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A Case of Eosinophilic Fasciitis Presenting with Pansclerotic Morphea

Julia K. Munchel, MD, & William E. Monaco, MD  |  Issue: May 2019  |  

Figure 3. The skin of the patient’s lower extremities was shiny, thickened and hairless.

Figure 3. The skin of the patient’s lower extremities was shiny, thickened and hairless.

The labs at the time were significant for an albumin of 2.1 gm/dL, a thyroid-stimulating hormone of 1.92 mIU/mL, a 24-hour urine protein of <0.14 gm/24h, a faint IgG kappa restriction (kappa free light chains 3.45 mg/dL with kappa lambda free light chain ratio 1.96), and a white blood cell count of 7.9 x 103 mcL with 21.9% eosinophils. An endoscopy and a colonoscopy were unremarkable; biopsies showed non-specific duodenitis and gastritis. Bone marrow biopsy was normal. Ultimately, she was referred to rheumatology for further workup.

On presentation at the rheumatology clinic, she complained of new symptoms, including jaw locking, hair loss (including loss of her pubic hair), hand swelling, 40 minutes of morning stiffness, fevers, chills and stocking distribution paresthesias.

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The physical exam revealed a sallow-complexioned female with temporal wasting bilaterally, a small, tight oral aperture, complete hair loss on her extremities, normal nailfold capillaries, no telangiectasias, no digital ulcers and skin thickening on her face, neck, upper back, arms, hands, legs and feet (see Figures 1–3, below). A joint exam was significant for her inability to abduct her shoulders beyond 90º and bilateral flexion contractures of her elbows. No synovitis was noted.

Laboratory results for creatinine kinase, anti-nuclear antibody, complement, extractable nuclear antigens, vascular endothelial growth factor and RNA polymerase III were all normal, but her C-reactive protein and sedimentation rate peaked at 66 mg/L and 42 mm/hr, respectively. Further imaging included computed tomography of her chest, abdomen and pelvis, which revealed no evidence of malignancy, as well as a skeletal survey that was negative for any osteolytic bone lesions. Electromyography showed diffuse myopathic changes, proximally and distally.

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A skin biopsy revealed increased dermal fibroblasts, mucin with hyalinization and a negative congo red stain that was interpreted as diagnostic of scleromyxedema. Treatment with IVIG 2 gm/kg every four weeks was initiated, with initial improvement in her subjective skin thickness.

Approximately six months later, she sought a second opinion at Johns Hopkins when her symptoms worsened. Per their recommendation, forearm magnetic resonance imaging demonstrated a bright T2 signal suggestive of fasciitis, and a full-thickness skin biopsy showed bands of fibrosis and hyalinization consistent with eosinophilic fasciitis. Following this biopsy she was started on 60 mg of prednisone, as well as 720 mg of mycophenolate sodium, twice a day.

Unfortunately, she continues to suffer from neuropathic pain and skin thickening.

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