|IMAGES IN RHEUMATOLOGY
|Ahead of print publication
Inverse Gottron's papules
Arun C Inamadar, Keshavmurthy A Adya, Ajit B Janagond
Department of Dermatology Venereology and Leprosy, Shri B M Patil Medical College Hospital and Research Centre, BLDE (Deemed to be University), Vijayapur, Karnataka, India
|Date of Submission||11-Apr-2022|
|Date of Acceptance||27-May-2022|
|Date of Web Publication||20-Sep-2022|
Arun C Inamadar,
Department of Dermatology Venereology and Leprosy, Shri B M Patil Medical College Hospital and Research Centre, BLDE (Deemed to be University), Vijayapur, Karnataka
Source of Support: None, Conflict of Interest: None
Keywords: Anti-MDA5 autoantibodies, dermatomyositis, Gottron's papules
A 40-year-old woman presented with facial edema and painful skin lesions involving the hands. On examination, heliotrope rash [Figure 1]a, hyperkeratotic ulcerated papules on the knuckles [Figure 1]b and erythematous, flat-to-raised keratotic lesions involving the palmar aspect of the interphalangeal joints [Figure 1]c were noted. A diagnosis of dermatomyositis (DM) was made with clinical features and raised muscle enzymes.
|Figure 1: Clinical photograph showing heliotrope with periorbital edema (a) and Gottron's sign over dorsal aspect of hand (b), flat-to-raised lesions involving palmar aspect of the fingers in a distribution “inverse” of the classic Gottron's papules/sign (c, black arrow) and fingertip scars suggestive of healed ulcers (c, white arrow)|
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Gottron's papules are pathognomonic of DM. Inverse Gottron's sign/papules [Figure 1]c, due to vasculopathy are a rare but specific cutaneous finding in DM, usually associated with digital ulceration, interstitial lung disease, and anti-MDA5 autoantibodies.,,
Anti-MDA5-positive DM occurs in 7.2% of adults and 9.7% of children, respectively, in the Indian subcontinent. Cutaneous vasculopathic features such as cutaneous ulceration, and periorbital edema as seen in the index may indicate a worse poor prognosis in adults with anti-MDA-5 DM.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Quinter SD, Chiu YE, Lyon VB, Holland KE, Ruggeri SY, Drolet BA. Inverse Gottron's papules: An unusual cutaneous manifestation of juvenile dermatomyositis. Pediatr Dermatol 2012;29:641-4.
Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): A retrospective study. J Am Acad Dermatol 2011;65:25-34.
Jain S, Sharma A. Inverse Gottron's sign in anti-MDA5 antibody- associated dermatomyositis. Rheumatology (Oxford) 2020;59:10.
Dunga SK, Kavadichanda C, Gupta L, Naveen R, Agarwal V, Negi VS. Disease characteristics and clinical outcomes of adults and children with anti-MDA-5 antibody-associated myositis: a prospective observational bicentric study. Rheumatol Int. 2021. doi: 10.1007/s00296-021-04897-1. Epub ahead of print. PMID: 34050793.