Ulcerative Gottron: A sinister sign in dermatomyositis

A 45 yr old male† presented to the department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education & Research, Puducherry, India, in May 2018 with fever, polyarthritis and rapidly progressive dyspnoea over seven months. One month prior to presentation, the patient noticed bilateral ulcerative lesions over the metacarpophalangeal joints, indicating ulcerative Gottron (Figure A) along with roughening of the radial border of the fingers, suggestive of Mechanic's hands. High-resolution computed tomography of the thorax suggested fibrotic non-specific interstitial pneumonitis (NSIP) pattern of interstitial lung disease (ILD) (Figure B). Immunological workup was positive for anti-MDA-5 (melanoma differentiation associated protein-5) antibody. Diagnosis of rapidly progressive ILD (RP-ILD) with amyopathic dermatomyositis was considered, and treatment with high-dose steroids, intravenous immunoglobulin, cyclophosphamide and cyclosporine was initiated. Due to lack of response to the immunosuppressant, the patient developed pneumomediastinum (Figure C) leading to demise. Post-mortem lung biopsy revealed interstitial pneumonia without evidence of infection (Figure D). The presence of ulcerative Gottron should alert physicians for the possibility of RP-ILD associated with MDA-5, requiring aggressive immunosuppression at early stages itself.

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(A) Bilateral ulcerative Gottron lesions on the second metacarpophalangeal joints (red arrow). (B) High-resolution computed tomography thorax, axial section. Diffuse intra-lobular septal thickening in both lung fields (orange arrows). (C) Chest X-ray, pneumomediastinum (blue arrow) and subcutaneous emphysema (blue arrow). (D) Lung and respiratory mucosa with underlying interstitial fibrosis (black arrow) (H and E, ×40).

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