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Resolution of pansclerotic morphea after treatment with antithymocyte globulin

Nature Reviews Rheumatology volume 5, pages 513–516 (2009)Cite this article

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.

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Figure 1: Photographs of the patient's skin before treatment and 10 months after administration of ATG and ciclosporin.
Figure 2: Histology of a bone marrow biopsy sample after initial immunosupporessive therapy, showing megakaryocytic and erythroid precursor aplasia.
Figure 3: Timeline of clinical disease course and therapy.

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Acknowledgements

Written consent for publication was obtained from the patient.

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Authors and Affiliations

  1. Division of Rheumatology, Johns Hopkins University School of Medicine Baltimore, MD, USA

    Philip Song, Fredrick M. Wigley & Francesco Boin

  2. Department of Pathology, Johns Hopkins University School of Medicine Baltimore, MD, USA

    Christopher Gocke

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Correspondence to Francesco Boin.

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Song, P., Gocke, C., Wigley, F. et al. Resolution of pansclerotic morphea after treatment with antithymocyte globulin. Nat Rev Rheumatol 5, 513–516 (2009). https://doi.org/10.1038/nrrheum.2009.159

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