ANGIOSPASTIC MACULES (BIER SPOTS)
These were first described by Bier in 1898 and represent a pattern of vascular mottling consisting of white macular areas surrounded by a red to occasionally blue cyanotic background. Bier spots most commonly occur on the arms and legs of young adults (Fig. 106.17), but may also occur on the trunk. They are more noticeable in patients with lightly pigmented skin. Angiospastic macules may be induced by venous congestion and can be elicited by placing a limb in a dependent position or by placing a tourniquet around a limb. The spots disappear with limb elevation or release of the tourniquet. As with nevus anemicus, the border of the lesion becomes imperceptible with pressure (diascopy). The vascular pattern is thought to result from a benign physiological response consisting of vasoconstriction. Bier spots have also been associated with pregnancy, lymphedema, cryoglobulinemia, capillary malformationarteriovenous malformation syndrome (especially type 2 due to EPHB4 mutations), tuberous sclerosis complex, and aortic hypoplasia. BASCULE syndrome features Bier anemic spots, cyanosis (acral), and an urticarialike eruption in association with orthostatic intolerance.
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Fig. 106.17 Angiospastic macules (Bier spots).
of the donor site (donor dominance). No histologic abnormalities have been reported. No treatment is required or effective. Of note, nevus anemicus can co-exist with vascular malformations and is seen in up to half of patients with neurofibromatosis type 1.