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复发性口疮:白塞病的顿挫型?

Complex aphthosis: a forme fruste of Behçet's syndrome?

作者信息

Jorizzo J L, Taylor R S, Schmalstieg F C, Solomon A R, Daniels J C, Rudloff H E, Cavallo T

出版信息

J Am Acad Dermatol. 1985 Jul;13(1):80-4. doi: 10.1016/s0190-9622(85)70147-8.

DOI:10.1016/s0190-9622(85)70147-8
PMID:4031155
Abstract

The evaluation of the rare patient who presents with oral and genital aphthae or almost constant, multiple (greater than 3) oral aphthae, but no systemic signs or symptoms (i.e., complex aphthosis), is difficult because no laboratory test is available to exclude Behçet's syndrome. Six patients with complex aphthosis were evaluated. In addition, patients with simple aphthosis, those with seronegative arthritis, and normal controls were assessed for circulating immune complexes (CIC) by in vitro and in vivo assays and for neutrophil migration by subagarose methods, since these tests have given significant results in patients with Behçet's syndrome. Patient 1, with complex aphthosis, had Raji cell evidence for CIC (51.2 mg aggregated human gamma globulin Eq/ml), C1q, and C3 in dermal blood vessels 4 hours post intradermal histamine injection and had a Sweet's syndrome-like vasculitis 24 hours post histamine injection. In addition, her serum enhanced the migration of patient neutrophils (3.6 +/- 0.6 to 4.6 +/- 0.5; N = 6, p less than or equal to 0.01). All other test and control patients had negative or normal CIC and neutrophil migration determinations. Sixteen-month clinical follow-up has confirmed that Patient 1, but not Patients 2 to 6, has developed overt manifestations of Behçet's syndrome.

摘要

对出现口腔和生殖器阿弗他溃疡或几乎持续存在多个(大于3个)口腔阿弗他溃疡但无全身体征或症状(即复杂性阿弗他病)的罕见患者进行评估很困难,因为没有实验室检查可用于排除白塞病。对6例复杂性阿弗他病患者进行了评估。此外,对单纯性阿弗他病患者、血清阴性关节炎患者和正常对照者,通过体外和体内试验评估循环免疫复合物(CIC),并通过琼脂糖下方法评估中性粒细胞迁移,因为这些检查在白塞病患者中已得出显著结果。患者1患有复杂性阿弗他病,在皮内注射组胺后4小时,Raji细胞检测显示其真皮血管中有CIC(51.2mg聚集人γ球蛋白当量/ml)、C1q和C3,且在注射组胺后24小时出现类Sweet综合征的血管炎。此外,她的血清增强了患者中性粒细胞的迁移(从3.6±0.6增至4.6±0.5;N = 6,p≤0.01)。所有其他测试患者和对照患者的CIC及中性粒细胞迁移测定均为阴性或正常。16个月的临床随访证实,患者1已出现白塞病的明显表现,而患者2至6未出现。

相似文献

1
Complex aphthosis: a forme fruste of Behçet's syndrome?复发性口疮:白塞病的顿挫型?
J Am Acad Dermatol. 1985 Jul;13(1):80-4. doi: 10.1016/s0190-9622(85)70147-8.
2
Behçet's disease and complex aphthosis.白塞病与复杂性口疮
J Am Acad Dermatol. 1999 Jan;40(1):1-18; quiz 19-20. doi: 10.1016/s0190-9622(99)70523-2.
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Complex aphthosis and Behçet's disease.复发性口疮与白塞病。
Dermatol Clin. 2003 Jan;21(1):41-8, vi. doi: 10.1016/s0733-8635(02)00058-x.
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Behçet's syndrome: immune regulation, circulating immune complexes, neutrophil migration, and colchicine therapy.白塞病:免疫调节、循环免疫复合物、中性粒细胞迁移及秋水仙碱治疗
J Am Acad Dermatol. 1984 Feb;10(2 Pt 1):205-14. doi: 10.1016/s0190-9622(84)70024-7.
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[Dermato-mucosal manifestations of Behçet's disease].白塞病的皮肤黏膜表现
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Thalidomide effects in Behçet's syndrome and pustular vasculitis.沙利度胺对白塞病和脓疱性血管炎的影响。
Arch Intern Med. 1986 May;146(5):878-81.
7
Complex aphthosis: a large case series with evaluation algorithm and therapeutic ladder from topicals to thalidomide.复杂性口疮:一个包含评估算法和从局部用药到沙利度胺治疗阶梯的大型病例系列。
J Am Acad Dermatol. 2005 Mar;52(3 Pt 1):500-8. doi: 10.1016/j.jaad.2004.10.863.
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Bipolar aphthosis. A forme fruste of Behçet's disease. Long term follow-up of 26 cases.双相口疮病。白塞病的顿挫型。26例长期随访
Adv Exp Med Biol. 2003;528:321-2. doi: 10.1007/0-306-48382-3_63.
9
Behçet's disease and complex aphthosis.白塞病与复杂性口疮
Dermatol Clin. 1987 Oct;5(4):769-78.
10
[Apthae: histologic, immunofluorescent and immuno--electron microscopy study of their pathogenesis].
Hautarzt. 1981 Jul;32(7):364-9.

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