Diagnostic delay in sternocostoclavicular hyperostosis: impact on various aspects of quality of life.

OBJECTIVE: Sternocostoclavicular hyperostosis (SCCH) is a rare, debilitating, chronic inflammatory disorder of the anterior chest wall due to a chronic sterile osteomyelitis of unknown origin, often associated with characteristic skin lesions of palms and soles: pustulosis palmoplantaris. SCCH goes often unrecognized for years before the diagnosis is established and treatment instituted. The objective of this study was to trace the diagnostic paths of patients with SCCH and to investigate the consequences associated with diagnostic delay. METHODS: Data were collected through structured interviews of 52 patients with a clinically, scintigraphically, and radiologically established diagnosis of SCCH. RESULTS: The majority of patients presented with swelling and/or pain in the sternocostoclavicular region and/or limited movement of the shoulder girdle. Pustulosis palmoplantaris was present in approximately 30% of patients. The disease went unrecognized for a median of 3.5 years. Patients were often seen by at least 3 members of the medical profession before the diagnosis was suspected and eventually established. Lack of recognition of the clinical manifestations of the disease and delay in diagnosis were associated with important physical, psychological, and socioeconomic consequences affecting quality of life. CONCLUSION: SCCH remains an ill-recognized disease despite its characteristic clinical features. A low level of awareness of the disorder leads to a delay in diagnosis, which has a significant impact on various aspects of quality of life. Awareness should be raised for this disorder, enabling timely diagnosis and initiation of treatment to prevent the irreversible physical and psychological sequelae associated with the protracted untreated state.

Tonsillar focal infectious disease involving IgA nephropathy, pustulosis, and ossification.

We report a rare case of IgA nephropathy (IgAN), that was considered as showing tonsillar focal infection, involving pulmoplantar pustulosis (PPP), and sternocostoclavicular hyperosteosis (SCCH). A 53-year-old man with a 3-year history of PPP had hematuria and proteinuria, and he sometimes had anterior chest pain. He was also diagnosed with IgAN and SCCH. We performed tonsillectomy as a treatment. The tonsillectomy was done with the patient under general anesthesia, and this treatment was followed by steroid therapy. Interestingly, all the symptoms of IgAN, PPP, and SCCH were alleviated 6 months after the tonsillectomy. Thus, tonsillectomy and steroid therapy may be effective and could be considered as treatment for these diseases.

[Isolated bilateral Sternocostoclavicular Hyperostosis (SCCH) with a pathological clavicular fracture--a 10-year follow-up]. / 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.

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.

[Acquired hyperostosis syndrome (so-called pustular arthro-osteitis). Review of the literature including 73 personal cases]. / Akquiriertes Hyperostose-Syndrom (sogenannte pustulöse Arthroosteitis). Literaturübersicht einschliesslich 73 eigener Beobachtungen.

The acquired hyperostosis syndrome (AHS) (best known synonym: pustulotic arthro-osteitis) is a system disease of the supporting and gliding tissue with sites of predilection characterized by inflammation-induced bony reconstruction of positive balance. This syndrome is affiliated with the seronegative spondylarthropathies. The main finding is the sternocostoclavicular hyperostosis in about 80% of patients. Focal hyperostoses also occur on the skeleton of truncus and extremities and joints. AHS is accompanied by psoriasiform and acneform dermatoses. Overlapping findings with spondylitis ankylosans are reported. Terminology, aetiology, nosology, pathogenesis, histomorphology, clinical and laboratory findings, complications, imaging diagnostic, differential diagnosis and therapy of AHS are discussed. Knowing AHS helps to prevent misdiagnoses (as especially bacterial osteomyelitis, spondylitis, osteoplastic tumor and metastases) and interventional diagnostic procedures.

[The acquired hyperostosis syndrome. Synthesis of 13 personal observations of sternocostoclavicular hyperostosis and 300 cases from the literature. 1]. / Das akquirierte Hyperostose-Syndrom (AHS). Synthese aus 13 eigenen Beobachtungen von sternokostoklavikulärer Hyperostose und über 300 Fällen aus der Literatur--Teil 1.

Sterno-costo-clavicular hyperostosis (SCCH) is the most common manifestation of a syndrome, consisting of increased bone metabolism, mostly new bone formation and heterotopic ossification of fibrous tissue, which we have characterised as the acquired hyperostosis syndrome. In part I we discuss the terminology, radiological appearances, scintigraphy, clinical and laboratory findings, bacteriology, histology, nosology, complications, treatment and differential diagnosis of SCCH. Chronic recurrent multifocal osteomyelitis (CRMO) is regarded as a phenotype of SCCH, depending on the age. CRMO occurs in children, adolescents and young adults, SCCH predominantly in middle-aged and elderly adults.