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]

Annie S. Morrison, M.D.
Kelly Magliocca, D.D.S., M.P.H.
Page views in 2024 to date: 512
Cite this page: Morrison A. Diffuse sclerosing. website. Accessed May 19th, 2024.
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
  • Pathophysiology and etiology is unknown
  • Terminology
    • Sequestrum:
    • 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:
        1. Acute Osteomyelitis (AO)
        2. Secondary Chronic Osteomyelitis (SCO)
        3. 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
    • 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
    • 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
    • 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
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