Resolution of pansclerotic morphea after treatment with antithymocyte globulin.

Abstract

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.

• Authors: Philip Song, Christopher Gocke, Fredrick M Wigley, Francesco Boin
• Journal: Nature reviews. Rheumatology (Nat Rev Rheumatol) Vol. 5 Issue 9 Pg. 513-6 (Sep 2009) ISSN: 1759-4804 [Electronic] United States
• PMID: 19710673 (Publication Type: Case Reports, Journal Article)

Chemical References

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

Topics

• Antilymphocyte Serum (therapeutic use)
• Bone Marrow Cells (drug effects, 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, drug therapy, pathology)
• Remission Induction
• Scleroderma, Localized (complications, drug therapy, pathology)
• Treatment Outcome