Resolution of pansclerotic morphea after treatment with antithymocyte globulin.
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.


METHODS
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.


METHODS
Pansclerotic morphea associated with red cell aplasia and immune-mediated thrombocytopenia.


RESULTS
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.
Song
Philip
P
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA.
Gocke
Christopher
C
Wigley
Fredrick M
FM
Boin
Francesco
F
D000961:Antilymphocyte Serum / Q000627:therapeutic use*; D001854:Bone Marrow Cells / Q000187:drug effects / Q000473:pathology; D016572:Cyclosporine / Q000627:therapeutic use; D003879:Dermatologic Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D012010:Red-Cell Aplasia, Pure / Q000150:complications / Q000188:drug therapy / Q000473:pathology; D012074:Remission Induction; D012594:Scleroderma, Localized / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
D000961:Antilymphocyte Serum; D003879:Dermatologic Agents; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D008775:Methylprednisolone