Orthostatic hypotension revealed by BASCULE syndrome

Bier anemic spots, cyanosis, and urticaria‐like eruption (BASCULE) syndrome: Report of two new cases and literature review

“…These histological findings were generally non‐specific, with only a few dilated capillaries in the upper dermis without any specific infiltrate noted in the majority of cases. An eosinophilic infiltrate was reported in two cases 1‐4 . The findings thus resemble the non‐specific findings of common urticaria: a sparse perivascular and interstitial mixed inflammatory infiltrate with edema in the upper dermis 5 .…”

“…While not observed in our cases, it is noteworthy that three published reports of BASCULE syndrome have occurred in association with different forms of chronic orthostatic intolerance; POTS, orthostatic hypotension, and syncopal episodes, respectively 3,4,7 . Six of the nine previously reported cases plus our two new cases were tested for POTS with only one positive outcome.…”

“…Pharmacological treatments, including fludrocortisone, propranolol, desmopressin, midodrine, pyridostigmine, clonidine, or ivabradine may be administered as second‐line therapy 8‐10 . In our two cases and in published cases, antihistamine treatment was ineffective 1‐4 . The prognosis of BASCULE syndrome is otherwise favorable, with skin lesions typically resolving after months to years.…”

“…Danescu et al reported a case of BASCULE syndrome associated with chronic orthostatic intolerance that presented as initial orthostatic hypotension, the most common form of orthostatic intolerance in young people 4,10 . Initial orthostatic hypotension is defined as a 40 mm Hg decrease in systolic BP and/or >20 mm Hg decrease in diastolic BP within 15 seconds of standing.…”

“…The initial BP drop results from a mismatch between increased cardiac output and decreased systemic vascular resistance due to a failure of the autonomic nervous system. The BP rebound, in contrast, is due to sympathetic vasoconstriction in the peripheral vasculature 4,8,10 . BASCULE syndrome patients may also suffer from presyncopal or syncopal episodes which is the third form of orthostatic intolerance, without evidence of orthostatic tachycardia or orthostatic hypotension, such as described in Martín et al 3,10 …”