Hypergammaglobulinemic purpura: Does hypergammaglobulinemia cause purpura?
Hypergammaglobulinemic purpura: Does hypergammaglobulinemia cause purpura?
复制标题
高丙种球蛋白血症性紫癜：高丙种球蛋白血症会引起紫癜吗？
DOI：
10.1111/1346-8138.16122
复制
发表时间：
2021
期刊：
影响因子：
--
通讯作者：
Ishiguro Naoko
中科院分区：
文献类型：
--
作者：
Kimura Keiko;Miyabe Chie;Miyata Ryujin;Fukuya Yasuko;Ishiguro Naoko
尊敬的编辑，瓦尔登斯特伦高丙种球蛋白血症性紫癜（HP）是一种以复发性下肢紫癜伴高丙种球蛋白血症为特征的综合征。 1 HP 可能是原发性疾病，但有时与其他疾病相关，例如干燥综合征 (SS)、系统性红斑狼疮 (SLE)、类风湿性关节炎 (RA) 和丙型肝炎。 1 据推测，高水平的循环免疫球蛋白 (Ig) 通过免疫复合物形成和血管壁沉积与 HP 发病机制相关。 2 然而，尚无证据表明高丙种球蛋白血症直接导致 HP。因此，我们试图探讨皮肤症状与高丙种球蛋白血症之间的相关性。我科确诊的HP患者9例（男4例，女5例），年龄21～87岁（平均56岁）。在所有病例中，血清 IgG 水平均超过 2000 mg/dL（正常值，870-1700）。 5 例诊断为 SS，2 例诊断为 RA，1 例诊断为丙型肝炎。SS 患者中有 4 例（80%）存在抗 SSA（Ro）和抗 SSB（La）抗体。大多数患者腿部出现双侧瘀点，长时间站立或行走会加剧瘀点。组织学上，所有病例均观察到真皮上层至中层小血管周围有炎症细胞浸润。
Dear Editor, Hypergammaglobulinemic purpura (HP) of Waldenström is a syndrome characterized by recurrent lower extremity purpura with hypergammaglobulinemia. 1 HP can be a primary disease, but is sometimes associated with other diseases, such as Sjögren’s syndrome (SS), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and hepatitis C. 1 It has been hypothesized that high levels of circulating immunoglobulins (Ig) are associated with HP pathogenesis via immune complex formation and deposition in the vessel walls. 2 However, evidence showing that hypergammaglobulinemia directly causes HP is absent. Therefore, we sought to explore the correlation between skin symptoms and hypergammaglobulinemia. Of the nine patients diagnosed with HP in our department (four men and five women), the age ranged from 21 to 87 years (mean, 56). In all cases, serum IgG levels were more than 2000 mg/dL (normal, 870–1700). Five cases were diagnosed with SS, two with RA, and one with hepatitis C. Anti-SSA (Ro) and anti-SSB (La) antibodies were present in four cases among the patients with SS (80%). Most patients showed bilateral petechiae on their legs, which were exacerbated by prolonged standing or walking. Histologically, infiltration of inflammatory cells around the small vessels in the upper to middle dermis was observed in all cases.