Case report of SAPHO syndrome in children / 中华实用儿科临床杂志

Retrospective analysis.To report a child with synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome admitted to the Department of Pediatric Nephrology and Rheumatology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University in March 2020.A 14-year-old boy presented to hospital due to acne for more than 2 months, fever for more than 50 days, and hip pain for 2 weeks.The patient was diagnosed and treated as acne combined with infection and arthritis in other hospitals for more than 2 months without remission.He was initially diagnosed as juvenile idiopathic arthritis, acne combined with infection on admission.The disease was aggravated even after medications of anti-infection and anti-inflammatory drugs.After multidisciplinary consultation, the patient was diagnosed as SAPHO syndrome.The patient recovered from medications of Celecoxib, Sulfasalazine, recombinant human tumor necrosis factor receptorⅡ: IgG Fc fusion protein and other drugs.SAPHO syndrome is rare, especially in children, which is easily misdiagnosed.This case report suggested that SAPHO should be considered in children with acne, especially those combined with arthritis.

Síndrome Sapho: reporte de caso / SAPHO syndrome: report of a case

El síndrome SAPHO es una enfermedad inflamatoria de origen multifactorial que incluye una asociación entre manifestaciones dermatológicas y osteoarticulares. La presencia de osteítis e hiperostosis definen la enfermedad. No existe un tratamiento estándar, pero se usan AINES como primera línea. Se presenta el caso de un varón de 18 años con acné severo en dorso, pecho, barbilla y zona retroauricular, asociado a fiebre alta y placas eritematosas en ambas piernas. La gammagrafía presenta hipercaptación en tibia derecha, con relación a osteítis no infecciosa. Tuvo evolución favorable con antibióticos y corticoide oral.

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a multifactorial inflammatory condition that includes an association between dermatological and bone and joint manifestations. The presence of osteitis and hyperostosis define this disease. There is no standard therapy for SAPHO syndrome, but NSAIDs are used as first line. We present the case of an 18-year-old male subject with severe acne on his back, chest, chin, and the retroauricular area, being this associated to high fever and erythematous plaques in both legs. The bone scan showed right leg hyper-uptake, related to non-infectious osteitis. The patient did well with antibiotics and oral steroids.

Analysis of imaging and skin characteristics of SAPHO syndrome / 实用放射学杂志

Objective To explore the clinical and imaging characteristics of synovitis,acne,pustulosis,hyperostosis,osteitis(SAPHO syndrome),to improve the recognition of this disease.Methods Clinical and imaging data were respectively analyzed in 12 SAPHO patients with the review of literature.Results In the 1 2 SAPHO cases,9 were females and 3 were males,with an average age of (45 ±6)years.All the 1 2 cases had bone changes on the anterior-superior chest wall,which manifested as osteosclerosis,hyperplasia and joint destruction.There were 5 cases manifested as symmetrical lesion which were the typical sea gull sign or cow head sign with the proportion of 41.7%.There were 4 cases with the lesion involving the spine including 3 cases in the thoracic vertebra and 1 case in the lumbar vertebra,which showed the erosion and sclerosis in the endplates and destruction of the anterior and posterior vertebral angles.There were 2 cases with the lesion involving the sacroiliac joints,including 1 unilateral and 1 bilateral case,which showed as joint destruction.In the 12 cases,there were 11 cases with obvious cutaneous pathological changes,including palmoplantar pustulosis in 8 cases and acne in 3 cases.Conclusion SAPHO syndrome can be diagnosed by combining skin lesions and bone changes on the patient’s anterior-superior chest wall.

A Case of SAPHO Syndrome That Was Introduced to a Palliative Care Unit as Multiple Bone Metastases of Unknown Primary Carcinoma

Introduction: This study reports on a case of SAPHO syndrome in a patient in the palliative care unit who had multiple bone metastases of an unknown primary carcinoma. Case: A 74 year old male presented with broken eleventh thoracic and third lumbar vertebrae. Magnetic resonance imaging (MRI) revealed multiple lesions, which were thought to be bone metastases of a carcinoma, in the thoracic and lumbar spine. Attempts to identify the location of the primary lesion were unsuccessful. Therefore, the differential diagnosis was multiple bone metastases of an unknown primary carcinoma. The patient was transferred to the palliative care unit. The patient’s condition remained largely unchanged for several years; therefore, a second set of clinical examinations was conducted. Computed tomography revealed sternocostoclavicular hyperostosis, and there was symmetric uptake on bone scintigraphy. Further MRI studies revealed that the spinal lesions were comparatively reduced, leading to a diagnosis of SAPHO syndrome. Discussion: SAPHO syndrome is a rare, benign disease that presents as a variety of osteoarticular disorders with specific dermatoses. SAPHO syndrome is difficult to diagnose; therefore, it is important to consider it as a differential diagnosis in patients with bone lesions.

A Case of SAPHO Syndrome Associated with Lytic Bone Lesions Resembling Metastases / 대한피부과학회지

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a variety of inflammatory bone disorders associated with dermatologic pathology. A 57-year-old female presented with pustulosis on both hands that had persisted for several months. She also had lower back pain without trauma history. On physical examination, tenderness on her lower back and left anterior chest wall pain were found, and claudication was observed. Radiological studies including computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)-CT showed endplate lytic changes in her spine, a focal hypermetabolic lesion in a left rib, and costochondral junction. These findings raised doubt on the presence of metastatic bone lesions, and there was no indication for primary cancer after a complete medical checkup. Palmoplantar pustulosis was well controlled by treatment with acitretin. The osteitis associated with SAPHO syndrome usually presents as osteosclerosis, while reports on osteolytic lesions are rare. We report herein a rare case of SAPHO syndrome associated with bone lesions resembling bone metastasis.

A Case of Palmoplantar Pustulosis Present in the Daughter of a SAPHO Syndrome Patient

SAPHO syndrome is a rare inflammatory, pseudoinfectious disease. Initially it was an acronym for Syndrome Acne Pustulosis Hyperostosis Osteitis, and the meaning of S was later changed to synovitis. It occurs predominantly in children and adults and is not common over 60 years. The most common clinical presentation is osteoarticular involvement at the anterior chest wall and skin manifestations may be evident, but it could occur years earlier or develop later. We report on two cases of mother and daughter. A 51-year-old female was diagnosed with SAPHO syndrome with costochondritis and palmoplantar pustulosis. Five years later, her 31-year-old daughter presented with similar skin manifestations of the hand and foot.

SAPHO Syndrome in a Patient with Breast Cancer Mimicking Bone Metastasis: A Case Report

A 66-year-old woman was transferred to our hospital due to her right breast cancer. Preoperative breast MRI shows 1.9 cm malignancy on her right breast (cT1N0M0) and incidentally found osteosclerotic change of left coststernoclavicular region. Bone scintigraphy showed hot uptake and the possibility of bone metastasis was not excluded. However, because the bone metastasis is not common in early stage cancer and the costosternoclavicular region is not common site, other possibility should be considered. SAPHO syndrome can be diagnosed even in the absence of dermatosis when there is an axial or appendicular osteitis and hyperostosis, especially in costosternoclavicular region. Though breast imaging specialists are not accustomed to this disease entity, awareness and diagnosis of the SAPHO syndrome can help differentiate bone metastasis.

A Case of Successful Treatment of Refractory Synovitis Acne Pustulosis Hyperostosis Osteitis (SAPHO) Syndrome with Adalimumab / 영남의대학술지

Synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome is a rare disease that involves the skin, bones and joints. It is thought to be caused by infection with low-toxicity bacteria and to be the result of reactive infectious osteitis. However, this hypothesis has not yet been clearly established. New SAPHO syndrome treatment methods are needed because the disease does not respond to treatment in many cases. In this paper, a case is reported of SAPHO syndrome with pain in the acromioclavicular joint and with squamous and pustular macules on the palms and soles. First, the patient was treated with aceclofenac, prednisolon and sulfasalazine for two weeks. However, the symptoms were not relieved, so methotrexate and pamidronate were added to the treatment. Since no improvement was seen after four weeks of treatment, adalimumab was prescribed. The skin lesions were relieved two weeks later, and the bone pain and arthralgia, four weeks later. No recurrence or adverse effects were observed at the 22-week follow-up.

A Case of Refractory SAPHO Syndrome Treated with Etanercept

SAPHO syndrome, which has different skin changes and osteoarticular inflammation, is an acronym that stands for synovitis, acne, pustulosis, hyperostosis, and osteitis. Treatment of SAPHO syndrome includes non-steroidal anti-inflammatory drugs (NSAIDs), anti-rheumatic drugs, such as colchicines, corticosteroids and bisphosphonates, and disease-modifying agents. However, the treatment of SAPHO syndrome is controversial because it is a new clinical entity with unclear etiopathogenesis and inadequate clinical studies. We report a case with refractory SAPHO syndrome, which was successfully treated with a tumor necrosis factor (TNF)-alpha blocker.

A Case of SAPHO Syndrome / 대한피부과학회지

The term, SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is an acronym to designate a clinico-radiologic entity with combination of skin, bone, and joint manifestations. We, herein, present a case of SAPHO syndrome in a 27-year-old man with pruritic, localized, crops of deep-seated pustules on both palms and soles for 2 years, and multiple joints pain including anterior upper chest pain for 3 years. Histopathologically, the palm showed a large intraepidermal cavity filled with many neutrophils, and many typical spongiform pustules at the wall of it. Radiographically, the left sternoclavicular joint showed synovitis, osteitis and hyperostosis.

Musculoskeletal Causes of Anterior Chest Wall Pain

OBJECTIVE: To investigate the musculoskeletal causes of anterior chest pain and know the prevalencies of the diseases. METHOD: During 4-year periods (1997-2001), 37 patients with anterior chest wall pain was analyzed with regard to the causes of pain and the frequencies of the diseases. RESULTS: 17 patients (45.9%) had systemic diseases and 20 patients (54.1%) had focal joint problems. Systemic disease included the undifferentiated spondyloarthropathy (18.9%), ankylosing spondylitis (13.5%), psoriatic arthritis (2.7%), SAPHO (Synovitis, Acne, Psoriasis, Hyperostosis, Osteitis) syndrome (8.1%), and rheumatoid arthritis (2.7%). Focal joint diseases included costochondritis (10.8%), sterno clavicular inflammatory arthropahty (5.4%), sternoclavicular hyperostosis (2.7%) and infective arthritis (2.7%). Other focal joint problems were pain in sternoclavicular joint with the tenderness and swelling (2.7%), pain in costochondral joint (13.6%), sternoclavicular joint (5.4%), xyphoid process (2.7%) with only focal tenderness. 3 (8.1%) patients had pain in chest wall which had no focal tenderness and swellings on the joint. CONCLUSION: Diverse systemic diseases were identified as causes of the anterior chest wall pain. So physiatrist keep in mind this result and make use of them in diagnostic approaching of the anterior chest pain due to chest wall skeletal involvemen.

Two cases of SAPHO syndrome / 대한내과학회지

We describe two cases of SAPHO syndrome with history of palmoplantar pustulosis and pain on the anterior chest wall and lower back area. The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome includes a group of disorders characterized by bony lesions commonly involving the anterior chest wall and associated with skin manifestations. The skeletal manifestation is characterized by the association of inflammation and hyperostotic change, in the form of sternocostoclavicular hyperostosis, spondyloarthropathy and chronic recurrent multifocal osteomyelitis. Common cutaneous lesions include palmoplantar pustulosis, pustulotic psoriasis, and severe forms of acne. The pathogenesis remains elusive, but a link with seronegative spondyloarthropathy is probable. To date, the treatment is empirical. Nonsteroidal anti-inflammatory drugs are the first choice, and other drugs including corticosteroid, disease modifying antirheumatic drugs, pamidronate, and infliximab have been tried with some therapeutic benefit. SAPHO syndrome is a condition in the differential diagnosis of infectious or tumorous conditions of the bone. Early and proper diagnosis is important to avoid unnecessary investigations or treatments.

Two cases of SAPHO syndrome / 대한내과학회지

We describe two cases of SAPHO syndrome with history of palmoplantar pustulosis and pain on the anterior chest wall and lower back area. The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome includes a group of disorders characterized by bony lesions commonly involving the anterior chest wall and associated with skin manifestations. The skeletal manifestation is characterized by the association of inflammation and hyperostotic change, in the form of sternocostoclavicular hyperostosis, spondyloarthropathy and chronic recurrent multifocal osteomyelitis. Common cutaneous lesions include palmoplantar pustulosis, pustulotic psoriasis, and severe forms of acne. The pathogenesis remains elusive, but a link with seronegative spondyloarthropathy is probable. To date, the treatment is empirical. Nonsteroidal anti-inflammatory drugs are the first choice, and other drugs including corticosteroid, disease modifying antirheumatic drugs, pamidronate, and infliximab have been tried with some therapeutic benefit. SAPHO syndrome is a condition in the differential diagnosis of infectious or tumorous conditions of the bone. Early and proper diagnosis is important to avoid unnecessary investigations or treatments.

SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) Syndrome occurred on the Clavicle: A Case Report / 대한정형외과학회잡지

Thirty years ago, osteoarticular disorders which are related to various skin lesions such as pustulosis, psoriasis and acne have been reported in Japan and northwestern Europe. This disease entity is composed of three major disorders, which include the hyperostosis of the anterior chest wall, chronic multifocal recurrent osteomyelitis involving the metaphysis of long bone and pustulotic arthro-osteitis. In 1987, Kahn and Chamot first coined the term SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) . Early lesions of the SAPHO syndrome contain acute inflammation, edema, and prominent periosteal bone formation, and which is indistinguishable from ordinary bacterial osteomyelitis. In addition, late lesions of that demonstrate bony sclerosis. Therefore, it is necessary to avoid misdiagnosis as Paget's disease and Ewing sarcoma. We experienced a case of SAPHO syndrome and report the clinical, radiological and pathological findings in detail.