Resolution of pansclerotic morphea after treatment with antithymocyte globulin.

Philip Song, Christopher Gocke, Fredrick Wigley, Francesco Boin

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)513-516
Number of pages4
JournalNature Reviews Rheumatology
Volume5
Issue number9
DOIs
StatePublished - Sep 2009

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Localized Scleroderma
Antilymphocyte Serum
Methylprednisolone
Methotrexate
Upper Extremity
Skin
Mycophenolic Acid
Biopsy
Coombs Test
Idiopathic Thrombocytopenic Purpura
Blood Cell Count
Serology
Immunosuppressive Agents
Deglutition
Prednisone
Cyclosporine
Physical Examination
Blood Vessels
Weight Loss
Neck

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Resolution of pansclerotic morphea after treatment with antithymocyte globulin. / Song, Philip; Gocke, Christopher; Wigley, Fredrick; Boin, Francesco.

In: Nature Reviews Rheumatology, Vol. 5, No. 9, 09.2009, p. 513-516.

Research output: Contribution to journalArticle

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