Mandible & maxilla
Osteomyelitis and inflammatory conditions
Diffuse sclerosing
Last author update: 1 March 2015
Last staff update: 19 April 2023
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PubMed Search: Diffuse sclerosing osteomyelitis [title]
Table of Contents
Definition / general | Terminology | Sites | Clinical features | Diagnosis | Radiology description | Case reports | Treatment | Gross description | Microscopic (histologic) description | Differential diagnosis | Additional referencesCite this page: Morrison A. Diffuse sclerosing. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mandiblemaxillaDSO.html. Accessed September 27th, 2023.
Definition / general
- Sclerosing osteomyelitis has traditionally been divided into focal and diffuse types:
- The focal type, also known as condensing osteitis, describes a relatively common, limited, well circumscribed radiopaque alteration of bone surrounding the apex of a root of a tooth, the latter often with an inflamed or necrotic dental pulp
- Diffuse sclerosing osteomyelitis, on the other hand, is an uncommon condition affecting a large portion of the bone, usually mandible, and is associated with mandibular expansile remodeling and clinical symptoms
- Many views of DSO exist in the literature:
- DSO is a form of primary chronic osteomyelitis (PCO) - the remainder of this topic follows this view
- DSO is synonymous to juvenile chronic osteomyelitis and an early onset form of PCO
- DSO is a localized form of SAPHO
- DSO is distinct from PCO and the term DSO is only used when an obvious infectious process is responsible for sclerosis of bone (i.e. with a chronic intraosseous bacterial infection which creates chronically inflamed granulation tissue that incites sclerosis of the surrounding bone)
- DSO is used only as a description of radiological features
- Both primary and secondary chronic osteomyelitis may demonstrate radiographic changes of extensive sclerosis with periosteal reaction (proliferative periostitis) and variable osteolysis on diagnostic imaging
Terminology
- Sequestrum:
- Predominantly nonviable bone that has become separated during the process of necrosis from the original surrounding native bone (Medical Dictionary: sequestrum [Accessed 4 June 2018] )
- Periosteum
- Connective tissue membrane lining the outer / external surface bones, except at the joints of long bones
- Is composed of an outer fibrous layer and an inner cambium / osteogenic layer
- Chronic recurrent multifocal osteomyelitis (CRMO)
- Chronic recurrent multifocal osteomyelitis (CRMO) is a relapsing inflammatory disease and is considered a type of seronegative spondyloarthropathy characterized by periods of exacerbations and remissions over many years
- Characterized by an inflammatory process presenting with findings similar to infectious osteomyelitis; however, no infectious source is identifiable
- Most often characterized by the insidious onset of low grade fever, local swelling and bone pain
- Radiologic findings may suggest osteomyelitis, and bone seeking isotopes reveal other asymptomatic sites of involvement
- The lesions occur mainly in the metaphyses of long tubular bones, as well as the clavicles, spine, and pelvis; the lesions are sometimes symmetrically distributed
- The precise relationship between primary chronic osteomyelitis, diffuse sclerosing osteomyelitis, CRMO and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is highly debated and not clear at this time
- Some consider CRMO and SAPHO as primary chronic osteomyelitis with additional extragnathic manifestations
- Whether CRMO in juvenile patients and SAPHO syndrome are the same disorder in different age groups remains to be determined
- Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO)
- Acronym for a complex clinical presentation that includes Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis
- Unknown cause but may arise in genetically predisposed individuals who develop an autoimmune disturbance due to exposure to dermatologic bacteria
- Increased prevalence of HLA-27 (10 - 15% cases of SAPHO)
- SAPHO and Chronic recurrent multifocal osteomyelitis / CRMO are distinguished from other forms of osteitis / osteomyelitis as these two diseases may have extragnathic skeletal involvement
- The precise relationship between primary chronic osteomyelitis, diffuse sclerosing osteomyelitis, CRMO and SAPHO is controversial - some consider CRMO and SAPHO as primary chronic osteomyelitis with additional extragnathic manifestations
- Proliferative periostitis
- Represents a periosteal reaction to the presence of inflammation
- The affected periosteum forms several rows of reactive vital bone that parallel each other and expand the surface of the altered bone
- Garrè sclerosing osteomyelitis
- In 1893, Swiss physician Carl Garrè reported on patterns of and complications of acute osteomyelitis
- Garrè did not have any pathologic specimens for microscopic examination nor radiographs to augment his descriptions
- Nonetheless, his name is often associated with the condition in which periosteal duplication or "onion-skinning" pattern of periostitis leads to enlargement of the jaw, usually mandible
- Cases of osteomyelitis with a predominant periosteal reaction are in some instances falsely labeled as Garrè osteomyelitis
- Garrè osteomyelitis and Garrè sclerosing osteomyelitis are often used synonymously and should be disassociated with this clinical presentation
- Note that Garrè name was and is often misspelled as Garré with an improper accent
- Numerous synonyms for Garrè sclerosing osteomyelitis have been used:
- Chronic osteomyelitis with proliferative periostitis
- Chronic sclerosing osteomyelitis of Garrè
- Chronic non-suppurative osteomyelitis with proliferative periostitis
- Garrè chronic sclerosing osteomyelitis
- Garrè chronic nonsuppurative sclerosing osteitis
- Garrè proliferative periostitis
- Garrè sclerosing osteomyelitis
- Ossifying periostitis
- Osteomyelitis of Garrè
- Osteomyelitis sicca
- Osteomyelitis with proliferative periostitis
- Periostitis ossificans
- Sclerosing osteomyelitis of Garrè
- Condensing osteitis
- Localized areas of radiographic bone sclerosis associated with apices of inflamed, dead or dying teeth (pulpitis or pulpal necrosis)
- The association with an area of inflammation, usually the apex of an associated tooth, is critical, because these lesions can resemble several other intrabony processes that produce a somewhat similar radiographic pattern
- This secondary sclerosis of bone is not considered a true / classical osteomyelitis
- Zurich Classification of Osteomyelitis:
- Primarily based on clinical course and imaging features
- Histopathology is considered a secondary classification criterion because findings are mostly unspecific and inconclusive by themselves; however, tissue examination is irreplaceable to confirm diagnosis, when clinical and radiological appearance are unclear / atypical, and to exclude possible differential diagnoses
- The three major classifications are:
- Acute Osteomyelitis (AO)
- Secondary Chronic Osteomyelitis (SCO)
- Primary Chronic Osteomyelitis (PCO)
- Further subclassification is based on presumed etiology and pathogenesis of disease
- These criteria are considered tertiary classification criteria, and are helpful to determine the necessary therapeutic strategies which may differ somewhat among the subgroups
Sites
- When involves the jaws, predominantly involves the mandible
Clinical features
- Insidious onset, lacking an obvious acute state
- Often affects children and young adults
- Pain (days to weeks, often with symptom free intervals), swelling and limitation of mouth opening
- Jaw expansion, usually mandible, in the absence of pus, fistula and sequestration
Diagnosis
- Clinical diagnosis challenging because the clinical picture and course of the disease may vary significantly
- Whether DSO represents variations of a single disorder or different pathologic processes is highly debated with no clear answer at this time
- Clinicians should consider all possibilities to ensure appropriate care
Radiology description
- Expansile remodeling of jaw, usually mandible
- Sclerosis, punctuated by areas of lytic change
- Periosteal duplication
Case reports
- Diffuse sclerosing osteomyelitis (DSO) of the mandible in SAPHO syndrome (J Craniomaxillofac Surg 2014;42:1990)
Treatment
- Treatment is influenced by stage, extent and symptoms of disease
- May include NSAIDs, TNF inhibitors, bisphosphonates
Gross description
- Biopsies to rule out neoplasia may be minute
Microscopic (histologic) description
- Histology may be nonspecific and may mimic acute and secondary chronic osteomyelitis due to microbiological infection
- An extensive microbiological workup including culture and PCR may be indicated
- Bone biopsy is usually performed to exclude malignancy and competing radiographic and clinical differential diagnostic entities
- Microscopic findings vary with area sampled (as do radiographics)
- Chronic phase: paucity of inflammation with predominant sclerosis and fibrosis
- Prior biopsy or sample site: organizing fibrous and granulation tissue with mixed inflammation
- Reactive and remodeling bone, particularly in a region of periosteal reaction
- Unerupted tooth buds may be disturbed or inadvertently removed during mandibular biopsy
Differential diagnosis
