Bascule syndrome associated with syncopal episodes

Abstract

Bascule syndrome is a recently described benign vasomotor dermatosis characterized by Bier anemic spots, cyanosis, and urticaria-like eruption. We report a case of a 13-year-old girl with cutaneous lesions consistent with Bascule syndrome who had had three exercise-related syncopal episodes. It would be recommended to exclude orthostatic intolerance or postural orthostatic tachycardia syndrome when evaluating patients with 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.…”

“…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 …”

Bier anaemic spots, cyanosis with urticaria‐like eruption (BASCULE) syndrome on trunk and upper limbs

“…Bier anaemic spots, cyanosis with urticaria‐like eruption syndrome is a new dermatosis that Bessis and colleagues described in 2016 (Table ) that is characterized by multiple urticaria‐like red papules on anemic white macules with a surrounding cyanotic background. These variably pruritic lesions are mainly located on the lower limbs .…”