The bullhead sign: scintigraphic pattern of sternocostoclavicular hyperostosis and pustulotic arthroosteitis.

OBJECTIVE: The purpose of this retrospective study was to examine the value of whole-body nuclear medicine imaging and to evaluate the typical scintigraphic pattern of sternocostoclavicular hyperostosis (SCCH) and/or pustulotic arthroosteitis (PAO). In this entity the correct diagnosis is frequently missed because of uncharacteristic changes in other imaging modalities. MATERIALS AND METHODS: Forty-nine patients (age range 15-65 years old, mean age 36 years) with sternocostoclavicular hyperostosis (SCCH) and/or pustulotic arthroosteitis (PAO) were examined with whole-body scintigraphy and conventional radiography. RESULTS: Forty-three of 49 patients with SCCH/PAO showed a characteristic "bullhead"-like high tracer uptake of the sternocostoclavicular region with the manubrium sterni representing the upper skull and the inflamed sternoclavicular joints corresponding to the horns (= bullhead sign). Scintigraphy revealed additional skeletal manifestations (spondylitis, sacroiliitis, osteitis) in 33 of 49 patients with SCCH and/or PAO. CONCLUSIONS: Bone scintigraphy is the imaging modality of choice for the diagnosis of skeletal involvement in PAO. Nuclear medicine reveals unexpected locations and shows the typical pattern of focal hot spots of the spine, sacroiliac joints and/or appendicular skeleton in the large majority of cases in combination with a bullhead-like tracer uptake of the sternocostoclavicular region. The bullhead sign is the typical and highly specific scintigraphic manifestation of SCCH and PAO in radionuclide bone scans and helps to avoid unnecessary biopsies.

[SAPHO syndrome. Case description of 3 patients with acne conglobata and osteoarticular symptoms]. / Das SAPHO-Syndrom. Fallbeschreibung von drei Patienten mit Akne conglobata und osteoartikulären Symptomen.

We report three new cases of the SAPHO syndrome. This acronyme consists of synovitis, acne, pustulosis, hyperostosis and osteitis. Symptoms of this syndrome, which may not all be present, are pustulotic skin diseases (pustulosis palmoplantaris or severe acne) associated with osteoarticular lesions (mainly sternoclavicular hyperostosis, spondylarthropathies or chronic recurrent multifocal osteomyelitis). The dermatological aspects of this syndrome are discussed in detail.

Osseous manifestations of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome.

The SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a complex group of disorders characterized by peculiar bone lesions, most commonly involving the anterior chest wall, and sometimes accompanied by dermatologic manifestations. The clinical and radiographic features of this syndrome are well described, but few studies have examined the histologic features of the bone lesions. We describe the clinical, radiographic, and histologic features of the osseous lesions encountered in eight patients with a clinical diagnosis of SAPHO syndrome. The patients included five female and three male patients ranging in age from 5 to 63 years (mean, 35.3 years). The most common clinical presentation was pain related to the sites of osseous involvement. Two patients also had some form of pustular dermatosis. The radiographic features of the osseous lesions varied but often suggested the possibility of a neoplasm. Nine pathologic specimens were available for review, five from the clavicle, two from the first rib, one from the calcaneus/cuboid, and one from the tibia. The histologic features varied but seemed related to the duration of the patients' musculoskeletal symptoms. Early lesions contained acute inflammation, edema, and prominent periosteal bone formation, histologically indistinguishable from ordinary bacterial osteomyelitis, whereas late lesions demonstrated markedly sclerotic bone trabeculae with prominent marrow fibrosis and only mild chronic inflammation; one of these biopsies appeared virtually identical to Paget's disease. One biopsy was performed after an intermediate duration of symptoms and contained prominent chronic inflammation only. The histologic findings in SAPHO are variable and nonspecific and may depend on the duration of disease, but it is important to recognize the spectrum of histologic changes possible in the syndrome and to realize that clinicopathologic correlation is necessary to avoid misdiagnosis and unnecessary long-term antibiotic therapy.

Arthro-osteitis--a clinical spectrum.

Arthro-osteitis is an uncommon condition which can be associated with palmoplantar pustulosis. It forms part of a group of conditions which include the synovitis, acne, pustulosis, hyperostosis, osteitis syndrome (SAPHO) and sternocostoclavicular hyperostosis. We report four cases illustrating the clinical spectrum of this condition which occurred in the absence of concomitant skin lesions. One patient had extensive aortic calcification a feature not previously reported in this condition, which may represent a low grade inflammatory aortitis.

Sternocostoclavicular hyperostosis.

Sternocostoclavicular hyperostosis is a chronic arthro-osteitis affecting mostly juxtasternal structures. It is characterized clinically by painful swelling of the upper anterior chest wall and occasionally is associated with pustulosis palmaris and plantaris. Pathologically there is osteitis, periostitis, and new bone formation of periarticular structures. Two patients are described, and the clinical, laboratory, and radiological findings of 251 reported cases in the English literature are reviewed.

SAPHO syndrome.

Four women with pustulosis palmoplantaris (PPP) and associated sternocostoclavicular hyperostosis are described. The connection between the two diseases is not coincidental: it is encountered in 9.4% of PPP patients, and was reported in the French literature as part of the SAPHO (synovitis acne pustulosis hyperostosis osteomyelitis) syndrome. This syndrome is linked to the spondyloarthropathies, having an increased prevalence of HLA B27 and occurrence of sacroiliitis. None of our patients had HLA B27, but HLA A26 was found in the 3 patients examined. No conclusion can be drawn from this finding, however, since this antigen is found in 21.7% of Ashkenazi Jews. Recognition of this syndrome by dermatologists will improve diagnosis and treatment.

Pustular vasculitis with clinical feature of pustular psoriasis and sternoclavicular hyperostosis.

We report the case of a 51-year-old Japanese man with a unique pustulosis. He had multiple erythematous plaques and numerous pinpoint pustules on the trunk and extremities resembling pustular psoriasis. Histologic features revealed a fully developed intraepidermal abscess filled with neutrophils and disrupted epidermal keratinocytes. Mild leukocytosclastic vasculitis was seen in the underlying dermis. A direct immunofluorescence study revealed IgM, Clq, C3 and fibrinogen deposits in the dermal vessels. The patient had also sternoclavicular hyperostosis. We think that this represents a unique type of pustular vasculitis distinct from pustular psoriasis.

Sternocostoclavicular hyperostosis. Presentation and long-term follow-up of three cases.

Sternocostoclavicular hyperostosis is a rare disease characterized by recurrent pain and skeletal swelling in the upper part of the chest. The clinical manifestations are closely linked to pustulosis palmo-plantaris but the etiology is still obscure. We present three cases of sternocostoclavicular hyperostosis with a follow-up period of 9-22 years at our department.

[Sternocostoclavicular hyperostosis]. / Hyperostosis sternocostoclavicularis.

On the basis of 4 patients from our own records we describe the rare syndrome of "Hyperostosis sternocostoclavicularis". The etiology of this disorder is still unclarified. In addition to a swelling that is generally only slightly tender to pressure, the most important symptom is usually a long-standing, intermittent, dull pain in the upper sternum, the claviculae and the adjacent uppermost ribs. Laboratory diagnosis usually shows an accelerated blood sedimentation rate, but other laboratory findings are unremarkable and rheumatological serology gives a negative result. The changes detected by radiography are described, as are the histological findings. Primary or secondary malignant osteomas are also to be taken into account in differential diagnosis. For therapy most authors recommend medicinal treatment with non-steroidal antiphlogistic agents.

Spinal changes in association with sternocostoclavicular hyperostosis.

A case of sternocostoclavicular hyperostosis was reported in a 63-year-old woman who had been followed for 15 years. Radiographic changes in the claviculo-sternal area were typical of this condition, and biopsy revealed abnormalities in the right clavicle and in the sternum compatible with infection. Radiographic changes in the thoracic and lumbar spine revealed findings compatible with infective spondylitis and a seronegative spondylarthropathy, respectively. The nature of these spinal changes as compared with those of diffuse idiopathic skeletal hyperostosis and seronegative spondylarthropathy is discussed.

Sternocostoclavicular hyperostosis or pustulotic arthroosteitis.

We describe a 68-year-old woman who had suffered pain, swelling, heat, and redness in the region of both clavicles for the last 2 years. Her erythrocyte sedimentation rate was markedly elevated; tests for rheumatoid factor were negative. At surgical exploration, ankylosis of the sternoclavicular joints, especially on the left side, was found. Biopsy revealed chronic nonspecific inflammation with new bone formation, consistent with the diagnosis of sternocostoclavicular hyperostosis or pustulotic arthroosteitis.

[Roentgenological analysis of sternocostoclavicular hyperostosis by computed tomography].

Roentgenological analysis of the anterior chest wall was performed in twenty six patients with sternocostoclavicular hyperostosis. Initial hyperostotic change was seen at the first costosternal junction with ventral protrusion. Hyperostosis gradually developed around the first ribs, and irregular hyperostotic changes were also seen along the costoclavicular ligaments. Even in the final stage, however, sternoclavicular joint spaces were well preserved. These findings suggest that sternocostoclavicular hyperostosis is a disorder initiated around the costal cartilage including the periosteum and perichondrium, and that arthritis is not a condition stemming from that disorder.