A previously healthy 37-year-old male presented with a 2-year history of skin erythema and thickening affecting the limbs and lumbar region, and sparing the skin of the face, hands and feet. He also complained of pain in the lower limbs and joint stiffness in the knees, ankles and wrists, precluding him from working. Furthermore, he reported fatigue and weight loss. These symptoms improved during a previous 3-month cycle of an SCS (prednisolone 60 mg/day), but relapsed after its suspension. Physical examination showed diffuse cutaneous cyanosis and indurated skin, especially in the forearms and legs but sparing the fingers, with a "peau d'orange" appearance involving the proximal areas and a "groove sign" visible on both forearms. Laboratory tests, including for peripheral eosinophils, the erythrocyte sedimentation rate (ESR) and immunological studies (immunoglobulins, antinuclear, antineutrophil cytoplasmic, anticentromere, anti-Scl-70, anti-U1 RNP and anti-RNA polymerase III antibodies), were all within the normal range. Work-up for HIV, hepatotropic viruses and latent tuberculosis was negative. The patient was submitted to thoracic radiography and oesophageal manometry, both of which showed normal results. Solid neoplasms were excluded. He had had a previous full thickness incisional skin biopsy, carried out after the corticosteroid cycle, that showed some characteristics suggesting FE: perivascular inflammatory cell infiltrate involving the fascia and adipose tissue as well as fascia and fibrous septae thickening. Considering the typical clinical presentation and skin biopsy, the diagnosis of FE was made, and the patient was started on an SCS (prednisolone 20 mg/day) and methotrexate (MTX; 12.5 mg/week). He responded favourably but with corticosteroid tapering and then suspension, symptoms relapsed. Hence, the SCS was reintroduced (prednisolone 5 mg/day) and the MTX dose was up-titrated (20 mg/week). However, due to hepatic toxicity and a concomitant insufficient response, MTX was discontinued. The patient was then started on tocilizumab (162 mg/week), with an excellent and sustained clinical response: after 1 year of treatment, the corticosteroid was successfully suspended. At the time of writing, the patient has been under tocilizumab alone for the last 12 months, showing significant and progressive improvement of the skin thickening (especially in the limbs) and absence of skin erythema, cyanosis, limb pain, joint stiffness or fatigue. He recovered his weight loss and is now able to work.