Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Net Letter
2018:84:5;645-645
doi: 10.4103/ijdvl.IJDVL_915_16
PMID: 29098991

Angiospastic macules of Bier: A cause of mottled skin

Cesar Daniel Villarreal-Villarreal, Juan Carlos Robles-Mendez, Angel Eduardo Guerra-Vazquez, Jorge Ocampo-Candiani
 Department of Dermatology, “Dr. José E. González” University Hospital of the School of Medicine of the “Universidad Autónoma de Nuevo León,” Monterrey, Mexico

Correspondence Address:
Jorge Ocampo-Candiani
Department of Dermatology, “Dr. José E. González” University Hospital of the School of Medicine of the “Universidad Autónoma de Nuevo León,” Monterrey 64610
Mexico
Published: 03-Nov-2017
How to cite this article:
Villarreal-Villarreal CD, Robles-Mendez JC, Guerra-Vazquez AE, Ocampo-Candiani J. Angiospastic macules of Bier: A cause of mottled skin. Indian J Dermatol Venereol Leprol 2018;84:645
Copyright: (C)2018 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

A 12-year-old girl with no past relevant medical history was referred to our Department of Dermatology at the university hospital “Dr. José E. González” Monterrey, México, with a 5-month history of asymptomatic white spots on her limbs which were first noticed on her legs and later on her arms. She recalled exercise and standing for a long time as triggering factors. The patient gave a history of malar erythema exacerbated with sun exposure and arthralgias of her knees. On physical exploration, oral ulcers and scarring alopecia were absent and she denied systemic symptoms, such as tachycardia, fever, weight loss and malaise.

Dermatologic examination revealed irregular hypopigmented macules surrounded by blanching diffuse erythema which were accentuated on orthostasis. The lesions disappeared when the limbs were raised and by applying pressure [Figure - 1] and [Figure - 2].

Figure 1: Irregular hypopigmented macules with bilateral distribution, surrounded by diffuse erythema on lower extremities
Figure 2: Lateral view of the patients lower extremities. Hypopigmented macules are noticed, giving the appearance of mottled skin

Skin biopsy with hematoxylin and eosin stain showed papillary dermal edema and dilated capillaries. Routine laboratory tests, including a complete blood count, blood chemistry including double-stranded deoxyribonucleic acid, Coombs test, C3, C4 and urine test, were within normal range. Although antinuclear antibodies were positive(1:160), the patient did not fulfill the criteria of systemic lupus erythematosus. Diagnosis of idiopathic Bier spots was made based on the patient's clinical features. The patient did not undergo treatment.

Angiospastic macules, speckled mottling of the limbs or Bier spots, received their name from the German surgeon Dr. Augustus Bier, who in 1898 described this entity by making a vascular experiment. He applied a tourniquet and occluded the blood supply of the arm. Bier spots were induced and disappeared once the blood supply was restored.

Bier spots are an abnormal and transient vascular response that occur with vasodilatation and vasoconstriction. They are mostly reported in patients between 20 and 40 years (range, 15–75 years of age) of age and men outnumber women with a 2:1 ratio.[1] It is proposed that an exaggerated vasospastic response occurs secondary to venous hypertension, arterial constriction or a lack of blood supply. Usually reported triggers are changes of position of the extremities (orthostasis) or cooler environments and hypoxia.[2]

Clinically, Bier spots are recognized as asymptomatic, multiple, small (5–10 mm) hypochromic macules with irregular shape and distribution, surrounded by blanching diffuse erythema. They are commonly seen on the extensor surface of extremities and even sometimes in a generalized distribution.[1] Diagnosis can be made easily through physical examination as these pale macules appear with orthostatic position and typically, they disappear by applying pressure on the surrounding skin or with elevation of the limbs, as was in our case.

Differential diagnoses include hypopigmentation disorders such as vitiligo, pityriasis versicolor, postinflammatory hypopigmentation and nevus depigmentosus which do not disappear neither by applying pressure nor elevating the extremities, as well as nevus anemicus which is a congenital and permanent vascular anomaly that usually appears on the trunk. All of these were ruled out by physical examination.[3]

Bier spots typically show no abnormalities on laboratory tests or skin biopsy. Although patients are usually healthy, it has been reported in different conditions, such as pregnancy, scleroderma, aortic hypoplasia and coarctation, as well as in autoimmune diseases, such as alopecia areata, lichen planus and cryoglobulinemia.[1] It is important to remember that, although positive (1:160) anti - nuclear antibody can be positive in upto 5% of general population, antinuclear antibody may develop years, if not decades, before the symptoms of autoimmune disease.[4],[5] After1-year follow-up, our patient had neither fulfilled the diagnostic criteria of systemic lupus erythematosus nor developed any systemic signs of autoimmune diseases.

Limitations in our article are that we only presented one patient with a short follow-up course (1 year). Another limitation is the fact that information about this entity is scarce as it is seldom reported and only some articles about this entity exist limiting our pool of references.

In conclusion, Bier spots represent an idiopathic and benign entity in the majority of cases and treatment is not needed as they tend to resolve spontaneously. Nevertheless, in some cases, systemic disease should be ruled out since they could be the first sign of a systemic disease. Reassuring the patient of the physiological and benign nature of this entity is the mainstay of treatment.

Acknowledgment

We thank Dr. Sergio Lozano for the review of this article.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References
1.
Fan YM, Yang YP, Li W, Li SF. Bier spots: Six case reports. J Am AcadDermatol 2009;61:e11-2.
[Google Scholar]
2.
Peyrot I, Boulinguez S, Sparsa A, Le Meur Y, Bonnetblanc JM, Bedane C. Bier's white spots associated with scleroderma renal crisis. ClinExpDermatol 2007;32:165-7.
[Google Scholar]
3.
Cabanillas M, Suárez-Amor O, Loureiro M, Ginarte M, Toribio J. Bier's spots in association with hypoplasia of the aorta. Dermatology 2007;215:166-7.
[Google Scholar]
4.
Arbuckle MR, McClain MT, Rubertone MV, Scofield RH, Dennis GJ, James JA, et al. Development of autoantibodies before the clinical onset of systemic lupus erythematosus. N Engl J Med 2003;349:1526-33.
[Google Scholar]
5.
Mahler M, Ngo JT, Schulte-Pelkum J, Luettich T, Fritzler MJ. Limited reliability of the indirect immunofluorescence technique for the detection of anti-Rib-P antibodies. Arthritis Res Ther 2008;10:R131.
[Google Scholar]

Fulltext Views
557

PDF downloads
393
Show Sections