Crouch Rohan, Akhras Victoria, Sarkany Robert
Department of Dermatology, St George's Hospital and Medical School, London, UK.
Australas J Dermatol. 2007 Aug;48(3):178-81. doi: 10.1111/j.1440-0960.2007.00375.x.
A 44-year-old man presented with a 2-year history of an intermittent urticarial rash, malaise, weight loss, night sweats, headaches and bone pains. Initial investigations indicated an elevated erythrocyte sedimentation rate, white cell count and a monoclonal immunoglobulin-M paraprotein. Histological examination revealed a perivascular mixed inflammatory infiltrate with leukocytoclasis, nuclear dust without fibrinoid necrosis and extravasated red blood cells. A diagnosis of Schnitzler's syndrome was made. Over an 8-year period, the patient was treated with continuous oral prednisone (minimum dose 20 mg/day) combined with multiple systemic agents. He was commenced on anakinra, a recombinant form of human interleukin-1 receptor antagonist, at a dose of 100 mg injected subcutaneously daily. On review 1 week later, the patient's systemic symptoms had resolved, and his previously elevated white cell count and inflammatory markers had normalized. The use of anakinra in our patient resulted in resolution of symptoms and has enabled cessation of oral prednisone. Our patient remains symptom free on anakinra after 14 months of follow up.
一名44岁男性，有间歇性荨麻疹皮疹、不适、体重减轻、盗汗、头痛和骨痛2年病史。初步检查显示红细胞沉降率、白细胞计数升高，以及单克隆免疫球蛋白M副蛋白。组织学检查显示血管周围混合性炎性浸润伴白细胞破碎、无纤维蛋白样坏死的核尘和红细胞外渗。诊断为施尼茨勒综合征。在8年期间，该患者接受了持续口服泼尼松（最小剂量20毫克/天）联合多种全身用药治疗。开始使用阿那白滞素，一种重组形式的人白细胞介素-1受体拮抗剂，剂量为每日100毫克皮下注射。1周后复查，患者的全身症状已缓解，其先前升高的白细胞计数和炎症标志物已恢复正常。在我们的患者中使用阿那白滞素使症状得到缓解，并得以停用口服泼尼松。经过14个月的随访，我们的患者在使用阿那白滞素后仍无症状。
