10.5826/dpc.1304a224
text/xml
2160-9381
3208
en
Adrian Imbernon-Moya
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Familial BASCULE Syndrome In Two Siblings
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Image Letter
2023-10-31
2023-02-02
2023-10-31
2024-01-15
e2023224
Dermatol Pract Concept
Adrian Imbernon-Moya
2023/10/31
10.5826/dpc.1304a224
e2023224
Department of Dermatology, Hospital Universitario Severo Ochoa, Leganés, Madrid, Spain
2160-9381
en
e2023224
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;60(9):1163-1164. DOI: 10.1111/ijd.15452. PMID: 33611787.
Familial BASCULE Syndrome In Two Siblings
https://dpcj.org/index.php/dpc/article/download/3208/2387
           Familial BASCULE Syndrome in Two Siblings
                Belén Romero-Jiménez1, Catalina Axpe-Gil1, Jorge Román-Sainz1,
          Fernando Gruber-Velasco1, Marcela Martínez-Pérez1, Adrián Imbernón-Moya1


1 Department of Dermatology, Hospital Universitario Severo Ochoa, Leganés, Madrid, Spain



Citation: Romero-Jiménez B, Axpe-Gil C, Romàn-Sainz J, Gruber-Velasco F, Martìnez-Pérez M, Imbernón-Moya A. Familial BASCULE
Syndrome In Two Siblings. Dermatol Pract Concept. 2023;13(4):e2023224. DOI: https://doi.org/10.5826/dpc.1304a224
Accepted: April 25, 2023; Published: October 2023
Copyright: ©2023 Romero-Jiménez et al. This is an open-access article distributed under the terms of the Creative Commons
Attribution-NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted
noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: None.
Authorship: All authors have contributed significantly to this publication.

Corresponding Author: Belén Romero-Jiménez, Department of Dermatology, Hospital Universitario Severo Ochoa, Leganés, Madrid, Spain.
ORCID https://orcid.org/0000-0002-1557-9284 E-Mail: belenromjim96@yahoo.es




Case Presentation                                                        sitting or lifting her legs. They had no history of cardiovas-
                                                                         cular disorders.
We present two siblings, a girl and a boy of 12 and 15 years                  Laboratory tests with TSH and autoimmunity were
respectively, who present asymptomatic multiple symmetri-                performed without anomalies. Cardiological abnormalities
cal hypopigmented macules with central telangiectasias sur-              were ruled out in both siblings. The diagnosis was familial
rounded by a background of erythrocyanosis on the dorsum                 BASCULE syndrome. None of the parents presented similar
of their hands since childhood (Figure 1, A and B). The le-              lesions in childhood or adolescence.
sions appeared after 1-2 minutes of keeping down their arms
and disappeared when they were elevated. The girl also had
similar lesions on her lower extremities which appeared after
a few minutes in standing position and disappeared when




Image Letter | Dermatol Pract Concept. 2023;13(4):e2023224                                                                           1
      Figure 1. (A) Clinical image of a 12-year-old girl with hypopigmented macules with central telangiectasias in a background
      of erythrocyanosis on the dorsum of her hands. (B) Clinical image of a 15-year-old boy with hypopigmented macules on the
      dorsum of his hands.




Teaching Point                                                       References
BASCULE syndrome (Bier anemic spots, cyanosis and                     1. El Nemnom P, Lauwerys B, Marot L, Tennstedt D, Dekeule-
                                                                         neer V. Bier anemic spots, cyanosis, and urticaria-like eruption
urticaria-like eruption) is a benign vasomotor dermatosis
                                                                         (BASCULE) syndrome: Report of two new cases and literature
that typically affects the lower extremities of children and
                                                                         review. Pediatr Dermatol. 2020;37(5):864-867. DOI: 10.1111
adolescents [1,2]. No familial cases have been reported.                 /pde.14213. PMID: 32648305.
The diagnosis is mostly clinical and biopsy is usually not            2. Barbé J, Escobar G, Bursztejn AC. Bier anemic spots, cyanosis
­necessary [2]. It is recommended to perform a blood test                with urticaria-like eruption (BASCULE) syndrome: a simple
with TSH and autoimmunity and refer to cardiology to rule                benign pathology, but probably underdiagnosed. Int J Derma-
                                                                         tol. 2021;60(9):1163-1164. DOI: 10.1111/ijd.15452. PMID:
out underlying heart disease. Treatment with antihistamines
                                                                         33611787.
is not effective. The prognosis is favorable with resolution of
the lesions with pubertal growth [1,2].
    Herein, we present a BASCULE syndrome in two mem-
bers of the same family, which may suggest the possibility of
having a hereditary component.




2                                                                           Image Letter | Dermatol Pract Concept. 2023;13(4):e2023224
