Anti-MDA5 dermatomyositis after COVID-19 vaccination: a case-based review

Abstract

Anti-MDA5 (Melanoma differentiation-associated protein 5) myositis is a rare subtype of dermatomyositis (DM) characterized by distinct ulcerative, erythematous cutaneous lesions and a high risk of rapidly progressive interstitial lung disease (RP-ILD). It has been shown that SARS-CoV-2 (COVID-19) replicates rapidly in lung and skin epithelial cells, which is sensed by the cytosolic RNA-sensor MDA5. MDA5 then triggers type 1 interferon (IFN) production, and thus downstream inflammatory mediators (EMBO J 40(15):e107826, 2021); (J Virol, 2021, https://doi.org/10.1128/JVI.00862-21 ); (Cell Rep 34(2):108628, 2021); (Sci Rep 11(1):13638, 2021); (Trends Microbiol 27(1):75-85, 2019). It has also been shown that MDA5 is triggered by the mRNA COVID-19 vaccine with resultant activated dendritic cells (Nat Rev Immunol 21(4):195-197, 2021). Our literature review identified one reported case of MDA5-DM from the COVID-19 vaccine (Chest J, 2021, https://doi.org/10.1016/j.chest.2021.07.646 ). We present six additional cases of MDA5-DM that developed shortly after the administration of different kinds of COVID-19 vaccines. A review of other similar cases of myositis developing from the COVID-19 vaccine was also done. We aim to explore and discuss the evidence around recent speculations of a possible relation of MDA5-DM to COVID-19 infection and vaccine. The importance of vaccination during a worldwide pandemic should be maintained and our findings are not intended to discourage individuals from receiving the COVID-19 vaccine.

Keywords: COVID-19 vaccine; Dermatomyositis; MDA5.

Fig. 1

Search strategy used for case-based review

Fig. 2

Case 1. Gottron’s papules seen over bilateral MCPs (A) with palmar erythema, and “reverse Gottron’s lesions” over PIP and DIP palmar creases (B). Diffuse edema of entire right arm extending to hand (C). Erosive retiform lesions over back (D). CT thorax showing bilateral ground opacities with nodular consolidate opacities that developed rapidly over a few days (E). CT of right upper extremity showing diffuse cutaneous thickening with circumferential subcutaneous fat stranding and soft tissue swelling (F)

Fig. 3

Case 2. Ulcerative lesion on right ear (A) and numerous papules seen over left antihelix (B). Distal finger with erythematous maculopapular lesion. (C). CT thorax showing ground glass opacities in bilateral lower lobes (D). Several ulcerative lesions seen on medial right foot and ankle (E). Ichthyosis over left forearm (F)

Fig. 4

Case 3. Significant facial and lower eyelid edema bilaterally with overlying erythema (heliotrope rash) (A). Erythematous lesions over MCPs (Gottron’s papules) with periungal erythema bilaterally (B). Erythematous rash over forearm (C). Initial CT thorax showing ground glass and reticular interstitial infiltrates in peripheral lobes (D). Repeat CT thorax 5 months later showing progression with patchy bilateral consolidations, ground glass opacities, and “Atoll” sign (central GGO surrounded by circumferential consolidation) (E)

Fig. 5

Case 4. A Erythema and scaling of entire scalp, forehead, cheeks, nose, with (B) V sign over chest. C Periorbital heliotrope rash. D Gottron’s sign overlying MCP and IP joints of hands. E Proximal nailfold dilated capillaries with F ragged cuticles and periungual erythema

Fig. 6

Case 5. Bilateral dorsal PIPs and MCPs with erythematous, ulcerative lesions (A). Reverse Gottron’s lesions on bilateral palms and over PIP and DIP palmar creases (B). Erythematous rash overlying lateral thigh (Holster sign) (C). Erythematous macular rash over upper back (Shawl sign) (D). High resolution CT of lungs showing organizing pneumonia pattern concerning for progressive ILD (E)

Fig. 7

Case 6. A Sunburn sign with bright red erythema over the forehead and cheeks and heliotrope rash. B V-sign. C Erythematous maculopapular lesions over the MCPs (Gottron’s papules) and periungual erythema