Acute onset dermatomyositis with NXP-2 antibodies after Sars-CoV-2 vaccination in a 20-year-old female

Dermatomyositis is an autoimmune connective tissue disease with variable cutaneous and systemic manifestations. While multiple cutaneous reactions to SARS-CoV-2 vaccination have been described, vaccine-associated emergence of dermatomyositis has rarely been reported. Cases of deterioration in pre-existing connective tissue diseases have been described following both vaccination and COVID-19 infection. A potential mechanism of immune system activation and dysregulation has been suggested. This case describes acute onset of dermatomyositis in a young female, presenting with classic cutaneous features including Gottron papules, Holster sign and proximal nail fold erythema, accompanied with myalgia and fatigue. Laboratory findings of elevated creatine kinase and myositis-specific autoantibodies confirmed the diagnosis, with supportive histological findings of necrotic keratinocytes at the dermoepidermal junction. In keeping with the typical progression of dermatomyositis with NXP-2 autoantibodies, cutaneous findings resolved rapidly, while systemic manifestations slowly improved with treatment. Thorough investigations for underlying malignancy were completed. This case adds to the limited medical literature surrounding these cases and further highlights the importance of consideration of SARS-CoV-2 vaccination in the immunologic pathophysiology of autoimmune connective tissue diseases.