The ultimate prognosis of eosinophilic fasciitis may not be groovy
Sept. 18, 2017
My patients with eosinophilic fasciitis (EF, Shulman syndrome) have been fortunate. They have been “classic” cases with mostly upper extremity lesions, precipitated by exercise, and have responded to corticosteroids. My sanguine impression of EF has been tempered by recent literature — it may not be so benign.
EF is characterized by erythema, edema, and induration of the extremities associated with joint contractures. The trunk and neck may be affected, with typical sparing of the face, hands, and feet. (1) The “groove sign” (linear depressions along the course of veins, caused by fascial sclerosis of the connective tissue surrounding superficial veins, rendering them immobile ) and a “peau d’orange” appearance may be noted. Nail fold capillary changes, Raynaud phenomenon, and sclerodactyly, as appreciated in systemic sclerosis, are not observed. (1) In a recent series of EF patients, 28% (8 of 29) had a history of trauma or intense exercise that preceded the illness. (3)
Aside from trauma and hematologic associations (noted in approximately 10%, with aplastic anemia being most common), other reported correlations or inciting factors include:
1. Drugs (simvastatin, atorvastatin, phenytoin, ramipril, heparin, trichloroethylene exposure). Recently, EF has been attributed to infliximab in a patient with psoriatic arthritis (4)
2. Infections (Borrelia burgdorferi, Borrelia afzelii, Mycoplasma arginini)
3. Solid tumors (choroidal melanoma, prostate cancer, lung cancer, breast cancer)
4. Autoimmune disorders (Hashimoto thyroiditis, Graves disease, primary biliary cirrhosis, lupus erythematosus, vasculitis, hemolytic anemia, idiopathic thrombocytopenic purpura, Sjögren syndrome)
5. Physical factors (radiotherapy, burns).
6. Laboratory findings include peripheral eosinophilia in 60-90% of cases, hypergammaglobulinemia and an increased erythrocyte sedimentation rate (ESR). No antibodies are detected, such as ANA or anti-SCL 70. Eosinophil counts may be very high, but are not associated with disease prognosis (5)
The diagnosis of EF may be confirmed by MRI scans revealing a high signal intensity of the deep and superficial fasciae, and/or histologic confirmation demonstrating fascial thickening associated with a polymorphous inflammatory infiltrate composed of plasma cells, eosinophils, histiocytes, and lymphocytes, that ultimately result in fibrosis. Although EF may resolve spontaneously, corticosteroids are considered first-line therapy; combination treatment with other immunosuppressive agents, such as methotrexate, may yield a higher complete response rate. Physical therapy is of value for those at risk of joint contractures (1)
Mertens et al studied 35 patients with EF (24 of whom were female [68.6%]) with a median age of 60 years) to determine if there is any residual disease in the long-term. All patients had detectable residual damage. Impairment of quality of life correlated to the extent of residual manifestations. Other statistically significant correlates with residual disease included an increased C-reactive protein level, involvement of the neck and trunk, prolonged time to disease remission, presence of concomitant morphea. and the maximum methotrexate dose. The authors concluded that although all patients with EF had detectable residual damage, it is those with advanced age and signs of severe disease at presentation that correlate with the severity of residual damage. (6)
As pointed out by Wright et al, frequent misdiagnoses, unnecessary procedures, and inappropriate therapies all confound the diagnosis and management of EF patients. Of the 63 EF patients in their review, more than 10% did not receive steroids, and only 37% were referred for physical therapy despite the high rate of joint contractures. Early treatment of EF results in better outcomes.(3) The grooviest accomplishment would be to diagnose EF as soon as early as possible and obviate the groove sign altogether.
(PS — In real life, I never use the word “groovy.”)
1. Heymann WR. Eosinophilic fasciitis: A firm warning. Skinmed 2017; 15: 139-40.
2. Fruchter RA, et al. Groove sign of eosinophilic fasciitis. J Clin Rheumatol 2017; 23: 169.
3. Wright NA, Mazori DR, Patel M, Merola JF, et al. Epidemiology and treatment of eosinophilic fasciitis: An analysis of 63 patients from 3 tertiary care centers. JAMA Dermatology 2016; 152; 97-99.
4. Hariman R. Development of eosinophilic fasciitis during infliximab therapy for psoriatic arthritis. Case Rep Rheumatol 2016; 2016: 7906013.
5. Lamback EB, et al. Eosinophilic fasciitis. An Bras Dermatol 2016 ; 91(5 suppl 1):57-59.
6. Mertens JS, et al. Long-term outcome of eosinophilic fasciitis: A cross-sectional evaluation of 35 patients. J Am Acad Dermatol 2017; 77: 512-7.
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