ABSTRACT
OBJETIVO: Descrever os achados clínicos, radiológicos e patológicos da síndrome SAPHO e sugerir que, apesar de ser considerada rara, esta síndrome deve estar sendo subdiagnosticada por clínicos e radiologistas, provavelmente em função do desconhecimento das suas características. MATERIAIS E MÉTODOS: Foi realizado estudo retrospectivo de seis casos confirmados desta síndrome, dando-se ênfase aos achados clínicos (idade, sexo e sintomas) e de imagem (cintilografia óssea, radiografia convencional, tomografia computadorizada e ressonância magnética). RESULTADOS: A manifestação clínica inicial de todos os pacientes foi dor na parede torácica ântero-superior há pelo menos quatro meses. Todos apresentavam achados de imagem de processo inflamatório e/ou osteíte e hiperostose nas articulações da parede torácica ântero-superior. As alterações cutâneas da síndrome, tipo pustulose palmoplantar, estiveram presentes em cinco dos seis pacientes. Em nenhum dos seis casos o diagnóstico foi sugerido na consulta clínica inicial ou na primeira interpretação das imagens feita por radiologistas não especialistas em sistema músculo-esquelético. CONCLUSÃO: Os nossos achados estão de acordo com os descritos na literatura, devendo ser considerado este diagnóstico em todo paciente que apresente quadro doloroso de parede torácica acompanhado de manifestações dermatológicas e/ou osteíte.
Subject(s)
Humans , Female , Adult , Middle Aged , Acquired Hyperostosis Syndrome , Hyperostosis, Sternocostoclavicular , Hyperostosis, Sternocostoclavicular/diagnosis , Osteitis , Psoriasis , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/pathology , Diagnosis, Differential , Retrospective Studies , Acquired Hyperostosis Syndrome/etiologyABSTRACT
Se presentan los estudios clínicos e imagenológicos realizados a un paciente con diagnóstico de hiperostosis esternocostoclavicular
Subject(s)
Humans , Male , Middle Aged , Hyperostosis, Sternocostoclavicular , Radiography , Rheumatic Diseases , TomographyABSTRACT
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.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Antirheumatic Agents , Diagnosis , Diagnosis, Differential , Hyperostosis , Hyperostosis, Sternocostoclavicular , Inflammation , Osteomyelitis , Psoriasis , Skin Manifestations , Spine , Spondylarthropathies , Thoracic Wall , InfliximabABSTRACT
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.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Antirheumatic Agents , Diagnosis , Diagnosis, Differential , Hyperostosis , Hyperostosis, Sternocostoclavicular , Inflammation , Osteomyelitis , Psoriasis , Skin Manifestations , Spine , Spondylarthropathies , Thoracic Wall , InfliximabABSTRACT
SAPHO syndrome is an acronym for an increasingly recognized syndrome of synovitis, acne, pustulosis, hyperostosis, and osteitis. Most of the previously reported case are from Japan and Europe. The authors report a case of SAPHO syndrome in Korean female who presented with extrasternal neurologic symptoms. A 60-year-old female with thoracic and right chest wall pain presented with the sternocostoclavicular hyperostosis, and recurrent aseptic osteitis. Previously, she had hystrectomy and thyroidectomy due to uterine malignancy 25 years ago. Also, she started to take medications for palm and sole pustulosis 7 days prior to admission. Through evaluation of clinical, radiological, serological studies, studies was done along with bone needle biopsy for the biological reassessment. The hyperostosis was found in the pedicles of thoracic 8, 9, 10 vertebrae and sternoclavicular joint. Radio-isotope bone scan showed an accumulation of tracer in thoracic vertebra. The bone biopsy from these site showed increased osseous turnover, thickening of trabeculae accompanied by mild acculuation of granulation tissue and round cell infiltration, compatible with mild chronic inflammation with marrow fibrosis. The patient showed good response to conservative management. The authors report a case of SAPHO syndrome with thoracic radiculopathy. It is considered that SAPHO syndrome is related to spondyloarthropathy, and appears to have benign disease process with good prognosis.
Subject(s)
Female , Humans , Middle Aged , Acne Vulgaris , Acquired Hyperostosis Syndrome , Biopsy , Biopsy, Needle , Bone Marrow , Europe , Fibrosis , Granulation Tissue , Hyperostosis , Hyperostosis, Sternocostoclavicular , Inflammation , Japan , Neurologic Manifestations , Osteitis , Prognosis , Radiculopathy , Spine , Spondylarthropathies , Sternoclavicular Joint , Synovitis , Thoracic Wall , ThyroidectomyABSTRACT
Sternocostoclavicular hyperostosis is an uncommon disease, characterized by an inflammatory arthrosteitis of the sternocostoclavicular region. Clinically, it manifests as a painful swelling of the upper anterior chest wall, which is associated with occasional pustulosis palmaris and plantaris. A 48-year-old man had suffered from pain in both shoulders and the upper anterior part of the chest for 6 months. On examination, a venous engorgement in the neck with dilated collateral veins in the upper chest and shoulders was observed. Swelling was noticed in his face, neck and both arms. Radiologically, the clavicles, the sternum and the first ribs were enlarged with complete fusion between them. 99Tc scintigraphy showed increased uptake in the clavicles and the sternum. Selective venography resulted in a bilateral subclavian and brachiocephalic vein occulation, which resulted from a subclavian vein thrombosis. All the above suggested a sternocostoclavicular hyperostosis. He underwent a vascular graft interposition between the right jugular vein and the left innomianate vein (using 8mm ringed Gore-Tex graft) and a resection of the bilateral medial half of clavicle and 1st rib. Here, we present a case on sternoclavicular hyperostosis with subclavian and brachiocephalic vein thrombosis, and report this case study with a review of the appropriate literature.
Subject(s)
Humans , Middle Aged , Arm , Brachiocephalic Veins , Clavicle , Hyperemia , Hyperostosis , Hyperostosis, Sternocostoclavicular , Jugular Veins , Neck , Phlebography , Polytetrafluoroethylene , Radionuclide Imaging , Ribs , Shoulder , Sternum , Subclavian Vein , Thoracic Wall , Thorax , Thrombosis , Transplants , Veins , Venous ThrombosisSubject(s)
Acne Vulgaris , Adult , Female , Humans , Hyperostosis, Sternocostoclavicular/diagnosis , Osteitis , Psoriasis/diagnosis , Syndrome , Tomography, X-Ray ComputedABSTRACT
Sternocostoclavicular hyperostosis is a chronic arthro-osteitis affecting mostly jux-tasternal structures. It is characterized clinically by painfull swelling of the upper anterior chest wall and occasionally is associated with pustulosis palmaris and plantaris. A 34-year-old female presented with upper anterior chest pain and low back pain. She was well until three months ago, then left shoulder pain and multiple pustule on both palms developed. She felt gradually exhausted in spite of symptomatic medication at drug store. Eight months before this admission she underwent an operation on the right sided breast due to a benign mass. On examination there was no evidence of conjunctivitis or aphthous oral ulcer, tenderness on left sternoclavicular joint was noted and flextion of the left hip joint was limited because of pain in the joint area. Multiple pustules were noted in the palms of both hands, but it wasn't associated with nail change. The mammographs was normal. Plain radiographs of the spine showed disc space narrowing of seventh thoracic spine and spondylolysis on fifth' lumbar vertebra. Radionuclide imaging with 99mTc-methylene diphosphonate of the bone showed multiple increased uptake on both sacroiliac joints, both first costochondra] joints, left sternoclavicular joint, and eighth thoracic vertebra. Magnetic Resonance Imaging reavealed low signal intensity in medial end of the left clavicle with joint effusion and Schmorl's node in seventh thoracic disc space. Laboratory tests were normal, except ESR 23mm/h. In this case we think that careful clinical differential diagnosis of sternocostoclavicular hyperostosis should include sclerotic metastasis.
Subject(s)
Adult , Female , Humans , Breast , Chest Pain , Clavicle , Conjunctivitis , Diagnosis, Differential , Hand , Hip Joint , Hyperostosis, Sternocostoclavicular , Joints , Low Back Pain , Magnetic Resonance Imaging , Neoplasm Metastasis , Oral Ulcer , Radionuclide Imaging , Sacroiliac Joint , Shoulder Pain , Spine , Spondylolysis , Sternoclavicular Joint , Technetium Tc 99m Medronate , Thoracic WallABSTRACT
Pustulotic arthro-osteitis is a rheumatic syndrome of unknovn cause, characterised by an inflammatory osteitis of the sternocostoclavicular region and pustuosis palmaris et plantaris. Although many ases of the disease have been reported in Japan, it, has not been reported in Korea so far. Three cases of pustulosis palmaris et plantaris associated i rith sternocostoclavicular hyperostosis or pustulotic arthro-osteitis are presented.
Subject(s)
Hyperostosis, Sternocostoclavicular , Japan , Korea , Osteitis , PsoriasisABSTRACT
Sternocostoclavicular hyperostosis is a benign ossifying diathetic disorder characterized by hyperostosis and soft tissue ossification of the clavicles, anterior portion of the first ribs, and manubrium, with variable hyperostosis or ankylosis in the spine and sacroiliac joints. A review of the literature and our own case describes the clinical findings and its characteristic features in RI, CT, and plain film.
Subject(s)
Ankylosis , Clavicle , Hyperostosis , Hyperostosis, Sternocostoclavicular , Manubrium , Ribs , Sacroiliac Joint , SpineABSTRACT
Sternocostoclavicular hyperostosis is a rare syndrome characterized by hyperostosis and soft tissue ossification between the clavicle and the anterior part of the upper ribs. Since first reported in 1974, the syndrome has been noted predominantly in Japan(1). Our case report describes the disorder and its radiologic manifestations.
Subject(s)
Clavicle , Hyperostosis , Hyperostosis, Sternocostoclavicular , RibsABSTRACT
Stemocostoclavicular hyperostosis (SCCH) is a very uncommon disease characterized by hyperostosis of the clavicle, upper ribs, sternum and soft tissue ossification, particularly of the costoclaviculr ligament. Although the cause of this disorder is unknown, clinical, radiological and histological examinations may allow the diagnosis of SCCH. This paper presents a case of SCCH and a brief review of the literature.