BASCULE syndrome: Is something brewing with Bier spots?
By Warren R. Heymann, MD, FAAD
Sept. 7, 2022
Vol. 4, No. 36
I have never paid too much attention to Bier spots, other than reassuring patients that they are of no concern. I may have been too cavalier by not probing further.
Classical Bier spots (BS, also referred to as Bier anemic spots, BAS in this commentary) were first described by the German surgeon Augustus Bier in 1898. BS are multiple, asymptomatic, pale, and irregularly shaped “anemic” macules appearing on a cyanotic background. BS may be seen at any age, but mainly affect young adults (20 – 40 years old) of both genders. BS are caused by excessive arteriolar vasoconstriction induced by venous stasis-associated hypoxia or a failure of physiological vasoconstriction, depending on the clinical circumstances. (1)
In 2016, Bessis et al described 4 cases of a possible “new” entity, which they named BASCULE syndrome (Bier Anemic Spots, Cyanosis with Urticaria-Like Eruption). The index case, similar to many subsequent reports, follows: “A 14-year-old girl with no relevant medical history was admitted for the evaluation of disseminated pruritic cutaneous lesions, which had appeared 2 years previously and were progressively worsening. The lesions developed after 1–2 min in a standing and immobile position, were associated with disabling pruritus and were relieved by sitting, walking around or raising the limbs. Cutaneous examination after 1–2 min in a standing position revealed multiple irregular white spots, which stood out from a marked cyanotic background. During the next few minutes, pruritic red–orange papules progressively appeared. These red–orange papules were located on the white macules, extending from their centre to cover most of their surface. The lesions faded completely within minutes of lying down.
Histopathological examination of a biopsy specimen from a papule showed multiple dilated capillaries of the upper dermis with rare interstitial and scattered eosinophils and mast cells. Treatment with desloratadine (10 mg per day for 7 days) resulted in partial improvement of the pruritus, whereas the cutaneous lesions remained unchanged.” The authors differentiated BASCULE syndrome from typical BS by the following criteria: 1) the invariable presence of cyanosis; 2) the occurrence of an urticaria-like eruption characterized by red–orange macules and papules always located on the central part of the anemic macules; 3) the variable presence of pruritus (two of four cases) and tenderness (one of four cases) of the affected areas; and 4) partial or diffuse edema of the involved limbs (two of four cases). (2)
Since the initial report by Bessis et al, BASCULE syndrome had been reported in infants with painful lesions (3), on the trunk and upper limbs (4), with exercise-related syncopal episodes (5), first-degree heart block (6), and a prolonged QT interval. (7)
McGibbon, upon reading the initial report by Bessis et al, questioned whether BASCULE syndrome was related to orthostatic acrocyanosis, suggesting that a tilt table be used to confirm or deny an association with postural orthostatic tachycardia syndrome (POTS). (8) In their report of 2 new cases of BASCULE syndrome, Ramírez-Lluch et al note that anomalies in the autonomic nervous system such as POTS, initial orthostatic hypotension and orthostatic symptoms without tachycardia have been described in association with BASCULE syndrome and conditions of orthostatic intolerance should be ruled out, even if there are no associated symptoms. (9)
The etiology of BASCULE syndrome remains to be defined. Dysautonomia and vasoconstrictive responses related to venous return, may be associated with localized tissue hypoxia, leading to mast cell activation, tissue eosinophilia (in some cases), and histamine release. (9, 10) In reviewing the literature, antihistamines are occasionally beneficial. The natural history of BASCULE syndrome is unpredictable, although it may spontaneously regress. (11)
In summary, as stated eloquently by El Nemnon et al: “BASCULE syndrome is a benign vasomotor dermatosis characterized by typical clinical features including erythrocyanosis, generally of the lower extremities, with associated Bier spots that evolve quickly into pruritic urticarial-like papules. Symptoms classically occur with standing and resolve with lying down. The diagnosis is clinical, as histopathology is non-specific. However, patients suspected of having BASCULE syndrome, even if otherwise asymptomatic, should undergo evaluation for chronic orthostatic intolerance. We hypothesize that the association of these conditions is likely not random, but may reflect a common underlying pathology, a benign vasomotor disease.” (7)
In my next encounter with a patient with Bier spots, I will look closer and ask some pertinent questions before offering carte blanche reassurance.
Point to Remember: BASCULE syndrome may be underdiagnosed. Ask about any associated urticarial lesions and symptoms related to orthostatic intolerance the next time you see a patient with Bier spots.
Our expert’s viewpoint
Barrett Zlotoff, MD, FAAD
Residency Program Director for Dermatology
Director Pediatric Dermatology
University of Virginia Health Science Center
“You learn from a conglomeration of the incredible past — whatever experience gotten in any way whatsoever.” – Bob Dylan
“You need something to open up a new door, to show you something you seen before but overlooked a hundred times or more.” – Bob Dylan
These Dylan quotes encompass the inherent contradiction in our approach to the medical “body of knowledge.” The condition or disease may not enter our collective mind until formally expressed, but the reality of its features is there, waiting to be described into existence. The tension of this dichotomy shapes diagnostic and therapeutic approaches for patients who suffer from unusual or unexplained health phenomena.
The spectrum of postural orthostatic tachycardia (POTS) and BASCULE syndrome currently fall into a realm of poorly understood conditions related to dysfunction of autonomic systems. Transient hyperemia, flushing, urticarial papules, pruritus, and dysthesias are theoretically associated with acute vasomotor changes. (12) It seems likely that there are a wide variety of conditions rooted in aberrant control of neurovascular dysfunction. Erythromelalgia is an example of one such condition where pathophysiologic clarity has been provided for a subset of patients. Genetic mutations in voltage gated sodium channels modulating peripheral nerve excitability have provided a rationale for therapeutic intervention in primary inherited erythromelalgia. (13)
With time, we will gain a better understanding of the neurologic and vasomotor underpinnings of erythromelalgia, reflex sympathetic dystrophy, POTS and BASCULE syndrome. Perhaps we will discover that vasomotor issues are key to the pathogenesis of other mysterious conditions such as Gardner-Diamond syndrome and hematohidrosis. (14) In the meantime — signs and symptoms manifested in the cutaneous vasculature are a valuable window into how dysfunction in these systems can manifest in the skin and beyond. The curious clinician and bench researcher will keep his eyes and mind open and remember:
“People have a hard time accepting anything that overwhelms them.” – Bob Dylan
Kluger N, Bessis D. Bier's spots. J Eur Acad Dermatol Venereol. 2019 Feb;33(2):e78-e79. doi: 10.1111/jdv.15222. Epub 2018 Sep 19. PMID: 30160813.
Bessis D, Jeziorski É, Rigau V, Pralong P, Pallure V. Bier anaemic spots, cyanosis with urticaria-like eruption (BASCULE) syndrome: a new entity? Br J Dermatol. 2016 Jul;175(1):218-20. doi: 10.1111/bjd.14589. Epub 2016 May 18. PMID: 27016170.
Piroth M, Guenot LM, Barbarot S. Acute painful blue-white-red rash of the limbs: BASCULE syndrome. Int J Dermatol. 2020 Jun;59(6):749-750. doi: 10.1111/ijd.14799. Epub 2020 Feb 11. PMID: 32043279.
Jiménez-Gallo D, Collantes-Rodríguez C, Ossorio-García L, Báez-Perea JM, Linares-Barrios M. Bier anaemic spots, cyanosis with urticaria-like eruption (BASCULE) syndrome on trunk and upper limbs. Pediatr Dermatol. 2018 Sep;35(5):e313-e315. doi: 10.1111/pde.13558. Epub 2018 Jun 26. PMID: 29943837.
Martín JM, Torres C, Cordero P, Sánchez S, Terradez L, Ramón D. Bascule syndrome associated with syncopal episodes. Pediatr Dermatol. 2018 Mar;35(2):e142-e143. doi: 10.1111/pde.13425. Epub 2018 Feb 13. PMID: 29436000.
Guillen-Climent S, Porcar Saura S, Pons Benavent M, García-Vázquez A, Martín JM. BASCULE syndrome associated with presyncopal episodes and first-degree atrioventricular block. Int J Dermatol. 2021 Apr 2. doi: 10.1111/ijd.15568. Epub ahead of print. PMID: 33797752.
El Nemnom P, Lauwerys B, Marot L, Tennstedt D, Dekeuleneer V. Bier anemic spots, cyanosis, and urticaria-like eruption (BASCULE) syndrome: Report of two new cases and literature review. Pediatr Dermatol. 2020 Sep;37(5):864-867. doi: 10.1111/pde.14213. Epub 2020 Jul 9. PMID: 32648305.
McGibbon DH. BASCULE syndrome: old wine in new bottles? Br J Dermatol. 2016 Nov;175(5):1109-1110. doi: 10.1111/bjd.14898. Epub 2016 Sep 14. PMID: 27470123.
Ramírez-Lluch M, Torre-Castro J, López-Balboa P, Martos-Cabrera L, Mateos-Mayo A, LoboBailón F, Hernández-Martín Á, Torrelo A. Bier anaemic spots, cyanosis and urticaria-like eruption syndrome: Report of two new cases. Australas J Dermatol. 2021 Aug;62(3):428-433. doi: 10.1111/ajd.13564. Epub 2021 Mar 4. PMID: 33660844.
Cunningham L, Dvorakova V, Browne F, Irvine AD. High-dose bilastine for the treatment of BASCULE syndrome. Clin Exp Dermatol. 2021 Mar;46(2):357-358. doi: 10.1111/ced.14377. Epub 2020 Aug 26. PMID: 32658312.
Barbé J, Escobar G, Bursztejn AC. Bier anemic spots, cyanosis with urticaria-like eruption (BASCULE) syndrome: a simple benign pathology, but probably underdiagnosed. Int J Dermatol. 2021 Sep;60(9):1163-1164. doi: 10.1111/ijd.15452. Epub 2021 Feb 20. PMID: 33611787.
Martin, S. M., & Zlotoff, B. J. (2021). A 15-year-old girl with transient patches of erythema. Pediatric dermatology, 38(4), 937-938.
Drenth, J. P., te Morsche, R. H., Guillet, G., Taieb, A., Kirby, R. L., & Jansen, J. B. (2005). SCN9A mutations define primary erythermalgia as a neuropathic disorder of voltage gated sodium channels. Journal of Investigative Dermatology, 124(6), 1333-1338.
Shafique, D. A., Hickman, A. W., Thorne, A., Elwood, H. R., & Zlotoff, B. J. (2021). Pediatric hematidrosis–A case report and review of the literature and pathogenesis. Pediatric Dermatology.
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