Eiling Elisabeth, Möller Maike, Kreiselmaier Inga, Brasch Jochen, Schwarz Thomas
Department of Dermatology, University of Kiel, Germany.
J Am Acad Dermatol. 2007 Aug;57(2):361-4. doi: 10.1016/j.jaad.2007.03.036. Epub 2007 Apr 30.
Schnitzler syndrome is a rare disease characterized by a chronic urticarial rash and a monoclonal gammopathy. The exact pathogenesis is still uncertain and treatment remains a challenge. Here, we report a patient who was only recently given the diagnosis of Schnitzler syndrome although the typical symptoms had been present for about 6 years. Administration of the B-cell antibody rituximab did not exert any beneficial effect despite effective elimination of B cells and a reduction of the paraprotein. In contrast, injection of the interleukin-1-receptor antagonist anakinra caused a complete remission within a few days.
施尼茨勒综合征是一种罕见疾病，其特征为慢性荨麻疹样皮疹和单克隆丙种球蛋白病。确切的发病机制仍不确定，治疗仍然是一项挑战。在此，我们报告一名患者，尽管典型症状已出现约6年，但直到最近才被诊断为施尼茨勒综合征。尽管有效清除了B细胞并降低了副蛋白，但给予B细胞抗体利妥昔单抗并未产生任何有益效果。相比之下，注射白细胞介素-1受体拮抗剂阿那白滞素在几天内就导致了完全缓解。
