¹⁸F-FDG PET/CT and MRI findings of Shulman syndrome also known as eosinophilic fasciitis

Excerpt

A 36-year-old man was admitted to our hospital for the exploration of polyarthralgia and skin eruption. The patient had suffered for 2 months from muscle pain, weakness and fatigue, and peripheral arthralgia. On admission, physical examination revealed hyperpigmented erythematous skin lesions on the anterior chest wall, without fever. Biological results showed eosinophilia (40% of leukocytes) and inflammatory syndrome (CRP = 86 mg/L). All autoantibodies and serology tests were negatives. Whole-body non-contrast-enhanced ¹⁸F-FDG PET/CT was performed. The 3D-PET MIP showed ¹⁸F-FDG uptakes in the shoulders, wrists, knees, and ankles (Fig. 1a). The fused PET/MRI, MRI, and PET/CT images showed diffuse thickening of the muscular fasciae (Fig. 1b and c). The histological examination of skin biopsy showed lymphocytic inflammation of the dermal/hypodermal layers with eosinophilic cells infiltration (Fig. 1d, blue arrows). These findings perfectly matched with the diagnosis of Shulman syndrome, also known as eosinophilic fasciitis. A glucocorticoid treatment (GC) was started at 1 mg/kg/day. Because of relapses, methotrexate and tofacitinib were added. The patient died from a severe pulmonary infection 1.5 years after the disease started. …