PMC4283393_01
((a) and (b)) Appearance of the unilateral heliotrope rash along with Gottron's papules before and after 6 months of therapy, respectively. (c) Focal perifascicular atrophy was focally appreciated on light microscopy (H&E, x100). (d) Ultrastructural examination revealed areas with prominent myofibrillar disarray with scattered rod-like structures (single arrow) and cytoplasmic bodies (double arrows) (x15000).
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On examination he had purple discoloration and swelling around the left eye (Figure 1(a) before therapy and Figure 1(b) after therapy), Gottron's papules over the metacarpophalangeal (MCP) joints bilaterally, and muscle weakness with a grade of 3/5 in the proximal group and 4/5 in the distal group. Perifascicular atrophy was not a prominent feature, but it could be focally appreciated (Figure 1(c)). Ultrastructural examination revealed findings that were in keeping with light microscopy, including prominent myofibrillar disarray (Figure 1(d)).