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[多中心性网状组织细胞增多症(类脂质性皮肤关节炎)。3例的放射学研究]

[Multicentric reticulohistiocytosis (lipoid dermato-arthritis). A radiologic study of 3 cases].

作者信息

Fiumicelli A, Bruni L

机构信息

Istituto di Radiologia, Università, Perugia.

出版信息

Radiol Med. 1990 Sep;80(3):277-85.

PMID:2236686
Abstract

The authors report their experience with 3 cases of multicentric reticulohistiocytosis observed over 6 years of outpatient radiological practice. The condition presents with the following radiological patterns: 1) clear-cut erosions of the articular surfaces, especially in the distal interphalangeal joints of the hands and in the metatarso-phalangeal joints of the feet, with symmetrical distribution (not necessarily); 2) osteolytic punched-out areas in the epiphyseal spongiosa, ranging in size from 1 mm to over 1 cm; 3) no osteoporosis, no osteo-proliferative or periosteal reactions, not even in the presence of large osteoarticular destructions; 4) frequent atlanto-epistropheal subluxation; 5) articular ankylosis at the sacroiliac joints only. The association of the above patterns and the relatively benign clinical course distinguish multicentric reticulohistiocytosis from rheumatoid arthritis, psoriasis arthritis, erosive osteoarthritis, and gout. A reliable diagnosis can be suggested on the basis of radiological findings alone, even before cutaneous or mucosal lesions appear--which are, at any rate, not sure to appear and typical of nails only. An unquestionable diagnosis can be made at histology of synovial and/or cutaneous nodules. Multicentric reticulohistiocytosis is considered an uncommon condition (nearly 100 cases in international literature to 1989); the authors believe it to be commoner though often misdiagnosed as a "variant of rheumatoid arthritis".

摘要

作者报告了他们在6年门诊放射学实践中观察到的3例多中心性网状组织细胞增多症的病例。该病具有以下放射学表现:1)关节面有明确的侵蚀,特别是手部远端指间关节和足部跖趾关节,呈对称分布(不一定);2)骨骺松质骨内有溶骨性筛孔状区域,大小从1毫米到超过1厘米不等;3)无骨质疏松,无骨增殖或骨膜反应,即使存在较大的骨关节破坏也无;4)常出现寰枢椎半脱位;5)仅骶髂关节出现关节强直。上述表现与相对良性的临床病程相结合,可将多中心性网状组织细胞增多症与类风湿关节炎、银屑病关节炎、侵蚀性骨关节炎和痛风区分开来。仅凭放射学表现即可提出可靠的诊断,甚至在皮肤或黏膜病变出现之前——无论如何,这些病变不一定会出现,且仅典型地出现在指甲上。在滑膜和/或皮肤结节的组织学检查中可做出明确诊断。多中心性网状组织细胞增多症被认为是一种罕见疾病(截至1989年国际文献报道近100例);作者认为它更为常见,尽管常被误诊为“类风湿关节炎的一种变体”。

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