Resolution of pansclerotic morphea after treatment with antithymocyte globulin

Nat Rev Rheumatol. 2009 Sep;5(9):513-6. doi: 10.1038/nrrheum.2009.159.


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.

Publication types

  • Case Reports

MeSH terms

  • Antilymphocyte Serum / therapeutic use*
  • Bone Marrow Cells / drug effects
  • Bone Marrow Cells / pathology
  • Cyclosporine / therapeutic use
  • Dermatologic Agents / therapeutic use
  • Drug Therapy, Combination
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Red-Cell Aplasia, Pure / complications
  • Red-Cell Aplasia, Pure / drug therapy
  • Red-Cell Aplasia, Pure / pathology
  • Remission Induction
  • Scleroderma, Localized / complications
  • Scleroderma, Localized / drug therapy*
  • Scleroderma, Localized / pathology
  • Treatment Outcome


  • Antilymphocyte Serum
  • Dermatologic Agents
  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclosporine
  • Methylprednisolone