There are irregularly shaped hyperpigmented macules along her lower extremities and abdomen with firm subcutaneous nodules scattered along her lateral lower extremities and hips. There are no bruises. No cranial nerve deficits are noted. Sensation is intact. Plantars are downgoing bilaterally.
Cerebellar function is intact.
Radiographs of her painful lower extremities are obtained. These radiographs demonstrate extensive soft tissue calcifications. The calcifications do not follow any particular vascular pattern and are not attached to bone. The pathophysiology of JDMS includes muscle lesions with a mix of degeneration and regeneration of muscle fibers, various muscle fiber sizes and perivascular lymphocytic infiltrates. There is also vasculitis of small arteries, venules, and capillaries of the skin, muscle and GI tract.