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N Goel, Sankar J, A Kumar, A Chandwani, H Jain, K Sivasami, P Saxena, V Vasdev, MDA5 dermatomyositis—skin says it all, QJM: An International Journal of Medicine, Volume 117, Issue 12, December 2024, Pages 868–869, https://doi.org/10.1093/qjmed/hcae153
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A 44-year-old male presented with history of periorbital rash and rash over palms and soles of 3 months duration. It was associated with proximal muscle weakness of 1 month duration. He also complained of shortness of breath of 15 days duration. On examination, there were multiple papules on flexor aspect of hands known as ‘Inverse Gottron sign’ (Figure 1a), periorbital rash called heliotrope rash (Figure 1c), multiple cutaneous ulcers over hands (Figure 1d) and hyperkeratosis of feet and soles known as ‘Hikers feet’1 (Figure 1e). He had weakness of proximal muscles of upper limb and lower limb. Chest examination revealed bilateral fine crepitations at the base of lungs. Hematological investigations revealed transaminitis (AST>ALT) with markedly raised creatinine kinase levels. He also underwent MRI bilateral thighs which showed T2 hyperintensities suggestive of inflammatory myositis. His nail fold capillaroscopy showed dilated capillaries (Figure 1f). He also underwent High resolution computed tomography of chest revealed extensive interstitial lung disease (Figure 1b). Based on typical cutaneous findings, myositis, ILD and anti MDA5 antibody positivity on myositis profile, he was diagnosed as case of subset of myositis known as MDA5 dermatomyositis. He was started on high dose oral steroids, monthly cyclophosphamide pulses and tacrolimus.
