Two cases of synovitis, acne, pustulosis, osteitis--SAPHO syndrome

Synovitis, acne, pustulosis, osteitis (SAPHO) syndrome is rare with yet unknown prevalence. The difficulty in recognizing the syndrome is due to the very wide diversity of its signs and symptoms, the lack of skin manifestations in many cases and to confusion in medical terminology in describing this syndrome. In this paper, we present two cases with characteristic bone lesions in bone scan and in radiology images that are considered to be SAPHO syndrome. In the first case the characteristic bone single photon emission tomography scan findings in a patient with spine involvement supposed by bone biopsy but were not followed by characteristic skin manifestations. The point of interest of this case lies on the significant improvement of both symptoms and scintigraphic findings after treatment with biphosphonates. In the second case the diagnosis was also based on the characteristic bone scan findings, although the patient referred to us for staging of prostate cancer. Detailed history and clinical examination revealed skin manifestations of the syndrome.     

SAPHO syndrome: MR appearance of vertebral involvement

PURPOSE: To retrospectively evaluate the magnetic resonance (MR) imaging findings of vertebral involvement in patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. MATERIALS AND METHODS: Ethics committee approval and informed patient consent were not required for this retrospective study. MR images obtained in 12 patients (seven female, five male; mean age, 42 years; range, 16-65 years) with SAPHO syndrome involving the spine were reviewed. One vertebral lesion separated by one or more normal vertebrae was analyzed as a distinct lesion. For each lesion, the number of associated vertebrae with abnormal signal intensity (SI) (ie, single vertebra, two adjacent vertebrae, or more than two adjacent vertebrae) was noted. The following MR imaging findings were evaluated: cortical bone erosion, abnormal vertebral body SI compared with normal vertebral body SI, increased anteroposterior diameter of the vertebral body, soft-tissue involvement, vertebral body height loss of more than 30%, and abnormal SI of the adjacent intervertebral disk compared with the SI of the other disks. RESULTS: Of 24 vertebral lesions found, 17 involved a single vertebra, four involved two adjacent vertebrae, and three involved three or four adjacent vertebrae. Vertebral corner cortical erosion was present in all lesions, and 23 (96%) lesions had anterior vertebral corner involvement. The erosion was confined to a vertebral corner in five (21%) lesions and included the adjacent endplate and/or the anterior cortex of the vertebral body in the remaining 19 (79%) lesions. In four (17%) lesions, involvement of two adjacent vertebral corners on either side of an intervertebral disk mimicked to some extent early disk space infection. An adjacent disk space was narrowed in six (25%) lesions and exhibited abnormal SI in two (8%). Prevertebral tissue thickening was observed in eight (33%) lesions. CONCLUSION: Erosion of a vertebral body corner is consistently seen on MR images of SAPHO vertebral lesions and may support the diagnosis of SAPHO syndrome in the appropriate clinical context.     

Vertebral involvement in SAPHO syndrome: MRI findings

We report on the MRI findings in the vertebrae and surrounding soft tissues in two patients with the SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis). The MRI findings include abnormal bone marrow signal, either focal or diffuse, of the vertebral bodies and posterior elements; hyperintense paravertebral soft tissue swelling and abnormal signal of the intervertebral discs. These changes are consistent with discitis and osteitis. Received: 27 July 1998 Revision requested: 14 October 1998 Revision received: 23 November 1998 Accepted: 24 November 1998     

获得性骨肥大综合征1例

获得性骨肥大综合征,即滑膜炎、痤疮、脓包疮、骨肥厚、骨炎综合征(synovitis,acne,pustulosis,hyperostosis,osteitis,SAPHO),包括骨关节病变和皮肤病变,是一种少见的原因未明的慢性进行性骨病,笔者遇到1例,现报告如下。患者男,50岁。因右胸锁关节处隆起、压痛、翻身疼痛难忍逐渐加重2年而来就诊。自述两年前曾因此处疼痛在当地医院就诊,诊为“畸形性骨炎”,用抗炎药物治疗后,症状有缓解。但近年来上述症状逐渐加重遂来本院就诊。检查:前上胸、双侧锁骨区及胸锁关节处隆起,以右胸锁关节隆起明显,局部皮肤无红、热,触诊质地硬,有压痛,无波…     

SAPHO综合征1例报道及文献综述

SAPHO综合征是累及骨、关节及皮肤的一系列病变的总称.骨关节病变没有特异性,易与其他疾病发生混淆.为加深对SAPHO综合征的认识.更好地做出早期正确诊断,本文报道了一例SAPHO综合征,并对其临床及影像学表现做了综述.     

Van Buchem's disease (hyperostosis corticalis generalisata)

The features of Van Buchem's disease are described, affecting six members of one family, spanning three generations of ages from 7 to 64 years.     

Generalized cortical hyperostosis (Van Buchem disease): nosologic considerations.

Six of 41 presumed cases of Van Buchem disease described in the literature fit uniform diagnostic criteria. Segregation analysis of these 6 cases, in addition to another the authors report, supports a recessive mode of inheritance. Genetic heterogeneity is confirmed by the demonstration of a dominantly-inherited phenotype resembling Van Buchem disease. The probable etiology is a defect in the endochrondral modulatory step regulating transformation of osteoclast to osteoblast.     

Cervical involvement in SAPHO syndrome: imaging findings with a 10-year follow-up

Osteoarticular manifestations of SAPHO syndrome include vertebral lesions, typically in the thoracic segment. Chronic inflammatory changes are well depicted by MRI. We report the imaging findings with a 10-year follow-up in a case of SAPHO syndrome with marked cervical lesions.     

Villonodular synovitis (PVNS) of the spine

Objective To describe the imaging features of spinal pigmented villonodular synovitis (PVNS).Design and patients We retrospectively reviewed 15 cases of pathologically proven spinal PVNS. Patient demographics and clinical presentation were reviewed. Radiologic studies were evaluated by consensus of two musculoskeletal radiologists for spinal location, spinal segments affected, lesion center, detection of facet origin and intrinsic characteristics on radiography (n =11), myelography (n =7), CT (n =6) and MR imaging (n =6).Results Women (64%) were more commonly affected than men (36%) with an average age of 28 years. Clinical symptoms were pain (45%), neurologic (9%) or both (36%). Lesions most frequently affected the cervical spine (53%) followed by the thoracic (27%) and lumbar regions (20%). The majority of lesions (93%) were centered in the posterior elements with frequent involvement of the pedicle (67%), neural foramina (73%), lamina (67%) and facets (93%). No lesions showed calcification. Determination of a facet origin by imaging was dependent on imaging modality and lesion size. A facet origin could be determined in 45% of cases by radiography vs 67% of patients by CT (n=6) and MR (n=6). Large lesions (greater than 3 cm in at least one dimension) obscured the facet origin in all cases with CT and/or MR imaging (44%,n=4). Small lesions (less than 3 cm in any dimension) demonstrated an obvious facet origin in all cases by CT and/or MR imaging (56%,n=5). Low-to-intermediate signal intensity was seen in all cases on T2-weighted MR images resulting from hemosiderin deposition with blooming effect in one case with gradient echo MR images.Conclusions PVNS of the spine is rare. Large lesions obscure the facet origin and simulate an aggressive intraosseous neoplasm. Patient age, a solitary noncystic lesion centered in the posterior elements, lack of mineralization and low-to-intermediate signal intensity on all MR pulse sequences may suggest the diagnosis in these cases. Small lesions demonstrate a facet origin on CT or MR imaging. This limits differential considerations to synovial-based lesions and additional features of a solitary focus, lack of underlying disease or systemic arthropathy, no calcification as well as low-to-intermediate signal intensity on all MR images should allow spinal PVNS to be suggested as the likely diagnosis.The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Departments of the Air Force, Army, Navy or Defense.     

Scintigraphic aspect of infantile cortical hyperostosis (Caffey's disease)

Two patients with infantile cortical hyperostosis (Caffey's disease) are presented in whom radionuclide imaging with 99mTc-MDP and 67Gallium-citrate was performed to eliminate multifocal osteomyelitis as a diagnosis. The scintigraphic appearance of this relatively uncommon disease of children was similar to that obtained in multifocal osteomyelitis; all osseous lesions showed a marked uptake of both radiotracers. However, the distribution of abnormalities (mandible, scapulae) and the degree of extension (mainly diaphyseal) of the lesions are important characteristics in the scintigraphic differential diagnosis but bone radiographs are best used in making the diagnosis.     

Concentric joint space narrowing of the hip associated with hemosiderotic synovitis (HS) including pigmented villonodular synovitis (PVNS)

Concentric joint space narrowing of the hip is an expected radiographic finding in cases of inflammatory arthritis such as rheumatoid arthritis or sepsis. However, similar joint space narrowing is associated with chronic hemorrhagic conditions that produce hemosiderotic synovitis. Hemosiderotic synovitis results from chronic intraarticular bleeding such as occurs in pigmented villonodular synovitis, generalized bleeding diathesis, synovial hemangioma, and chronic trauma. Five hips in five patients with concentric joint space narrowing not associated with inflammatory arthritis or with hemophilia were reviewed clinically, radiographically, and pathologically. All patients had a hemosiderotic synovitis. The definitive diagnosis of pigmented villonodular synovitis was made pathologically in two cases that demonstrated nodular areas of giant cell proliferation, collagen production, and lipid-laden histiocytes on histologic samples.