Background: A previously healthy 50-year-old man presented with thickening and hardening of the skin on his trunk, neck and upper extremities that had started after the appearance of a 5 cm web-like patch of blood vessels on his upper chest and progressed over 4 months. He also reported difficulties with swallowing and a 20 kg weight loss.
Investigations: Physical examination, laboratory testing, including complete blood count, autoimmune serology for antiplatelet, antinuclear and extractable nuclear antibodies, direct antiglobulin test, incisional skin biopsy, bone-marrow biopsy, and MRI of the upper extremities.
Diagnosis: Pansclerotic morphea associated with red cell aplasia and immune-mediated thrombocytopenia.
Management: Treatment with prednisone 60 mg per day and methotrexate 15 mg per week was started, but symptoms worsened. Methotrexate was replaced by pulsed intravenous methylprednisolone (1 g daily for 3 days), followed by mycophenolate mofetil started at 1 g per day and titrated up over 4 weeks to 3 g per day. Severe bicytopenia developed that did not improve with an 8-week washout of immunosuppressive agents. His fibrotic skin and hematologic conditions dramatically responded to antithymocyte globulin 40 mg/kg daily for 4 days, plus 10 mg/kg ciclosporin and methylprednisolone 1 mg/kg per day.