The Nevus Anemicus (NA) is a white, congenital lesion, relatively devoid of blood in which the skin's blood vessels are permanently vasoconstricted. The defect appears to be a result of increased local sensitivity to catecholamines. If the skin is grafted to another site, it retains its vasoconstriction, thus implying that the blood vessels themselves have an increased sensitivity to catecholamines as opposed to increased sympathetic stimulation.
A pale, hypopigmented and sharply demarcated, irregular patch is seen. The lesion occurs most commonly on the torso. The edge can be obliterated for a few seconds by compressing the skin with one's hand, thus verifying that it is blood and not melanin that causes the difference in color. Rubbing the edge will also accentuate the lesion as the surrounding skin becomes hyperemic.
Nevus anemicus appears more common in patients with neurofibromatosis. In one study, 51% of patients with NF-1 had a nevus anemicus compared with only 2% of control subjects. It may also be more common in tuberous sclerosis.
Phakomatosis pigmentovascularis IIb is NA along with nevus flammeus and a mongolian spot.
Bier anaemic spots, cyanosis with urticaria‐like eruption syndrome (BASCULE Syndrome) is a new dermatosis that Bessis and colleagues described in 2016 that is characterized by multiple urticaria‐like red papules on anemic white macules with a surrounding cyanotic background. BASCULE syndrome differs from Bier spots in that it also has urticaria‐like papules on the anemic macules.
No treatment is usually needed nor available. For the rare lesion on the face, makeup may be necessary.
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