Eosinophilic Fasciitis

Thank you to Dr. Dave Ellis for presenting a very interesting case of Eosinophilic Fasciitis. A middle aged person presented with diffuse myalgias for several months and subacute development of thickened skin lesions with peripheral eosinophilia and a normal CK. In conference, a differential was initially built around myalgias and then with the presentation of the skin findings, another differential was built around the thickened skin findings consistent with morphea (localized skin thickening). Those two sets of differential diagnoses along with the physical exam and labs were helpful here. The differential included a discussion of: systemic scleroderma, eosinophila-myalgia syndrome (secondary to exposures to L-tryptophan), toxic oil syndrome (secondary to adulterated rapeseed oil), hypereosinophilic syndrome, EGPA, nephrogenic systemic fibrosis, scleromyxedema, GVHD, amyloidosis, PMR, polymyositis/dermatomyositis, viral etiologies, medication use or discontinuation, environemental exposures and fibromyalgia.

Eosinophilic fasciitis or Shulman Syndrome was first described in 1974 and is a rare disorder with approximately 300 described cases. It is characterized by:

 The "groove sign": skin furrowed along the course of veins as a result of fascial sclerosis. This is accentuated by elevation of the limb. Image courtesy of the  International Journal of Dermatology

The "groove sign": skin furrowed along the course of veins as a result of fascial sclerosis. This is accentuated by elevation of the limb. Image courtesy of the International Journal of Dermatology

  • Skin: symmetric (upper and lower extremities, often spares the hands, feet and face); progression from erythema and edema in early phases to puckering and "peau d'orange" texture which is a characteristic distinguishing trait from the smooth/shiny texture in systemic sclerosis; exam notable for the Groove sign and a Prayer sign
  • Arthritis: inflammatory in regions adjacent to fasciitis with contractures and loss of mobility
  • Myalgias: commonly described in the setting of fasciitis but, myositis has not been extensively reported
  • Neuropathies: both cranial and peripheral
  • Viscera: not typical but occasionally pericarditis or renal involvement
  • Peripheral Eosinophilia: present in the early phases and then can wane in the later starges
  • Normal CK
  • Autoantibodies and ANCA often negative (can be seen in 15-20%)
  • Hematologic Association with aplastic anemia, lymphoma, MM< lymphocytic leukoemia, MDS

Often it may be challenging to differentiate eosinophilic fasciitis and systemic sclerosis. The review article in Current Rheumatology Reports has a nice table displaying the differences.

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Generally the diagnosis is made on a full thickness muscle biopsy with evidene of eosinophilas, plasma cells and macrophages in the thickened fascia. MRI imaging may be useful to help correlate findings or to find a biopsy site.

 Photomicrograph of a full thickness muscle biopsy showing thickened fascia with inflammation. The closed arrow shows a mononuclear infiltrate primarily in the fascia. The open arrow shows the muscle spared of inflammation. The line shows the inflammed fascia. Image courtesy of the International  Journal of Rheumatic Disease

Photomicrograph of a full thickness muscle biopsy showing thickened fascia with inflammation. The closed arrow shows a mononuclear infiltrate primarily in the fascia. The open arrow shows the muscle spared of inflammation. The line shows the inflammed fascia. Image courtesy of the International Journal of Rheumatic Disease

Treatment is usual high dose corticosteroids (prednisone 0.5 to 2 mg/kg/day). The PICO question for the case was investigating the use of steroid sparing agents. As one may imagine, the literature for a rare disease is not very robust. However, the International Journal of Rheumatic Disease describes the use of methotrexate with some success. A recent study in the International Journal of Dermatology describes use of rituximab also.