Destructive diseases of the jawbones and periodontium
Many systemic diseases lead to severe lesions of the supporting dental tissues and the jawbone itself.
- Takahara syndrome. This is a hereditary pathology associated with a deficiency of the enzyme catalase. As a result of hydrogen peroxide accumulation, an environment is created for the proliferation of anaerobes. The disease causes rapid progression of gingivitis to periodontitis, the formation of deep pockets, and tooth loss. An important distinguishing feature: after tooth loss, the process does not stop and often progresses to focal necrosis of the jawbones.
- Hand-Schüller-Christian disease. A storage disease (histiocytosis) affecting predominantly children. Specific radiolucent lesions form in the cranial bones. In the oral cavity, the disease manifests with bleeding, gum edema, and completely painless tooth loss (molars are usually the first to shed).
- Chédiak-Higashi syndrome. Occurs against the background of hereditary neutropenia. Patients suffer from hyperplastic gingivitis and pronounced atrophy of the alveolar process, which leads to severe tooth mobility.
TMJ pathology and dentofacial anomalies
Temporomandibular joint disorders are most often associated with systemic connective tissue defects.
In Ehlers-Danlos syndrome, congenital hyperelasticity of structures is observed. In the maxillofacial region, this leads to the development of chronic TMJ subluxation. In addition, dental anomalies are detected: the shape of the tooth roots is disrupted, and calcifications are found in the pulp.
Disorders of tooth eruption timing and shape are characteristic of several other conditions:
- Hutchinson-Gilford syndrome (progeria): deciduous teeth are retained for too long, and permanent tooth eruption is delayed.
- Jadassohn-Lewandowsky syndrome: teeth erupt late, are rapidly destroyed by caries, and are shed early.
- Urbach-Wiethe syndrome: prolonged retention of the deciduous bite is noted.
Symptomatology of soft tissues and mucous membranes
Although bone and joint changes are prominent in the described pathologies, they are always evaluated in conjunction with changes in the oral mucosa and tongue.
- Enlargement of the tongue (macroglossia). Characteristic of systemic amyloidosis (Strauss syndrome), when amyloid is deposited in the muscles, forming dense elevated formations. The tongue becomes enlarged, painful, and covered with a grayish-white coating.
- Ulcerative-necrotic lesions. In severe infectious-allergic lesions, a continuous erosive surface with a fibrin film forms on the oral mucosa. Such conditions differ from Fuchs syndrome (which is also characterized by pseudomembranous coatings on the lips and tongue) by the obligatory presence of fever.
- Granulation overgrowths. In children suffering from pertussis, constant mucosal trauma by the lower incisors during coughing can lead to Riga-Fede disease — the formation of a sublingual granuloma that impairs swallowing and sucking.
Changes in the function of the maxillofacial glands
Salivation assessment is an essential part of the examination in maxillofacial pathologies. In Sjögren's syndrome, marked dysfunction of all exocrine glands is observed. Patients suffer from xerostomia (dryness), burning tongue, lip fissures, and a sharp enlargement of the parotid salivary glands.
In turn, Heerfordt's syndrome (uveoparotid fever) also causes bilateral parotitis, but it occurs against the background of subfebrile fever, reversible facial nerve palsy, and eye lesions (uveitis, diplopia). Sialography reveals altered duct architecture of the parotid glands.
Briefly: answers to topical questions
What radiographic signs are characteristic of jaw osteomyelitis?
The radiographic picture of jaw osteomyelitis depends on the stage and clinical form of the disease.
- Acute stage — in the first 10–14 days, destructive changes are not defined; widening of the marrow spaces, blurring of the trabecular pattern, and a soft tissue infiltration shadow are detected.
- Chronic destructive form — foci of bone rarefaction with the shadow of a formed sequestrum inside, surrounded by a capsule and a rim of reactive osteosclerosis.
- Chronic destructive rarefying form — multiple small rarefaction foci with areas of osteosclerosis between them.
- Chronic productive hyperplastic form — jaw deformation, hyperostosis, intense bone densification areas, erased trabecular pattern without signs of sequestration.
What clinical examination methods for the temporomandibular joint exist?
Clinical examination of the temporomandibular joint includes functional assessment and palpation.
- Functional assessment — evaluation of mouth opening and closing, as well as lateral movements of the mandible (left and right). Normally, opening is free, painless, without clicking or deviation, and the distance between the central incisors is 5 cm.
- Palpation — examination of the condylar head and its articulation with the articular fossa.
Attention is also paid to the presence of acoustic phenomena (crepitus, clicking), stiffness, and pain, which may indicate joint pathology.
What radiographic changes in the jawbones are observed in Paget's disease?
Sources describe structural and morphological changes in the jaws in Paget's disease, occurring in three stages that form the radiographic picture.
- Osteolytic stage — active bone resorption and thinning of the cortical plate.
- Osteoblastic stage — bone trabeculae acquire a chaotic configuration, improperly oriented cement lines and mosaic structures are formed.
- Osteosclerotic stage — a sharp decrease in the number of osteoclasts and an absence of osteoid deposition signs.
What types of jaw deformities occur in unilateral TMJ ankylosis?
In TMJ ankylosis that develops in childhood, condylar hypoplasia occurs due to damage to the epiphyseal growth center. Unilateral retrognathia is characterized by underdevelopment of the mandible, deviation of the chin toward the affected side, and soft tissue asymmetry: the cheek is convex on the affected side and flattened on the healthy side. Secondary maxillary deformation manifests as depression of the alveolar process and dental arch on the healthy side.
In which systemic diseases is macroglossia observed?
Macroglossia (enlargement of the tongue) is observed in the following systemic conditions:
- Systemic amyloidosis (including Strauss syndrome) — macroglossia is one of the earliest signs along with anemia. The tongue is significantly enlarged, painful, covered with a grayish-white coating and multiple dense tumor-like amyloid deposits.
- Acromegaly — an endocrine disorder caused by a growth hormone-secreting pituitary adenoma in adults. It is accompanied by enlargement of the tongue, distal body parts, and the mandible.
What sialographic changes are characteristic of Sjögren's syndrome?
In Sjögren's syndrome, sialography in the early stage reveals punctate cavities in the salivary gland parenchyma. This is combined with xerostomia, xerophthalmia, and a systemic autoimmune or rheumatic disease.
Class and exam questions
In which disease does tooth loss occur painlessly and begin with the molars?
This symptom is a hallmark of Hand-Schüller-Christian disease (a type of histiocytosis).
Why is the TMJ affected in Ehlers-Danlos syndrome?
Due to a congenital connective tissue anomaly, increased laxity of the ligamentous apparatus occurs, leading to chronic subluxation of the temporomandibular joint.
What are the risks of Takahara syndrome following tooth loss?
Unlike conventional periodontitis, in Takahara syndrome the process does not resolve after tooth extraction and may progress to extensive focal necrosis of the jawbones.
How to differentiate Fuchs syndrome from severe infectious-allergic lesions with similar clinical presentations in the oral cavity?
A key differential diagnostic sign is the absence of fever in Fuchs syndrome.
What else to study on this topic
- Salivary gland changes in autoimmune processes
- Manifestations of histiocytosis in the jawbones
- Genetic causes of alveolar ridge atrophy
- Role of catalase in the development of bone tissue necrosis
- Association of systemic amyloidosis with macroglossia
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