Not just eosinophilic fasciitis

Abstract

This case report describes a rare case of progressive muscle weakness in a patient treated for eosinophilic fasciitis (EF) for many years before being diagnosed with a second autoimmune disease: dermatomyositis. Our case is a report of a 65-year-old male diagnosed with eosinophilic fasciitis 7 years before being evaluated in our service at Mayo Clinic in Jacksonville, Florida, due to progressive muscle weakness despite the chronic treatment with methotrexate. Contrast-enhanced magnetic resonance imaging of the lower extremity showed enhancement throughout the thigh musculature, which led us to pursue biopsies of the fascia and muscle in order to confirm the diagnosis of EF associated with myopathy. This case illustrates the need to consider the possibility of myopathy in patients diagnosed with EF whenever muscle weakness is more prominent than expected.

Keywords: CDC – Center for Disease Control and Prevention; DM – Dermatomyositis; EF – Eosinophilic fasciitis; EMS – Eosinophilia-myalgia syndrome; MRI – Magnetic Resonance Imaging; MTX – Methotrexate; dermatomyositis; eosinophilia-myalgia syndrome; eosinophilic fasciits; scleroderma.

Figure 1.

Elbow joint contracture and muscle atrophy. It shows groove signs in the forearm (open arrows).

Figure 2.

Biopsy of the skin and deep dermis of the thigh, including the adjacent fascia, showed patchy inflammatory infiltrates that included lymphocytes, plasma cells, and eosinophils with a predominance of the inflammation within the fascial layer (40x magnification).

Figure 3.

Biopsy of the vastus lateralis muscle demonstrates heavy inflammatory exudate, which is concentrated at perivascular sites in the perimysium (closed arrows) and fascia overlying the muscle. Important perifascicular muscle atrophy is present (40x magnification).