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1/27. Recurrent subcutaneous abscess of the sternal region in ulcerative colitis.

    An 18-yr-old female patient with extensive ulcerative colitis suffered from several episodes of recurrent aseptic subcutaneous abscesses of the sternal region with a course paralleling that of her colitis. The abscess seemed to occur secondarily to osteomyelitis of the sternum, which is a manifestation of the synovitis, acne, pustulosis, hyperostosis, and osteomyelitis (SAPHO) syndrome.
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keywords = hyperostosis
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2/27. Cervical spinal cord injury in sapho syndrome.

    Cervical spinal fracture and pseudarthrosis are previously described causes of spinal cord injury (SCI) in patients with spondylarthropathy. SAPHO (synovitis Acne Pustulosis hyperostosis osteitis) syndrome is a recently recognized rheumatic condition characterized by hyperostosis and arthro-osteitis of the upper anterior chest wall, spinal involvement similar to spondylarthropathies and skin manifestations including palmoplantar pustulosis and pustular psoriasis. We report the first case of SAPHO syndrome disclosed by SCI related to cervical spine ankylosis.
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3/27. The SAPHO syndrome in children: a rare cause of hyperostosis and osteitis.

    The SAPHO syndrome is a rare constellation of signs and symptoms characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis. The most common musculoskeletal complaints are hyperostosis, causing pain, tenderness, and swelling of the anterior chest wall, although any part of the axial and appendicular skeleton may be affected. There is a great degree of variability in the dermatologic involvement of this syndrome. A combination of clinical, radiographic, and pathological investigation is required to establish the correct diagnosis. No single treatment has been found to be effective, although nonsteroidal antiinflammatory drugs have been the most frequently used. Because there is no mention of SAPHO syndrome in the English orthopaedic literature, and pediatric orthopaedic surgeons may be the first caregivers to treat these children, we thought it appropriate to share our experience with a 5-year-old boy with SAPHO syndrome recently under our care.
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keywords = hyperostosis
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4/27. SAPHO syndrome of the temporomandibular joint associated with sudden deafness.

    We report a case of arthritis of the temporomandibular joint (TMJ) associated with sclerosing osteomyelitis of the mandible and temporal bone, causing deafness. The presence of a palmoplantar pustulosis established the diagnosis of SAPHO syndrome. SAPHO (an acronym referring to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis) syndrome is defined by the association of characteristic osteoarticular and dermatologic manifestations, with diffuse sclerosing osteomyelitis of the mandible being a part of this entity. We review the literature of SAPHO syndrome with mandibular manifestations and discuss the mechanisms of inflammatory spread from the TMJ to the cochlea. To our knowledge, this is the first description of skull base involvement in a patient with SAPHO syndrome leading to sudden deafness.
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keywords = hyperostosis
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5/27. SAPHO syndrome or psoriatic arthritis? A familial case study.

    OBJECTIVE: To discuss the relationships between SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome and the group of spondylarthropathies. methods: Few reports of familial SAPHO have been published. We describe three children, two sisters and one brother, whose clinical and radiological presentation was in accordance with SAPHO syndrome. RESULTS: Two children developed psoriasis, and one child palmoplantar pustulosis. Both sacroiliac and sternoclavicular joints were involved in these three cases. Some features in our observations are also common to psoriatic arthritis. No association was found with hla antigens, but a history of trauma preceding the onset of symptoms was present in all three children. CONCLUSIONS: We can consider that SAPHO is nosologically related to spondylarthropathies. Psoriatic arthritis could be the missing link between SAPHO and spondylarthropathies. It is likely that both genetic and environmental factors are involved.
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6/27. SAPHO syndrome: report of three cases and review of the literature.

    SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is an acronym that describes a seronegative syndrome of bone and joint lesions, frequently localized to the anterior chest wall, associated with variable dermatologic manifestations. Dermatologists should be aware of this entity, as early diagnosis can prevent unnecessary surgery and avoid prolonged ineffective antibiotic therapy. We report three new cases and review the literature.
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7/27. Primary chronic osteomyelitis associated with synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO syndrome).

    Diffuse sclerosing osteomyelitis may indicate the mandibular localisation of the SAPHO syndrome. Twelve patients with diffuse sclerosis of the mandible were examined for symptoms of the SAPHO syndrome. Nine patients were found to have primary chronic osteomyelitis and eight of these represented a SAPHO syndrome. Results in this series support the hypothesis of an association between primary chronic osteomyelitis and the SAPHO syndrome.
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ranking = 4
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8/27. Diffuse chronic sclerosing osteomyelitis and the synovitis, acne, pustolosis, hyperostosis, osteitis (SAPHO) syndrome in two sisters.

    Two sisters with diffuse chronic sclerosing osteomyelitis of the mandible and the humerus and the synovitis, acne, pustolosis, hyperostosis and osteitis syndrome (SAPHO syndrome) are presented. The diagnoses of diffuse chronic sclerosing osteomyelitis at the age of 12 years and 27 years, respectively, were based on typical medical history, clinical symptoms and radiographic, histologic and scintigraphic findings. Because skin lesions and scintigraphic enhancement of the sternoclavicular joints with hyperostosis were present, a SAPHO syndrome was diagnosed in both sisters. Microbiological cultures of biopsy specimens revealed coagulase-negative staphylococcus aureus at the humerus and haemophilus parainfluenzae, streptococcus, actinomyces and Veilonella species at the mandible. Repeated operative procedures, including decortications, resection and reconstruction, and multiple histologic and microbiologic studies were performed over a period of up to 20 years. Since HLA typing yielded identical gene loci, we suggest that hereditary and autoimmune factors may play a role in the pathogenesis of these cases.
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ranking = 6
keywords = hyperostosis
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9/27. synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome in childhood: a report of ten cases and review of the literature.

    Chronic recurrent multifocal osteomyelitis is a rare chronic inflammatory musculoskeletal process observed in children and young adults. Recently, the acronym SAPHO syndrome (for synovitis, acne, pustulosis, hyperostosis, osteitis) was coined to emphasise the association between osteo-articular inflammations and different skin abnormalities which are aseptic and filled with neutrophils. In adults, chronic recurrent multifocal osteomyelitis is now a classical manifestation of SAPHO syndrome. Chronic skin disorders were seen in eight of ten children on follow-up at the University Children's hospitals in Bern and Zurich and in 61 of 260 paediatric cases reported in the literature. The different skin lesions were palmoplantar pustulosis (n = 40), non-palmoplantar pustulosis (n = 6), psoriasis vulgaris (n = 16) or severe acne (n = 4). More rarely sweet syndrome (n = 2) or pyoderma gangrenosum (n = 1) were reported. Conclusion: The synovitis, acne, pustulosis, hyperostosis, osteitis syndrome is pertinent even in paediatrics since skin involvement is frequent.
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ranking = 6
keywords = hyperostosis
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10/27. synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome presenting as a primary calvarial lesion. Case report and review of the literature.

    The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a recently described, currently evolving clinical entity that groups together several idiopathic disorders of bone and skin formerly described under a variety of names. Among the spectrum of possible locations for the bone lesions, there is no previous report in the literature of primary involvement of the skull vault. A patient with primary involvement of the calvaria in the setting of SAPHO syndrome is described here, which, to the authors' knowledge, is the first report of such localization. The clinically and radiologically benign evolution of the different stages of the bone lesions is presented. The authors suggest that the SAPHO syndrome should be considered in the differential diagnosis of lytic, sclerotic, or hyperostotic lesions of the skull, particularly before considering invasive diagnostic procedures.
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ranking = 5
keywords = hyperostosis
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