Song Philip, Gocke Christopher, Wigley Fredrick M, Boin Francesco
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA.
Nat Rev Rheumatol. 2009 Sep;5(9):513-6. doi: 10.1038/nrrheum.2009.159.
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.
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.
Pansclerotic morphea associated with red cell aplasia and immune-mediated thrombocytopenia.
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.
一名既往健康的50岁男性，其躯干、颈部及上肢皮肤出现增厚和硬化，这是在其前胸出现一块5厘米宽的网状血管斑之后开始的，并在4个月内逐渐进展。他还报告有吞咽困难及体重减轻20千克。
体格检查、实验室检查，包括全血细胞计数、抗血小板、抗核及可提取核抗原的自身免疫血清学检查、直接抗球蛋白试验、切开皮肤活检、骨髓活检以及上肢磁共振成像。
泛发性硬斑病伴红细胞再生障碍及免疫介导的血小板减少症。
开始使用泼尼松每日60毫克及甲氨蝶呤每周15毫克进行治疗，但症状恶化。甲氨蝶呤被静脉注射甲泼尼龙冲击治疗（每日1克，共3天）所替代，随后开始使用霉酚酸酯，起始剂量为每日1克，并在4周内逐渐增至每日3克。出现严重的全血细胞减少，在停用免疫抑制剂8周后仍未改善。他的皮肤纤维化和血液学状况对抗胸腺细胞球蛋白每日40毫克/千克，共4天，加环孢素10毫克/千克及甲泼尼龙每日1毫克/千克有显著反应。
