Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis Syndrome Research Articles

Tumor Necrosis Factor-α Blockers in SAPHO Syndrome: Table 1.

To analyze the clinical efficacy of anti-tumor necrosis factor-alpha (TNF-alpha) therapy in treatment of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome, we describe cases of refractory SAPHO syndrome and review cases treated with anti-TNF-alpha reported in the literature. We describe 6 cases of patients with SAPHO syndrome treated with anti-TNF-alpha between 2004 and 2008. Therapeutic response was evaluated according to improvement in pain score, amelioration of disease activity, and improvement in function. The efficacy of treatment was considered to be reduced need for analgesics and/or antiinflammatory therapy. In our series, 4 patients received infliximab, 1 etanercept, and 1 adalimumab. These treatments brought clinical response in 4 patients (66.6%): response was sustained with infliximab in 1 case for 7 months; with adalimumab in another case for 22 months; and with etanercept in 2 cases for 1 and 42 months, respectively. In contrast, 2 other patients showed no response to infliximab. Improvement was initially temporary after infusions 1 and 2, then pain recurred at Week 14. Skin lesions were healed in 3 of 4 cases, but recurred or worsened in 2 cases, after infusion 2 of infliximab. Treatment was generally well tolerated. Paradoxical psoriasis was noted in 2 cases and urticaria in 1. Given our results and those from the literature, TNF-alpha blockers should be considered in the therapeutic strategy of refractory cases of SAPHO syndrome, despite their effect seeming less impressive than in other spondyloarthropathies.

SAPHO syndrome in an adult with ulcerative colitis responsive to intravenous pamidronate

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare group of sterile, inflammatory osteoarticular disorders classically associated with skin lesions. It is occasionally associated with enteropathic disease such as ulcerative colitis. We present a 39-year-old patient with chronic ulcerative colitis who developed enteropathic SAPHO and responded well to pamidronate. We discuss the clinicopathological features with particular attention to bone pathology, and perform a literature review of this fascinating syndrome.

Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome complicated by seven pulmonary emboli in a 15-year old patient

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome represents a spectrum of various dermatologic and musculoskeletal conditions. Thromboses have infrequently been reported in SAPHO syndrome, most often in the subclavian vein. There have been no reported cases of pulmonary emboli associated with SAPHO. We report a case of a young patient with SAPHO syndrome who later presented with extensive iliofemoral deep vein thromboses and seven pulmonary emboli.

A case of SAPHO syndrome with destructive spondylodiscitis suspicious of tuberculous spondylitis

We report a rare case of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome in which the differential diagnosis included tuberculous spondylitis and the patient ultimately required reconstructive spinal surgery. The patient was a 60-year-old woman who presented with severe low-back and leg pain after treatment for tuberculosis. Roentgenography and magnetic resonance imaging of the lumbar spine revealed destructive changes suggestive of tuberculous spondylitis. [(18)F]-fluoro-2-deoxyglucose positron emission tomography/computed tomography showed uptake in the cervical spines, lumbar spines, and sacroiliac joints from which she was suspected of having SAPHO syndrome without skin manifestations. However, as her symptoms did not respond to conservative treatment, we performed reconstructive surgery of the lumbar spine. Spinal specimens obtained surgically showed nonspecific inflammation and fibrous hypertrophy of the bone marrow, confirming a diagnosis of the SAPHO syndrome. Her symptoms improved markedly after surgery, although she required occasional prednisolone for moderate polyarthralgia and leg pain.

Mucosal lesions may be a minor complication of SAPHO syndrome: a study of 11 Japanese patients with SAPHO syndrome

Since the term synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome was proposed by Chamot et al. (Rev Rhum Mal Osteoartic 54:187-196, 1987), clinical reviews concerning this syndrome have been mainly reported from Europe. We carried out a retrospective analysis of 11 Japanese patients with SAPHO syndrome, and reviewed the clinical features of our series in comparison with those in a European large case study. In this study the major features of SAPHO syndrome were chronic osteitis of the anterior chest wall and pustulotic arthro-ostitis with middle age onset, and mucosal lesions seemed to be a minor complication of SAPHO syndrome. The non-erosive peripheral large joints arthritis and the particular HLA types (HLA-B51, B52, or A26), which had been reported to be increased in Behcet's disease, were frequently seen in SAPHO syndrome with mucosal lesions. This study also suggests that SAPHO syndrome with mucosal lesions may be part of a broader disease spectrum including Behcet's disease.

Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome with Associated Neutrophilic Dermatoses: A Report of Seven Cases and Review of the Literature

A growing body of literature has identified the association between neutrophilic dermatoses and multifocal, aseptic bone lesions in children, termed chronic recurrent multifocal osteomyelitis (CRMO). Classically, patients present with swelling, pain, and impaired mobility of the affected area, with skin lesions developing concurrently or in the future. Bone biopsy reveals inflammatory changes consistent with infectious osteomyelitis, but cultures and histologic staining invariably fail to identify an infectious source. Patients are refractory to antibiotic therapy, but dramatically respond to systemic steroids and may need to be maintained on low-dose steroids to prevent relapse. Numerous authors have suggested that CRMO and synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome lie along the same clinical spectrum. In fact some believe that CRMO is the pediatric presentation of SAPHO. The two syndromes share numerous characteristics, including osteitis, a unifocal or multifocal presentation, hyperostosis, and pustulosis, which all occur in a generally healthy individual. Our seven patients, five of whom were diagnosed with CRMO, and two of whom were diagnosed with SAPHO syndrome further strengthen the idea that CRMO and SAPHO syndrome do indeed lie along the same clinical spectrum. In addition, we include two rare cases of pediatric Sweet's syndrome with evidence of pathergy.

Expression of tumor necrosis factor-alpha converting enzyme and matrix metalloproteinase-3 in proliferated synovium in a patient with synovitis-acne-pustulosis-hyperostosis-osteitis syndrome: a case report

IntroductionSynovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a rare disorder. The etiology remains unknown and the treatment is still empirical. Synovitis is one of the major manifestations, but information on histopathological features is still lacking. In this case, we investigated the histopathological features of SAPHO syndrome synovitis.Case presentationWe present the case of a 53-year-old Japanese woman with SAPHO syndrome accompanied by marked knee synovitis and palmoplantar pustulosis. We found abundant sterile joint fluid in the right knee, and a blood test showed abnormally high values of C-reactive protein (17.26 mg/dl) and matrix metalloproteinase-3 (800 ng/ml). Arthroscopic surgery revealed marked proliferation of villous synovial tissues similar to rheumatoid arthritis and standard microscopic findings were also similar to rheumatoid arthritis. Furthermore, for the first time, we demonstrated by immunohistochemistry the expression of tumor necrosis factor-alpha (TNF-α) converting enzyme, TNF-α and matrix metalloproteinase-3 in the proliferated synovial lining cells. After arthroscopic synovectomy, her knee symptoms immediately diminished and laboratory data (matrix metalloproteinase-3 and C-reactive protein) normalized within 2 weeks of surgery.ConclusionWe demonstrate the expression of TNF-α converting enzyme, TNF-α and matrix metalloproteinase-3 in SAPHO syndrome synovitis for the first time and also show, both macro- and microscopically, the similarity between SAPHO syndrome and rheumatoid arthritis synovitis. These new findings support the recently reported successful treatment of SAPHO syndrome with antirheumatic drugs, especially with anti-TNF-α agents.

SAPHO syndrome in an adult with ulcerative colitis responsive to intravenous pamidronate: a case report and review of the literature

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare group of sterile, inflammatory osteoarticular disorders classically associated with skin lesions. It is occasionally associated with enteropathic disease such as ulcerative colitis. We present a 39-year-old patient with chronic ulcerative colitis who developed enteropathic SAPHO and responded well to pamidronate. We discuss the clinicopathological features with particular attention to bone pathology, and perform a literature review of this fascinating syndrome.

ASEPTIC MENINGITIS AS A RARE MANIFESTATION OF SAPHO SYNDROME

Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is characterized by multifocal aseptic osteomyelitis of an uncertain etiology. In 1987, it was first proposed as a unifying concept to describe the association between musculoskeletal disorders and various dermatologic conditions.1 Recently, we encountered a patient with SAPHO syndrome who exhibited aseptic meningitis and lower cranial nerve palsies. ### Case report. A 52-year-old woman was admitted to our hospital because of severe headache and a mild fever of 37.5°C. Her past and family histories were unremarkable; however, 2 months before admission, she felt some precordial pain and noticed eruptions on her palms and soles of her feet. On admission, her general condition was unremarkable. There were no palpable lymph nodes, though there were a few pustular eruptions on her palms and soles. On neurologic examination, she had no neurologic deficits except for mild neck stiffness. Brain CT and MRI results were normal. Her CSF showed a mild pleocytosis (101/mm3, 98% lymphocytes, 2% polymorphs), and slightly high protein (52 mg/dL) and normal glucose (58 mg/dL) levels. CSF culture was negative. PCR tests for herpes simplex virus and anti-varicella zoster virus antibody in the CSF were also negative. She was diagnosed with …

SAPHO syndrome associated spondylitis

The concept of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome has been well clarified, after Chamot et al. suggested this peculiar disorder in 1987. The most commonly affected site in SAPHO syndrome is the anterior chest, followed by the spine. However, the clinical course and taxonomic concept of SAPHO spinal lesions are poorly understood. This study was performed to analyze: (1) the detailed clinical course of spinal lesions in SAPHO syndrome, and (2) the relationship between SAPHO syndrome with spinal lesions and seronegative spondyloarthropathy. Thirteen patients with spondylitis in SAPHO syndrome were analyzed. The features of spinal lesions were a chronic onset with a slight inflammatory reaction, and slowly progressing non-marginal syndesmophytes at multi spinal levels, besides the coexistence of specific skin lesions. SAPHO syndrome, especially spinal lesions related to palmoplantar pustulosis, can be recognized as a subtype of seronegative spondyloarthropathy.

Characterization of the immune response in the synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome

The aetiology of SAPHO (synovitis, acne, palmoplantar pustulosis, hyperostosis, osteitis) syndrome seems to involve genetic, infectious and immunological components. We examined innate and adaptive immune responses in SAPHO syndrome, as compared with PsA and RA. We also studied the effect of etanercept on immunological parameters. We studied 29 patients with SAPHO syndrome, as well as 22 patients with RA, 21 patients with PsA and 15 healthy controls. Adaptive immune responses were investigated by assaying total serum immunoglobulins and several autoantibodies. Innate immunity was studied by quantifying blood PMN functions and plasma cytokine levels. PMN responses to Propionibacterium acnes were tested ex vivo. Eight patients who received etanercept for refractory rheumatic disorders were tested before and after 28 days of treatment. SAPHO syndrome was associated with elevated IL-8 and IL-18 plasma levels. IL-8 and TNF-alpha production by purified PMN was higher in the three patient groups than in the healthy controls, but the oxidative burst and IL-18 production were normal. No autoantibodies were detected in SAPHO patients. Induction of PMN IL-8 and TNF-alpha production by P. acnes was impaired in the SAPHO group as compared with the RA and PsA groups. After 28 days of etanercept therapy, PMN IL-8 and TNF-alpha production was down-regulated and TNF-alpha plasma levels were increased. These results support the view that the SAPHO syndrome may be triggered by an infectious state involving P. acnes, contributing to the strong humoral and cellular pro-inflammatory responses. Etanercept modulation of PMN activation status emphasizes these new immunological findings.

SAPHO syndrome with rapidly progressing destructive spondylitis: two cases treated surgically

The authors present two cases of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome with rapidly progressing destructive spondylitis treated surgically. The spinal lesions in SAPHO syndrome generally have a good prognosis and rarely cause the structural destruction or neurological deterioration. Case 1: a 63-year-old female had palmoplantar pustulosis for 2 years. At first, she only felt a pain in the nape with no inducing factor. Two months later, she had incomplete quadriplegia (ASIA scale C). Magnetic resonance imaging showed destruction of C4-C7, kyphotic deformity, and severe compression of the spinal cord. Decompression and reconstruction surgery using anterior and posterior approach improved her paralysis. Case 2: a 69-year-old female complained of persistent back pain. Magnetic resonance imaging revealed spondylitis of T7-T9. Although there were no typical skin lesions, we diagnosed SAPHO syndrome by hyperostosis of the sternocostoclavicular joint and sacral joint. Destruction with kyphotic deformity of the spine progressed gradually for 3 months. Curettage and reconstruction surgery using thoracic endoscope relieved her pain and prevented the destruction of the spine. The histopathology of the specimen obtained surgically showed non-specific inflammation in both cases. Spondylitis in SAPHO syndrome may cause severe destruction and kyphotic deformity followed by paralysis.

Tumor Necrosis Factor-α Inhibitor-Induced Psoriasis or Psoriasiform Exanthemata

Tumor necrosis factor-alpha (TNFalpha) inhibition is effective in the treatment of moderate-to-severe psoriasis. We report on 120 patients from the literature including six new patients (three women and three men) who developed pustular lesions during treatment with TNFalpha inhibitors. We identified 72 women and 36 men (several papers did not specify the gender of patients) with an age range of 13-78 years (mean 42.3 years). The primary diagnoses were rheumatoid arthritis (n = 61), ankylosing spondylitis (n = 21), psoriasis (n = 10), Crohn disease (n = 8), SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome (n = 3), psoriatic arthritis (n = 2), and other diagnoses (n = 15). Psoriasis (except palmoplantar pustular type) was the most common adverse effect during anti-TNFalpha treatment (n = 73), followed by palmoplantar pustular psoriasis (n = 37) and psoriasis of the nail (n = 6), sometimes combined in the same patient. Palmoplantar pustulosis and psoriasiform exanthema was the diagnosis in ten patients each. A positive personal history of psoriasis was recorded in 25 patients. A positive family history was noted in eight patients. No data about personal (n = 7) or family history (n = 46) were available in a number of patients. Newly induced psoriasis was diagnosed in 74 patients whereas an exacerbation or aggravation of a pre-existing psoriasis was noted in another 25 patients. All three TNFalpha inhibitors available on the market were involved: infliximab (63 patients), etanercept (37 patients), and adalimumab (26 patients). Several patients were treated with more than a single TFNalpha inhibitor. The timing of cutaneous adverse effects (psoriasis and psoriasiform rash) varied considerably among patients, ranging from after a single application to a delayed response of up to 63 months after initiation of treatment. The mean time to appearance of the cutaneous adverse effect for all TNFalpha inhibitors was 9.5 months. Cessation of the responsible TNFalpha inhibitor was carried out in 47 patients either alone or in association with adjuvant anti-psoriatic therapy (mostly topical). This resulted in complete remission in 21 patients, partial remission in 20 patients, and stable disease in another three patients; in the other three patients, the outcome was not reported. TNFalpha inhibition was continued in 47 patients but anti-psoriatic adjuvant therapy was introduced. The outcome in this group was complete remission in 22 patients, partial remission in 25 patients, and stable disease in 2 patients. The response rate (complete remission plus partial remission) was 93.2% and 95.9%, respectively, in each group. In six patients, switching from one TNFalpha inhibitor to another one immediately after cutaneous adverse effects occurred resulted in an improvement in five patients. In nine patients, a second TNFalpha inhibitor was initiated after a break in TNFalpha inhibition. The response to a second or third drug in these patients was mixed. The underlying pathomechanisms of induction of psoriasis or psoriasiform exanthemata by TNFalpha inhibitors remain elusive but there is reason to assume that induction of such adverse events has more than one pathophysiology.

Ilium Osteitis as the Main Manifestation of the SAPHO Syndrome: Response to Infliximab Therapy and Review of the Literature

To analyze the clinical efficacy of anti-tumor necrosis factor (TNF)-alpha therapy in the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. We describe 2 new cases with ilium osteitis as the main SAPHO syndrome feature and review reported cases treated with anti-TNF-alpha. A literature search of SAPHO syndrome cases treated with TNF-alpha blocking therapy with special emphasis on osteoarticular and skin responses was performed. Eighteen cases were identified: 17 SAPHO syndrome and 1 chronic recurrent multifocal osteomyelitis, a juvenile variant of SAPHO syndrome. Sixteen were reported cases and 2 were nonreported cases seen in our arthritis unit. Sixteen patients received infliximab and 2 received etanercept, with an early, sustained clinical improvement in most cases. Anti-TNF-alpha therapies are effective treatment for patients with refractory SAPHO syndrome, not only for cutaneous lesions but also for persistent bone lesions such as osteitis.

Poster 2, Acne fulminans: part of the spectrum of SAPHO

A 13‐year‐old boy was admitted to hospital with severe back pain and systemic upset. He had commenced isotretinoin 25 mg (0·5 mg kg–1) daily 17 days previously for severe acne unresponsive to oral erythromycin. Isotretinoin was stopped after 4 days due to severe lower back pain. On admission, he was unable to mobilize and the pain was uncontrolled with oral morphine sulphate. Investigations showed leucocytosis and neutrophilia. Magnetic resonance imaging of the vertebrae showed multiple areas of high signal consistent with an inflammatory process such as osteomyelitis. Oral prednisolone 40 mg daily and ibuprofen controlled the pain within 2 days. Sulfasalazine (1 g twice daily) was commenced 10 days later. The re‐introduction of isotretinoin 5 mg daily 12 days after admission precipitated severe back pain, necessitating 3 days of intravenous methylprednisolone. The oral prednisolone dose has been reduced over 6 weeks and stopped. The acne is currently controlled with clindamycin, although there is marked scarring. Acne fulminans is a rare condition characterized by sudden onset of severe acne and systemic features such as fever, leucocytosis and arthralgia.1 Osteomyelitic lesions are a recognized feature. In 1987, the term SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome was proposed to describe a clinical entity with skin, joint and bone manifestations. Associated skin conditions include severe acne, psoriasis and palmoplantar pustulosis. Reported sites of osteoarticular involvement include the anterior chest wall, vertebrae, pelvis and mandible.2 Our patient clearly has acne fulminans and fulfils the criteria for SAPHO syndrome. We believe this condition will be increasingly recognized by dermatologists. 1 Karvonen S. Acne fulminans: report of clinical findings and treatment of twenty‐four patients. J Am Acad Dermatol 1993; 28:572–9. 2 Hayem G, Bouchaud‐Chabot A, Benali K et al. SAPHO syndrome: a long‐term follow‐up study of 120 cases. Semin Arthritis Rheum 1999; 29:159–71.

Isolierte, bilaterale sternokostoklavikuläre Hyperostose (SCCH) mit pathologischer Klavikulafraktur - Ein Verlauf über 10 Jahre

The presented case demonstrates the clinical and radiological course of a woman suffering from bilateral sternocostoclavicular hyperostosis (SCCH) from early changes to a spontaneous clavicula fracture after 10 years. SCCH is characterised by a chronic recurrent painful swelling of the sternoclavicular region due to an aseptic inflammation and hyperostosis of the clavicula, sternum, upper ribs and the adjacent soft tissues. Frequently the picture is accompanied by cutaneous and other skeletal symptoms. The disease can be part of the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. The chronic process is represented by the typical "bull horn sign" in a bone scan. NSAIDS should represent the first line treatment.

Acne fulminans with synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome treated with infliximab

Acne fulminans is a syndrome of sudden onset hemorrhagic and ulcerative acne involving the back, chest, and face combined with systemic symptoms. It can be the dermatologic manifestation of the synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome. Current therapy for acne fulminans consists of wound care, topical and systemic corticosteroids, isotretinoin, and nonsteroidal anti-inflammatory drugs (NSAIDs). Infliximab, a recently developed monoclonal antibody against tumor necrosis factor-alfa, has shown efficacy in the treatment of psoriatic arthritis and ankylosing spondylitis both of which share clinical similarities to the SAPHO syndrome. We report the case of a patient with the SAPHO syndrome and acne fulminans who was treated with infliximab. Ten months after initiating therapy with infliximab, the area of the patient's ulcerative lesions was reduced by 70%. Infliximab might be considered as a treatment option for patients with acne fulminans unresponsive to conventional therapies.

Is primary chronic osteomyelitis a uniform disease? Proposal of a classification based on a retrospective analysis of patients treated in the past 30 years

Introduction: Primary chronic osteomyelitis of the jaw is a rare, non-suppurative, chronic inflammatory disease of unknown aetiology. To date, classification is confusing due to a non-uniform terminology. The aim of this study was to establish a simple (clinical) classification based on patient data from our clinic. Methods: Retrospective analysis revealed 30 cases of which clinical course, radiology, pathology, therapy and outcome were analysed. Results: Both sexes were equally represented. The mean age at onset of disease was 35 years (range 5–76 years). Onset of disease revealed two peaks of incidence, one in adolescence and one after age 50 years. While clinical symptoms were similar in all cases, an increased intensity of these symptoms was noted in younger individuals as well as in the early stages of the disease. Five adults and one adolescent presented with additional non facial bone, joint and skin manifestations consistent with the diagnosis of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome, or chronic recurrent multifocal osteomyelitis. Radiology demonstrated sclerosis, osteolysis and periosteal reaction in variable stages in all cases. However, findings were more extensive in younger patients. Histology revealed different stages of chronic inflammation in all cases. Microabscess formation was noted in 11 cases, six of which were children/adolescents. Therapy consisted mainly of surgery, antibiotics and hyperbaric oxygen therapy. At the end of the follow up period, 11 patients demonstrated complete remission, while in 14 cases amelioration and in 5 no significant improvement was noted. Conclusion: Based on differences in age at presentation, clinical appearance and course, radiology and histology, a subclassification into early and adult onset primary chronic osteomyelitis has been established. Cases with purely mandibular involvement should further be distinguished from cases associated with other syndromes.

Seronegative spondyloarthropathies

Seronegative spondyloarthropathies are characterized by bilateral or unilateral sacroiliitis, spondylitis, peripheral arthritides or extraarticular manifestations. The spondyloarthropathies are associated with a positive HLA-B27 status. Genetic factors, repetitive biomechanical stress and the immune response to bacterial products lead to the inflammatory changes at typical anatomical sites. Diseases classified as spondyloarthropathies include ankylosing spondylitis, reactive arthritis, psoriatic arthritis, juvenile spondyloarthropathy and inflammatory bowel disease associated with spondylitis. The SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome is also considered a member of the spondyloarthropathy family. An otherwise unclassified HLA-B27-linked peripheral arthritis is often considered an undifferentiated spondyloarthropathy. Treatment consists of physical therapy including patient education, non-steroidal anti-inflammatory drugs and disease modifying anti-rheumatic drugs.

Radiological features of long bones in synovitis, acne, pustulosis, hyperostosis, osteitis syndrome and their correlation with pathological findings

The purpose of this study was to demonstrate the radiological features of long bones in synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome and to correlate these with the clinical findings. Eleven long bone lesions in seven cases of SAPHO syndrome were examined. The patients ranged in age from 6 to 63 years, with a mean of 47 years. In all seven cases, radiography, 99mtechnetium bone scintigraphy, CT scan, and magnetic resonance imaging (MRI) were performed. In six of the cases, bone biopsy and bone culture were carried out for 7 long bones. Seven of the involved lesions were from the shaft of the femur, one each was from the neck and the shaft of the humerus, and one was from the proximal tibia. These lesions showed radiologically hyperostosis, osteolysis, and bone infarction-like lesion. Osteolysis was occasionally accompanied by sclerotic change. Hyperostosis usually showed diaphyseal involvement, presenting low signal intensity on T1- and T2-weighted MR images. Histologically, these findings corresponded to massive bone necrosis, new bone formation, fibrosis, or a mixture of these associated with mild inflammatory cell infiltration. Osteolysis involved dyaphysis, metaphysis, or epiphysis associated with arthritis, and presented low signal intensity on T1-weighted images, nonhomogeneous signal intensity lower than fat on T2-weighted images, and high signal intensity on fat suppression images. These findings corresponded to fibrosis, granulation, and inflammatory cell infiltration with lymphocyte aggregation. Bone infarction-like lesion was observed in the shaft or neck of the femur and the humerus and accompanied by calcification and cystic change. Bone cultures were negative in all cases in which bone biopsy was performed. Although hyperostosis is thought to be a characteristic bone lesion in SAPHO syndrome, the long bone lesion can occasionally show not only hyperostosis but also osteolytsis and bone infarction-like lesions.