BASCULE syndrome is an acronym for Bier anemic spots and cyanosis with an urticaria-like eruption. This vasomotor disorder is characterized by asymptomatic irregular white macules on a cyanotic background that appear after a few minutes of immobile standing, followed by the appearance of pruritic, orange-red papules superimposed on the white macules. The lesions are distributed on the legs and may also be seen on the arms or, rarely, on the trunk. They resolve with sitting, walking, or moving the affected limbs.
Patients ranging in age from 3 months to 19 years have been reported. The anemic spots are most likely caused by an exaggerated vasoconstrictive response of the microvasculature. The subsequent pruritic papules may be a paradoxical urticaria reaction; however, the exact mechanism is unknown.
Orthostatic hypotension and exercise-induced syncopal episodes have been noted in a single patient each.
BASCULE syndrome
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Synopsis
Codes
ICD10CM:
L98.8 – Other specified disorders of the skin and subcutaneous tissue
SNOMEDCT:
11263005 – Vascular disease of the skin
L98.8 – Other specified disorders of the skin and subcutaneous tissue
SNOMEDCT:
11263005 – Vascular disease of the skin
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Last Reviewed:08/10/2019
Last Updated:10/11/2022
Last Updated:10/11/2022
BASCULE syndrome