A Case of Eosinophilic Fasciitis Presenting as Pitting Edema of the Lower Extremities
A 19-year-old female visited our hospital with a 2 wk history of progressive pitting edema in both lower legs associated with a 2 kg weight gain. Symptom onset began shortly after intense physical exertion (dance practice). Her medical history included allergic rhinitis with sensitization to house dust mite and pollen, and she had visited the pediatric allergy clinic for about 10 years. None of her family members had a history of similar symptoms or specific diseases. Additionally, she noted mild pain in both knees and ankles, but tenderness was not remarkable on physical examination. She denied fever, dyspnea, chest pain, and urinary difficulty. She was not taking any medications. Physical examination revealed moderate bilateral pretibial pitting edema. No redness, induration, or thickened skin was observed. No contractures of joints were found, and muscle strength was normal in the upper and lower extremities.\nAt first, she visited the cardiovascular surgery clinic and was admitted for evaluation and treatment of deep vein thrombosis. However, computed tomography (CT) angiography of her lower extremities revealed no evidence of venous disturbance. Treatment with heparin and application of compression stockings did not alleviate her symptoms. Initial laboratory findings included a white blood cell count of 8,090 cells/µL with 28.9% eosinophils. Her eosinophil count was 2,340 cells/µL. Her hemoglobin level and platelet count were normal and her serum total IgE level was 786 kIU/L, but no remarkable change was observed compared to the previous year. The erythrocyte sedimentation rate and C-reactive protein level were not elevated. Serum urea nitrogen and creatinine were normal and urinalysis revealed no proteinuria or hematuria. Serum albumin was unremarkable at 4.2 g/dL. Serum protein electrophoresis results were within the normal range. Other laboratory data, including serum aminotransferase, alkaline phosphatase, cholesterol, and electrolyte levels, were normal. Further evaluations to determine the cause of her symptoms and peripheral eosinophilia were performed. There was no evidence of parasitic infection. A thyroid function test revealed normal thyroid function. The results of electrocardiogram and pulmonary function tests were normal. The patient was negative for rheumatoid factor and antinuclear antibody. An abdominal ultrasound showed no pathological changes. However, during the time of evaluation, the patient's blood eosinophil count increased rapidly to 4,880 cells/µL.\nThe patient was referred to our division of pediatric allergy considering the findings of peripheral eosinophilia and a past history of allergy, and a magnetic resonance imaging (MRI) was performed in her lower thigh. As shown in , the signal intensity of subcutaneous lesions was increased in fat-saturated T2-weighted images, suggesting inflammation of the subcutaneous fat layer and superficial fascia. Mild synovitis of both knees was also suggested. No evidence of thickening or fibrosis of the deep fascia was present. A biopsy of the right dorsal foot was performed, and the biopsy specimen included skin and underlying subcutaneous tissue. Biopsy examination revealed dermal edema and perivenular lymphoplasmacytic and eosinophilic infiltration in the subcutaneous fat tissue (). Based on the MRI and biopsy results, we diagnosed EF. We started intravenous corticosteroid therapy: dexamethasone 15 mg/day on the day of diagnosis. After 2 days of dexamethasone therapy, the symmetric pitting edema improved dramatically. The eosinophil count decreased rapidly from 4,880 to 140 cells/µL. We changed the intravenous dexamethasone to 30 mg/day of oral prednisolone, and the patient received an additional day of therapy with oral steroids. After a total of 3 days of intravenous plus oral steroid therapy, the patient recovered fully. She was discharged from the hospital without medication. Mild edematous changes in both legs developed 2 weeks later, but the patient's symptoms immediately relieved after administration of 30 mg/day prednisolone for 1day. She was monitored in an outpatient clinic for 4 months after cessation of therapy. The most recent eosinophil count was 320 cells/µL, and no relapses were observed.