Prognosis — Capillary malformation

  • Chapter: Capillary malformations

    Article: 8 of 14

    Update: Mar 30, 2021

  • Author(s): Ott, Hagen

The clinical appearance of capillary malformations (CM) is subject to age-related changes, especially in the facial region. Only nevus simplex (“angel’s kiss”), which appears in the midline of the face, usually shows spontaneous complete regression within the first two years of life. Classic capillary malformations (“port wine stains”) do not regress completely (unlike infantile hemangiomas). They may fade from about the 3rd month of life. This is because patients in this phase of infancy show a physiological decrease in the formation of red blood cells, and the increased thickening of the skin that begins with growth makes the skin more opaque, thus reducing the extent to which the dilated capillaries show through. However, this fading tends to be more pronounced on the extremities and trunk, and less in the face/neck area.

In the extremities fading usually progresses from proximal to distal. However, complete regression is not to be expected.

Parents should be advised that a change in color may be expected as the condition progresses. This is especially the case with capillary malformation in the face in the context of Sturge-Weber syndrome. The color intensity often increases over the years, the color often changes from light red in childhood to dark red and finally blue-purple in adulthood. Owing to an increase of connective tissue and fatty tissue in the dermis and increasing dilation of the capillaries, additional skin thickening and soft tissue hyperplasia may occur in the area of the CM, especially on the face and lips. The smooth surface in childhood and adolescence may develop into irregular, sometimes nodular areas reminiscent of cobblestones and sometimes pronounced swelling of a lip, especially in adults with Sturge-Weber syndrome. However, these transformative changes of a capillary malformation are extremely rare outside the facial area; as a rule, these changes do not occur with capillary malformations in other locations.

In combined capillary malformation (especially with lymphatic or venous malformation components as in Klippel-Trénaunay syndrome), the formation of blood-tinged, small cutaneous vesicles filled with lymph may occur. These form small, frogspawn-like crusts and may secrete fluid and blood. Therapy by laser or sclerotherapy is then indicated.

The prognosis of both simple isolated and syndromal capillary malformations depends on building up a trusting doctor-patient or doctor-parent relationship early. Providing patients and their families with comprehensive information enables them to cope independently with skin changes that are often perceived as stigmatizing. This also includes participatory decision-making for or against invasive therapy, which can often significantly improve the prognosis of affected children and adults.