Leukocytoclastic vasculitis in a patient with type 1 cryoglobulinemia.
Leukocytoclastic vasculitis in a patient with type 1 cryoglobulinemia.
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DOI：
10.1155/2011/124940
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发表时间：
2011
影响因子：
--
通讯作者：
Kukes G
中科院分区：
文献类型：
--
作者：
Liu PY;Prete PE;Kukes G
1型冷球蛋白血症的皮肤表现通常与非炎症性血栓阻塞血管有关；1型冷球蛋白血症很少出现白细胞破溃性血管炎。我们报告一例64岁的男性谁提出了孤立的皮肤白细胞破坏血管炎，最初归因于必要的混合冷球蛋白血症后彻底的诊断评估。对治疗缺乏足够的临床反应促使进一步的研究，包括低温沉淀电泳和免疫固定，发现IgM kappa单克隆伽玛病与1型冷球蛋白血症一致。对潜在恶性肿瘤的重新研究导致了早期华登斯特罗姆巨球蛋白血症的发现。虽然白细胞破碎性血管炎是混合冷球蛋白血症的特征性表现，但它也可能是1型冷球蛋白血症的表现。
Cutaneous manifestations of type 1 cryoglobulinemia are usually related to vascular occlusion by noninflammatory thrombosis; rarely is leukocytoclastic vasculitis seen in type 1 cryoglobulinemia. We report the case of a 64-year-old male who presented with isolated cutaneous leukocytoclastic vasculitis that was initially attributed to essential mixed cryoglobulinemia after thorough diagnostic evaluation. A lack of adequate clinical response to therapy prompted further investigation, including cryoprecipitate electrophoresis and immunofixation, which revealed an IgM kappa monoclonal gammopathy consistent with type 1 cryoglobulinemia. A renewed search for an underlying malignancy led to the discovery of early Waldenstrom's macroglobulinemia. Although leukocytoclastic vasculitis is more characteristic of mixed cryoglobulinemia, it can be a presenting manifestation of type 1 cryoglobulinemia.