S. and vascular malformations.

From a clinical point of view, a distinction can be made:

• The homogeneous "Port-wine" type
• The teleangiectatic type
• Naevus flammeus with associated symptoms
  • phacomatosis pigmentovascularis
    • Type I: Nevus flammeus combined with Nevus pigmentosus verrucosus
    • Type II: Nevus flammeus combined with mongol stain (dermal melanocytosis)
    • Type III: Nevus flammeus combined with Nevus spilus
    • Type IV: Nevus flammeus combined with Nevus spilus and Mongolian stain, possibly also Nevus anaemicus.
  • Vascular vascularis mixtus (Vascular twin nevus): Rare clinical picture characterized by the combination of a flammeus nevus with an anaemic nevus .
  • Klippel-Trénaunay syndrome
  • Sturge Weber Crab Syndrome
  • Proteus syndrome
  • Cloves Syndrome

Capillary malformations (KM) occur in about 0.3% of the population (exact epidemiological data are not available). There is no gender preference.

Usually bizarrely configured, light red spot in the first years of life, later deep red to blue-red (red wine) spot (completely compressible by glass spatula printing). The size ranges from 0.5 cm in diameter to large patches that extend over wide areas of the body. Bizarre formations can occur. An arrangement in the Blaschko lines does not exist.

A nevus flammeus does not show spontaneous area growth, but only increases in size according to the growth of the body. After decades of existence, bumpy, blue-red plaques, papules or nodules form. There is a tendency to bleed with banal injuries.

• Pale naevi (infancy and childhood): The best results are obtained with the pulsed dye laser (585 nm or 577 nm, see laser). Disadvantage: Financially expensive, only available in specialized institutions. Brightening effects on approx. 70% of the naevi. Scarring occurs very rarely if used properly. Naevi respond best to the head, trunk or neck. Sufficient are usually 5-7 sessions in intervals of 6-8 weeks with energy densities of 5.5-8 J/cm2, depending on the best whitening after previous trial laser treatment (1 cm2). Preparation: Especially in children, the prior administration of local anesthesia (e.g. EMLA cream) is recommended due to painfulness, if necessary, also use systemic analgesics or general anesthesia. However, 10-year results show that almost 60% of laser-treated nevi flamei darken again.
• Dark nevi (adult): Dark nevi often respond relatively well to the argon laser. A lengthy treatment is usually necessary. The response can often only be assessed after several sessions. Careful dosing can greatly reduce the risk of scarring and hypopigmentation. If the response is insufficient, pulsed dye lasers may be considered. Here too, 5 or more sessions are usually necessary. Nodular components respond only poorly. More can be achieved with Photoderm VL (high-energy flash lamp with a broad wavelength spectrum).
• Before starting a laser treatment: Inform the patient about 1-2 weeks of persistent discoloration, avoid direct sunlight and use light protection (e.g. Anthelios) for the entire duration of the laser treatment. Alternative: Cosmetic cover e.g. with Dermacolor. Use cryosurgery only if you have sufficient experience with the therapy.
• Clarification and if necessary treatment of accompanying anomalies, see below respective syndrome.

Remember! A facial nevus flammeus is a compelling indication for ophthalmological clarification (glaucoma, retinal damage). The dogma that facial nevi flammeus reflect the trigeminal dermatomes V1,V2 and V3 has obviously been rightly doubted (Happle R 2014). The patients treated with laser should be informed about a possible darkening of the areas.

The frequent nuchal-frontal capillary malformations of the neck or the upper eyelids (stork bite, angel kiss), formerly called "Naevus flammeus medialis", are completely harmless. The capillary nuchal nevus persists in most cases for life.

1. Happle R (2014) How common are genetic mosaics of the skin? dermatologist 65: 536-541
2. Huikeshoven M et al (2007) Redarkening of port-wine stains 10 years after puls-dye-laser-treatment. N Engl J Med 156: 1235-1240
3. Katugampola GA et al (1996) The clinical spectrum of naevus anaemicus and its association with port
   winestains: report of 15 cases and a review of the literature. Br J Dermatol 134:292-295.
4. Larralde M et al (2014) Capillary malformation-arteriovenous malformation: a clinical review of 45 patients. Int J Dermatol 53:458-461.