A 52-year-old woman of Cuban origin was referred to our clinic for skin hardening for over 15 years. Her past medical history was pertinent for mild asthma and severe glaucoma. On skin examination, there were three ill-defined indurated, depressed plaques measuring 10 cm x 15 cm on the left mid-back, 7 cm x 7 cm on the left posterior shoulder, and 15 cm x 3 cm linear induration extending to the left forearm (Figure 1(a) and (b)). Examination of the oral mucosa and genitalia was unremarkable. Workup, including complete blood count, chemistry, renal and liver function tests, borrelia serology, antinuclear, and systemic sclerosis specific antibodies, was normal, except for mild eosinophilia. A punch biopsy of the left upper back lesion demonstrated a normal epidermis and superficial dermis. There was non-inflammatory extensive sclerosis in the deep dermis, subcutaneous tissue, and fascia.
The diagnosis of linear localized scleroderma profunda versus eosinophilic fasciitis was made. Pre-treatment screening for hepatitis B, hepatitis C, human immunodeficiency virus, QuantiFERON-TB Gold, and strongyloides serology were performed and subsequently revealed the presence of strongyloides antibodies, which were confirmed by fecal culture. The patient was treated with oral ivermectin resulting in a resolution of her respiratory symptoms and normalization of the eosinophil counts.
After successful eradication of strongyloidiasis, we planned to initiate methotrexate monotherapy, which was delayed due to scheduled hysterectomy for uterine fibroids. When the patient returned to our clinic, a new linear depressed plaque on the mid-forehead was noted (Figure 1(c)). She also complained of worsened vision in the right eye and new-onset migraine-type headache. Given the typical clinical appearance of the forehead lesion, a diagnosis of ECDS scleroderma was made. She was referred to her treating ophthalmologist, and a magnetic resonance imaging (MRI) of the brain was ordered. The MRI showed no skeletal or parenchymal abnormalities. Ocular examination revealed stable glaucoma. Oral prednisone 1 mg/kg/day (tapered over 3 months) and methotrexate 20 mg/week with folic acid and vitamin D supplementation were prescribed with regular follow-up. After 3 months of therapy, the size and induration of the truncal plaques had improved (Figure 2(a) and (b)). The linear groove over her forehead remained stable (Figure 2(c)). No new episodes of migraine headaches have occurred.