Resolution of pansclerotic morphea after treatment with antithymocyte globulin.

Full 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.

Author information

Author/s: Song, Philip (P); Gocke, Christopher (C); Wigley, Fredrick M (FM); Boin, Francesco (F);

Affiliation: Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA.

Journal and publication information

Publication Type: Case Reports; Journal Article

Journal: Nature reviews. Rheumatology (Nat Rev Rheumatol), published in United States. (Language: eng)

Reference: 2009-Sep; vol 5 (issue 9) : pp 513-6

Dates: Created 2009/08/27; Completed 2009/10/29;

PMID: 19710673, status: MEDLINE (last retrieved date: 10/29/2009)

Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.

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MeSH headings (categories)

This article was linked to the MeSH Headings (categories) shown below.

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

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

Note: Bold headings indicate primary MeSH headings or qualifiers.

Associated Chemicals: Antilymphocyte Serum (0) ; Dermatologic Agents (0) ; Glucocorticoids (0) ; Immunosuppressive Agents (0) ; Cyclosporine (59865-13-3) ; Methylprednisolone (83-43-2)

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