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 Table of Contents  
Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 355-356

V sign in dermatomyositis

Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India

Date of Submission13-Sep-2021
Date of Decision30-Sep-2021
Date of Acceptance08-Oct-2021
Date of Web Publication29-Dec-2021

Correspondence Address:
Dr. Rashmi Roongta
Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, 244, A. J. C. Bose Road, Kolkata - 700 020, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/amhs.amhs_207_21

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V sign is a patchy or discrete macular erythema over the sun-exposed parts of the anterior neck and upper chest in patients with dermatomyositis (DM). It is one of the clinical hallmarks of DM and aids in diagnosis. It can be associated with telangiectasias and a shawl sign, which is a similar rash in the upper back, posterior neck, and shoulders.

Keywords: Cutaneous, dermatomyositis, V sign

How to cite this article:
Mondal S, Roongta R, Sinha D. V sign in dermatomyositis. Arch Med Health Sci 2021;9:355-6

How to cite this URL:
Mondal S, Roongta R, Sinha D. V sign in dermatomyositis. Arch Med Health Sci [serial online] 2021 [cited 2022 Aug 15];9:355-6. Available from: https://www.amhsjournal.org/text.asp?2021/9/2/355/334008

  Introduction Top

Dermatomyositis can have a range of cutaneous signs. It is sometimes diagnosed based on the cutaneous signs rather then muscle involvement. Here we discuss one of the common signs of the disease- the V sign.

  Case Report Top

A 36-year-old female woman attended the Rheumatology Clinic of Institute of Post Graduate Medical Education and Research, Kolkata, in August 2019 with a history of progressive proximal muscle weakness, polyarthralgia, and skin rash for the last 6 weeks. She had no history of fever, oral ulcers, or Raynaud's phenomenon. Examination revealed decreased power in neck flexors, shoulder, and hip girdle muscles, without any muscle atrophy. Dusky, macular, skin rash was noted over the anterior aspect of her chest and neck [“V” sign, [Figure 1]a], which was initially erythematous. Her serum glutamic pyruvic transaminase (134 U/l, normal <40 U/L), serum glutamic oxaloacetic transaminase (192 U/l, normal <40 U/L), and creatine phosphokinase level (2015 U/L, normal 26–190 U/L) were elevated. Serum antinuclear antibodies were positive (intensity: 3+, fine speckled pattern), and myositis profile showed anti-Ro-52 antibody positivity. Electromyogram showed a myopathic pattern [Figure 1]b. Muscle biopsy findings were consistent with a diagnosis of dermatomyositis (DM). There was no evidence of interstitial lung disease. She was treated with oral prednisolone 1 mg/kg/day with slow tapering along with methotrexate (15 mg/week) and sunlight protection. Symptomatic improvement was noted from 4 weeks. During her last follow-up at 6 months, there was no residual muscle weakness.
Figure 1: (a) Dusky macular rash over anterior neck and chest (“V” sign), (b) electromyogram showing small amplitude, short duration, polyphasic motor unit action potential with early recruitment consistent with myopathic pattern

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  Discussion Top

DM is a rare disease that belongs to idiopathic inflammatory myositis. Typical skin rashes[1] and progressive symmetric proximal muscle weakness, as seen in this patient, should alert a clinician to consider DM as a differential diagnosis. Cutaneous manifestations of DM are varied.[1] V sign is a discrete macular erythema over the anterior neck and chest and is commonly associated with anti-Mi2 antibody.[2],[3] Presence of a similar rash in the upper back, posterior neck, shoulders, and lateral aspect of arms is known an Shawl sign.[4] Violaceous rash over the lateral aspects of thighs is known as Holster sign.[5] The sun-protected areas, such as the submental region, are often spared. An association of malignancy has been reported with DM; consequently, a thorough evaluation for such should be done in diagnosed patients of DM.[6] Immunosuppressives are the mainstay in the management of DM.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol 1999;11:475-82.  Back to cited text no. 1
Dugan EM, Huber AM, Miller FW, Rider LG, International Myositis Assessment and Clinical Studies Group. Photoessay of the cutaneous manifestations of the idiopathic inflammatory myopathies. Dermatol Online J 2009;15:1.  Back to cited text no. 2
Love LA, Leff RL, Fraser DD, Targoff IN, Dalakas M, Plotz PH, et al. A new approach to the classification of idiopathic inflammatory myopathy: Myositis-specific autoantibodies define useful homogeneous patient groups. Medicine (Baltimore) 1991;70:360-74.  Back to cited text no. 3
Marvi U, Chung L, Fiorentino DF. Clinical presentation and evaluation of dermatomyositis. Indian J Dermatol 2012;57:375-81.  Back to cited text no. 4
[PUBMED]  [Full text]  
Sontheimer RD. Dermatomyositis: An overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin 2002;20:387-408.  Back to cited text no. 5
András C, Ponyi A, Constantin T, Csiki Z, Szekanecz E, Szodoray P, et al. Dermatomyositis and polymyositis associated with malignancy: A 21-year retrospective study. J Rheumatol 2008;35:438-44.  Back to cited text no. 6


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