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. Access through your institution Buy or subscribe This is a preview of subscription content, access via your institution ACCESS OPTIONS Access through your

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references ACKNOWLEDGEMENTS Written consent for publication was obtained from the patient. AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Division of Rheumatology, Johns Hopkins University

School of Medicine Baltimore, MD, USA Philip Song, Fredrick M. Wigley & Francesco Boin * Department of Pathology, Johns Hopkins University School of Medicine Baltimore, MD, USA

Christopher Gocke Authors * Philip Song View author publications You can also search for this author inPubMed Google Scholar * Christopher Gocke View author publications You can also search

for this author inPubMed Google Scholar * Fredrick M. Wigley View author publications You can also search for this author inPubMed Google Scholar * Francesco Boin View author publications

You can also search for this author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to Francesco Boin. ETHICS DECLARATIONS COMPETING INTERESTS The authors declare no competing

financial interests. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE 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 Download citation * Issue Date: September 2009 * DOI:

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