Age / Sex : 47 / F

Age / Sex : 35 / M

Chief complaint : Pain & rigidity in joints

1) What is your impression?

Two weeks later, you can see the final diagnosis with a brief discussion of this case.

* Send Application Answers to In Sook Lee, MD (lis@pusan.ac.kr)

Courtesy : Sang-Hee Choi, Sungkyunkwan University, Samsung Medical Center

Eosinophilic fasciitis

Discussion

Findings: Bilateral symmetric thickening and hyperintensity of muscle fasciae of the thigh, especially gracilis, biceps and rectus femoris muscle on T2-weighted sequences, with strong enhancement after administration of IV contrast agent

Differential Diagnosis: Eosinophilic fasciitis, Necrotizing fasciitis. (However, MRI signal changes are usually not symmetric, and fascial thickening with contrast-enhanced muscular edema may be present. In addition, MR images of necrotizing fasciitis frequently show fluid collections, abscess formation, and cellulitis.)

Diagnosis:  Eosinophilic fasciitis

Discussion:

EF is an uncommon sclerodema-like disease clinically characterized by painful thickening and induration of the skin and subcutaneous tissues of the affected limbs. The disease may affect the upper and lower extremities and the trunk but spares the head. Raynaud’s phenomenon is usually not present, and organs are not involved. Patients may present with bilateral symmetric muscle weakness, swelling, and stiffness of the extremities and skin changes. Joint contractures may occur as a sequela of induration and sclerosis of the subcutaneous tissue.

 Peripheral blood eosinophilia is described as a hallmark of the disease, although its severity varies over time, even in the absence of specific treatment.

 A full-thickness skin-to-muscle biopsy is still considered necessary for establishing the diagnosis.

MRI features of EF are characteristic and include thickening of the superficial and, less frequently, of the deeper muscle fasciae, with high signal intensity on T2-weighted or STIR sequences and strong fascial enhancement after IV administration of extracellular gadolinium- based contrast agents. Contrary to myositis and dermatomyositis, EF shows no or only minimal signal changes within the muscle fibers and only occasional MRI abnormalities in the skin and subcutaneous tissue.

 References:

•       Baumann et al; AJR 2005;184:169–174

•       Moulton et al; AJR 2005;184:975–978