EOSINOPHILIC FASCIITIS

By Gary M. White, MD


Eosinophilic fasciitis (EF) is an uncommon disease characterized by the sudden onset of erythema, swelling and then induration of the skin.

Clinical

The rapid onset of symmetric erythema and swelling of the of the extremities combined with a striking peripheral blood eosinophilia is characteristic. The skin then takes on a dimpling or peau d'orange appearance which then evolves into woody induration. The trunk may be involved but the hands and feet are usually spared. In approximately half of the cases, a bout of strenuous exercise preceded the onset of symptoms. Internal involvement is rare.

Systemic

EF has been frequently reported associated with autoimmune and hematologic diseases such as aplastic anemia, hemolytic anemia, peripheral autoimmune thrombocytopenia, pernicious anemia, leukemias and lymphomas. EF may also be associated with autoimmune conditions such as HashimotoÂ’s thyroiditis. Arthritis, low-grade myositis and carpal tunnel syndrome may occur. Isolated cases of pulmonary, esophageal and cardiac involvement have been reported.

Work up

Work up includes HP, PFT's, CXR, ANA, CBC, full thickness biopsy to include the fascia, and inquiry about a history of Raynaud's phenomenon. Absence of Raynaud's phenomenon, lack of sclerodactyly and normal nail fold capillaroscopy help exclude systemic sclerosis. Exposure to trichloroethylene and L-tryptophan should be determined.

Treatment

In a large cohort of 63 patients, the most successful treatment was a combination of prednisone and methotrexate, inducing clearing in 64% [JAMA Derm 2016;152;97]. In another study of 12 patients, high-dose IV pulse methotrexate was safe and effective [JAMA Derm 2016;152;1262]. Alternatively systemic steroids may be used alone. Pulsed methylprednisolone has been helpful when daily prednisone has failed [BJD 1999;140;1185]. Sirolimus 2 grams/d with prednisone 5 mg/day was helpful in one man [JAMA Derm 2016;152;488]. Hydroxychloroquine 200-400 mg has also been employed. Antihistamines have been reported helpful, principally cimetidine and cetirizine. Other agents which have been reported helpful include ketotifen, penicillamine, methotrexate, PUVA, bath PUVA and cyclosporin.

Referral to physical therapy is often needed, as involvement over the joints can reduce mobility.

References

Progressive sclerosis after a sunburn. Washington University Case of the Month

Rheum Dis Clin North Am. 2008 Feb; 34(1): 199

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