A 61-year-old female, with a history of diabetes mellitus, presented with a 2-year history of skin thickening affecting the lower limbs, inner thighs, lumbar region, abdominal flanks and forearms. She also reported asthenia and arthralgia (tibiotarsal joint). She denied having dysphagia or Raynaud's phenomenon. Physical examination showed indurated skin in the regions previously mentioned, sparing the fingers. These areas were hyperpigmented. She also had a "peau d'orange" appearance involving both legs (Fig. 1) and forearms as well as a "groove sign" visible on both forearms (Fig. 2).
Laboratory tests showed peripheral eosinophilia and an elevated ESR. Much like the previous patient, solid neoplasms were excluded, immunological studies were within the normal range and work-up for HIV, hepatotropic viruses and latent tuberculosis was negative. Pulmonary function tests and the thoracic CT scan were all normal. A full thickness skin biopsy showed fibrosis of the dermis, subcutaneous adipose tissue, fascia and muscle, with perivascular lymphocytes and eosinophil infiltration compatible with FE. The diagnosis of FE was made, and the patient was started on an SCS (prednisolone 60 mg/day) and MTX (15 mg/week), resulting in partial improvement of the lesions: lumbar region and forearms. However, due to evidence of corticosteroid adverse effects, such as uncontrolled diabetes and femoral head avascular necrosis, the dose was slowly tapered, while MTX was raised (until it was 20 mg/week). Considering that after 9 months of treatment she only demonstrated a partial response to MTX (20 mg/week), we decided to start the patient on tocilizumab (8 mg/kg). After 2 cycles of tocilizumab she finally started to show leg improvement, regaining mobility and enabling us to suspend MTX. At the time of writing, the patient is under tocilizumab alone and maintains clinical improvement: skin thickening limited to distal lower limbs.