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PubMed1993

The acquired hyperostosis syndrome: a little known skeletal disorder with distinctive radiological and clinical features.

The acquired hyperostosis syndrome (AHS) is a chronic inflammatory disorder of bone of unknown etiology. It is accompanied by circumscribed hyperostosis which can be associated with ossifying lesions at sites of tendinous and ligamentary insertions and erosive or non-erosive arthritis. The predominant location of lesions is the sternocostoclavicular region (approximately 80% of patients), less frequent are involvement of the spine, pelvis, and appendicular skeleton. In 20%-60% of cases AHS is associated with palmoplantar pustulosis, psoriasis, or severe acne (acne fulminans or conglobata). The X-ray appearance of AHS is a more or less homogeneous increase in density with blurred margins, which on scintiscan with labeled phosphate compounds is associated with intense accretion of tracer. These features are associated with a variable increase in the acute phase reactants and a conspicuously low increase, if any, in serum alkaline phosphatase. The therapeutic modalities which have been used so far are entirely symptomatic. Long-lasting improvement has been reported following percutaneous anti-inflammatory radiation therapy.

Acne Vulgaris↗
PubMed1993

[Acquired hyperostosis syndrome (so-called pustular arthro-osteitis). Review of the literature including 73 personal cases].

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.

Diagnosis, Differential↗
PubMed1997

Acquired hyperostosis syndrome--AHYS--(sternocostoclavicular hyperostosis, pustulotic arthro-osteitis, SAPHO-syndrome): bone scintigraphy of the anterior chest wall.

The objective of this report is to provide a description of diagnostically significant scintigraphically recognizable sites and patterns of acquired hyperostosis syndrome (AHYS) on the anterior chest wall (ACW), which is involved in 82% of AHYS patients. In 49/90 of our own AHYS patients, planar bone scans of the ACW were performed with the gamma camera, applying an average of 650 MBq of 99mTc-phosphate complexes. In addition, 53 atraumatic patients with extrathoracic cancer were available for routine whole-body scintigraphy. None of these patients had increased uptake identifiable as metastasis clinically or by imaging modalities in either the ACW or the rest of the skeleton. The scintigraphic involvement of the various morphological ACW structures is described in AHYS. Moreover, attention is called to the diagnostic significance of focal hyperactivities at the anterior end of the 2nd-8th rib of adults, which are in the 5th place with respect to their frequency in AHYS. The diagnostic significance of sternocostal-joint involvement in AHYS can likewise be recognized by bone-scan scintigraphy and will be discussed. Bone scintigraphy is more sensitive than radiomorphological imaging in AHYS. This, however, only applies under three conditions. 1. The increased radiotracer uptake in the upper sternocostoclavicular region must be assessed on both the anterior and the posterior view of the ACW scan. 2. In addition to the anterior view of the routine scintiscan, further anterior scans with reduced scan time of the gamma camera are usually necessary. This ensures better visibility of the involvement of certain morphological structures that are important for AHYS diagnosis. Moreover, a statement can be made about the inflammatory ossifying activity/inactivity of the AHYS on ACW.3. Increased radionuclide uptake in the manubrium sterni and corpus sterni on the anterior scan should be verified by additional lateral or oblique scans of the thorax (sternum).

Acne Vulgaris↗
PubMed1988

[The acquired hyperostosis syndrome. Synthesis of 13 personal observations of sternocostoclavicular hyperostosis and 300 cases from the literature. 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.

Adult↗
PubMed1991

Acquired hyperostosis syndrome: spectrum of manifestations at the sternocostoclavicular region. Radiologic evaluation of 34 cases.

Thirty-four patients with chest wall hyperostosis, a condition which has been designated by various terms in the literature were evaluated radiologically. We prefer the name acquired hyperostosis syndrome (AHS), which we categorize into the complete, incomplete and possible form. In complete AHS, sternocostoclavicular hyperostosis is associated with axial and/or peripheral (endosteal, periosteal, enthesopathic, metaplastic) hyperostosis and with psoriasiform or acneform dermatosis. In addition, these three manifestations are accompanied by erosive or non-erosive peripheral and/or axial arthritis to a variable degree. Sometimes, concomitant findings which are consistent with ankylosing spondylitis are also to be found in the axial skeleton. AHS is manifested at 11 different sites on the anterior chest wall. Ossification forms of the costal cartilage, inflammatory enthesopathies (three different insertions) and focal hyperostoses as well as processes of remodelling of the ribs, clavicles and sternum which are described in detail have particular diagnostic significance. AHS can start simultaneously at one, two or several sites on the anterior chest wall. Conventional tomography (possibly supplemented by CT) is necessary for early diagnosis and for analysis of the various findings on the anterior chest wall.

Adult↗
PubMed1988

[The acquired hyperostosis syndrome. 2].

In the second part of this publication, we describe some additional findings in cases of sternocostoclavicular hyperostosis. These include focal hyperostosis of the spine, in the pelvis and in the extremities and psoriatic skin lesions and severe forms of acne (acne conglobata, acne fulminans). Other features, which are not diagnostic, include erosive or non-erosive peripheral arthritis and unilateral or bilateral sacro-iliitis. An analysis of our 13 patients and of the relevant literature indicates that the hyperostosis is due to increased bone metabolism and heterotopic ossification of fibrous tissue and that these are the pathogenic bases of the changes in the axial skeleton, the pelvis and the bones of the extremities. We have suggested a scheme which would categorize the syndrome into complete, incomplete and possibly acquired forms.

Adult↗
PubMed2000

[Spinal stiffness. Differential imaging diagnosis with reference to acquired hyperostosis syndrome and spinal loading disease].

There are numerous diseases which can irreversibly stiffen the whole spine or parts of the axial skeleton. Due to didactic reasons one can distinguish 3 groups of such disorders: Paradigmatic diseases of the spine with an inherent proneness to stiffness. Rare diseases which involve a certain danger of stiffening the spine. Rigidity of the spine due to scientifically unrecognised morphologic phenomena.

Diagnosis, Differential↗
PubMed1998

[Chronic recurrent multifocal osteomyelitis (CRMO)].

Chronic recurrent multifocal osteomyelitis (CRMO) is an unusual clinical entity. More than 200 cases are described in the literature and it is presented here with special reference to its radiological aspects. It is an acquired disease of the skeleton which occurs predominantly during childhood and adolescence. About ten per cent of cases begin in early or, rarely, in later adult life. This variant is described here for the first time and is discussed as "adult CRMO". The underlying pathology is a bland, predominantly lympho-plasma cellular osteomyelitis which is self-limiting and leads to bone sclerosis (Garrè). It probably involves an abnormal immune process which follows an infection but remains clinically latent and remains aseptic and sterile. In a quarter of cases there is an association with pustulosis palmo-plantaris and its relationship with psoriatic arthropathy is discussed. The clinical, histopathological and imaging features (radiological and particularly MRT) and the bone changes are described. This provides a spectrum of symptoms; the radiological differential diagnosis and the relationship with hyperostotic spondyloarthroses during adult life are discussed. The relationship between CRMO, the SAPHO syndrome and acquired hyperostosis syndrome are analysed.

Adult↗
PubMed1998

[Spondarthritis hyperostotica pustulo-psoriatica: nosologic study with clinical and radiologic presentation in relation to the SAPHO syndrome].

Spondarthritis hyperostotica pustulo-psoriatica (Spond.hyp.pp-Schilling), corresponding nosologically to pustulotic arthroosteitis, is a dermato-skeletal "double system" disease of adults. It consists of the triad (a) palmo-plantar pustulosis (Ppp) or, alternatively, Königsbeck-Barber-type psoriasis, (b) sternocostoclavicular hyperostosis (SCCH), and (c) truncal-skeletal changes with syndesmophite-like, hyperostotic and/or parasyndesmophite-like ossifications of layers of the anterior vertebral ligament taken together in the sense of a desmophytal hyperostosis. There is also a potential for sclerosing inflammatory arthritis of the sacro-iliac joints and "dry" inflammatory arthritis of peripheral joints. Thus, the pustulo-psoriatic terrain seems to have a decisive influence on osseous pathology. A total of 38 cases from a study during the years 1982 to 1992 is analysed with regard to morphological characteristics. Rare cases with diaphyseal and pelvic hyperostotic lesions subsequent to bland sclerosing osteomyelitis constitute an overlapping region to chronic recurrent multifocal osteomyelitis (CRMO) and illustrate the relationship between hyperostotic spondarthritis and CRMO. The syndromes of "acquired hyperostosis" and "SAPHO", the former more radiologically oriented and the latter more clinically oriented, together with mainly CRMO and hyperostotic spondarthritis and its forms, constitute the "Spond.hyp.pp.". Although hyperostosis is a guidepost for the radiologist and SAPHO symptoms are one for the clinician, the syndrome does not represent a diagnosis by itself and requires further differentiation. In this report the entity "Spond.hyp. pp." is considered and required contributions from rheumatologically and osteologically oriented radiologist.

Acquired Hyperostosis Syndrome↗
PubMed1995

Commented glossary for rheumatic spinal diseases, based on pathology.

OBJECTIVES: To redefine and comment on terms on a pathological basis, in order to avoid the confusion due to the use of terms with different meanings, to standardise usage among clinicians, radiologists and pathologists, and to facilitate literature searches. METHODS: Within the Committee of Pathology of the European League against Rheumatism, a study group was set up to analyse the medical literature and common practice concerning the nomenclature of rheumatic spinal diseases. The group tried to amalgamate the main trends in the field, to reconcile etymology, historical background, morphology, and common practice. RESULTS: The group warns against use of the terms 'acquired hyperostosis syndrome', '(von) Bechterew's disease', 'Kümmel's disease', 'pseudospondylolisthesis', 'rheumatoid spondylitis', 'spondylarthropathy' in the sense of spondarthritis, and 'spondylosis'. It recommends intercorporal or interapophyseal rather than intervertebral (osteo) chondrosis, zygapophyseal diverticulum rather than cyst, disc hernia rather than prolapse, spondyloarthritis rather than spondyloarthropathy, marginal rather than anterior spondylitis, and discarthrosis. It proposes 'zygarthrosis' to designate zygapophyseal osteoarthrosis. CONCLUSIONS: Knowledge of the pathological basis of diseases and an understanding of the original definitions given by those who coined new terms make it possible to avoid most of the confusion arising from improper use of spinal terms.

Humans↗
PubMed2004

[SAPHO syndrome].

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Acquired Hyperostosis Syndrome↗