Chow R K, Benny W B, Coupe R L, Dodd W A, Ongley R C
Division of Dermatology, University of British Columbia, Vancouver.
Arch Dermatol. 1996 Nov;132(11):1360-4.
We review the literature and report a case of refractory erythema elevatum diutinum associated with IgA paraproteinemia that was successfully controlled with intermittent plasma exchange (PLEX).
Typical lesions of erythema elevatum diutinum developed in a 72-year-old patient with IgA paraproteinemia; the condition predictably flared whenever IgA levels reached a threshold of 8 g/L. After 8 years of unsuccessful treatment with various agents, we instituted a trial of PLEX during an acute flare. Following 6 exchanges over a period of 2 weeks, the IgA level decreased from 8 to 2 g/L and the skin lesions cleared. Three weeks later, new skin lesions developed and the IgA level had rebounded from 2 to just over 8 g/L. A second course of PLEX was administered, with excellent results, and a 3-month course of oral chlorambucil (2 mg/d) was initiated. The patient's condition remained in clinical remission for 10 months. Over the ensuing 9 years, she suffered 11 further flares, each of which was associated with IgA levels of 8 to 10 g/L and each responding dramatically to 3 to 5 PLEXs followed by a consolidative dose of intravenous cyclophosphamide (250-500 mg).
We believe that PLEX may have an important role in the management of severe erythema elevatum diutinum associated with monoclonal paraproteinemia refractory to other therapy.
我们回顾了文献并报告了一例与IgA副蛋白血症相关的顽固性持久性隆起性红斑病例，该病例通过间歇性血浆置换（PLEX）成功得到控制。
一名72岁的IgA副蛋白血症患者出现了典型的持久性隆起性红斑病变；每当IgA水平达到8 g/L阈值时，病情就会如期发作。在使用各种药物治疗8年无效后，我们在一次急性发作期间进行了PLEX试验。在2周内进行6次置换后，IgA水平从8 g/L降至2 g/L，皮肤病变消退。三周后，新的皮肤病变出现，IgA水平从2 g/L反弹至略高于8 g/L。给予第二个疗程的PLEX，效果极佳，并开始为期3个月的口服苯丁酸氮芥（2 mg/d）治疗。患者的病情临床缓解持续了10个月。在随后的9年里，她又发作了11次，每次发作都与IgA水平在8至10 g/L有关，每次发作对3至5次PLEX治疗以及随后一剂巩固剂量的静脉注射环磷酰胺（250 - 500 mg）反应显著。
我们认为，PLEX在治疗与单克隆副蛋白血症相关的、对其他治疗无效的严重持久性隆起性红斑中可能具有重要作用。
