Viravan S, Wisuthsarewong W, Manonukul J, Tanphaichitr V S
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Asian Pac J Allergy Immunol. 1997 Sep;15(3):161-6.
This is a report of a case, 7 1/2 year-old-boy having chronic febrile and recurrent crops of painful subcutaneous nodules on lower extremities, which had previously been diagnosed as Weber-Christian disease, which progressed to have cytophagic histiocytic activity in the skin, bone marrow with abnormal liver function and hemorrhagic diathesis. He was subsequently treated with corticosteroid without good response. After he was diagnosed as having cytophagic histiocytic panniculitis, cyclosporin A was administered intravenously in an initial dosage of 1 mg/kg/day and in oral maintenance dose of 10 mg/kg/day with a successful response and the patient completely recovered within 6 months with mild hypertension as an adverse effect.
这是一例报告，一名7岁半男童，下肢出现慢性发热及反复出现的疼痛性皮下结节，此前被诊断为韦-克病，病情进展至皮肤、骨髓出现噬血细胞活性，伴有肝功能异常及出血倾向。他随后接受皮质类固醇治疗，但效果不佳。在被诊断为噬血细胞性脂膜炎后，给予环孢素A静脉注射，初始剂量为1毫克/千克/天，口服维持剂量为10毫克/千克/天，治疗取得成功，患者在6个月内完全康复，仅出现轻度高血压这一不良反应。
