Macrognathia

General factors which would influence and tend to favour mandibular prognathism are as follows:

• Increased height of the ramus

• Increased mandibular body length

• Increased gonial angle

• Anterior positioning of the glenoid fossa

•Decreased maxillary length

• Posterior positioning of the maxilla in relation to the cranium

• Prominent chin button

•Varying soft-tissue contours

Source- textbook of oral pathology Shafers and Google images

REITER’S SYNDROME

Reiter’s syndrome is associated with urethritis, balanitis, conjunctivitis, and mucocutaneous lesions.

 It is a disease of unknown aetiology, although there is evidence of an infec- tious origin.

It is one of the most common complications of non-specific urethritis and it clinically mimicks gonorrhoea, although the urethral discharge is negative for Neisseria.

CLINICAL FEATURES

>Reiter’s syndrome is more prevalent in young adult men, usually between 20 and 30 years of age. 

>The male-to-female ratio is 9:1. 

>There is a typical tetrad of manifestations: non- gonococcal urethritis, arthritis, conjunctivitis, and mucocutaneous lesions. 

>Urethritis may be the first sign. The urethral discharge is usually associated with itching and burning sensation. 

>The arthritis is often bilaterally symmetrical and usually polyarticular.

 >Conjunctivitis is often so mild as to be overlooked. 

>The skin lesions are similar to those seen in keratoderma blennorrhagica and consist of red or yellow keratotic macules or papules which eventually desquamate.

Oral Manifestations

Sites—it is seen on the buccal mucosa, lips and gingiva.

Oral lesions appear as painless, red, slightly elevated areas, some- times granular or even vesicular, with a white circinate border on the buccal mucosa, lips, and gingiva. 

The palatal lesions appear as small, bright red purpuric spots, which darken and coalesce, while the lesions on the tongue closely resemble ‘geographic’ tongue.

Laboratory Findings

The patients usually have a mild leukocytosis, an elevated erythrocyte sedimentation rate, and pyuria.

  • Differential Diagnosis
  • • Geographic tongue and stomatitis—no skin changes, no visceral lesions are seen.
  • • Pustularpsoriasis—Auspitz’ssignpresent.
    • Behcet’ssyndrome—nourethritis,aphthaewithredhalo.
  • • Stevens-Johnson syndrome—acute appearance, moresevere clinical course, no arthritis or urethritis.
    • Benign mucosal pemphigoid—blister formation, nourethritis, found in older patients.
Histologic Features
The microscopic findings are not diagnostic. They consist of parakeratosis, acanthosis, and polymorphonuclear leukocyte infiltration of epithelium, sometimes with mi- croabscess formation similar to psoriasis. The connective tissue shows a lymphocyte and plasma cell infiltrate.
  • Management
  • • Spontaneous remission—many patients undergo spontaneous remission.• Antibiotics—incasesymptomaticpatient,doxycycline or minocycline may be given.
  • • Analgesics—nonsteroidalanti-inflammatorydrugsare given to manage arthritis.
  • • Immunosuppressiveagents—immunosuppressiveagents like azathioprine and methotrexate are given in cases of most resistant cases.

REFERENCE- SHAFER’S TEXTBOOK OF ORAL PATHOLOGY [8TH ED} AND ANIL GHOM TEXTBOOK OF ORAL MEDICINE

Fibromatosis gingivae

Histopathology

Treatment-

•When tooth eruption is impeded, surgical removal of the excessive tissue and exposure of the teeth are indicated.

•The cosmetic appearance may also require surgical excision.

•The lesion sometimes recurs. It has been reported that tooth extraction alone will cause the tissues to shrink almost to normal and that recurrences can be prevented by this.

Source- textbook of oral pathology Shafers and Google images

Letterer – Siwe Disease

  • Rare, acute aggressive disseminated proliferative lethal disease of Langerhans cells that affects infants <2 years old.
  • Accounts for 10% of cases of Langerhans cell histiocytosis

Clinical features:-

  • Initial manifestation often skin rash involving trunk, scalp & extremities.
  • Recurrent pyoderma – like lesions with crusting and scaling, vesicopustular and purpuric eruption; resembling seborrhoeic dermatitis.
  • Denuded skin may facilitate microbial invasion, leading to sepsis.
  • Persistent low grade spiking fever with malaise & irritability.
  • Splenomegaly, hepatomegaly & lymphadenopathy are early manifestations.
  • Nodular lesions in body flexures and intraoral haemorrhage.
  • Myelophthisic anemia , leukopenia and thrombocytopenia may also be seen.
  • Bone lesions are not common initially.
  • Diffuse involvement of skeletal system occurs later in the disease.
  • The uvea may be affected; orbital involvement is most unusual
  • The pustules are sterile and Tzanck smear of the vesicopustules shows pale histiocytes which can be used as a rapid screening test.

Oral manifestations:-

  • Ulcerative lesions, gingivitis, loose teeth, ectopic teeth also may be seen.
  • Parents frequently report precocious eruption of teeth, when in fact the gums are receding to expose immature dentition
  • Diffuse destruction of maxilla & mandible.
  • Some cases extreme rapid course of disease; oral involvement does not occur.

Histologic Features:-

1.Very similar to HSC; histiocytic proliferation with or without eosinophils.

2.These histiocytes do not contain significant amounts of cholesterol.

3.‘Foam cells’ not a feature.

Treatment & Prognosis:-

  • Extremely poor; Course of disease rapid & terminates fatally in short time.
  • Poor prognostic features include younger age; dissemination of lesions; involvement of lungs, liver, CNS and RE system; associated infection; anaemia, thrombocytopenia and purpura
  • Chemotherapy coupled with radiation to localised bony lesions and supportive measures are useful in treatment.
  • Thymic extracts have been successfully used to treat LS disease

References:-

Shafer’s 8th edition

Hand Schuller Christian Disease (Multifocal Eosinophilic Granuloma)

  • Characterized by widespread skeletal & extra – skeletal lesions & chronic clinical course.
  • Occurs primarily in early life, before the age of 5; more common in boys & girls.
  • Most cases reported from Western literature, authentic cases from India are few.

Clinical features:-

  • Classic triad of :-
  1. Single or multiple areas of ‘punched out’ bone destruction in skull
  2. Unilateral or bilateral exophthalmos
  3. Diabetes insipidus with or without other manifestations of dyspituitarism
  • Involvement of facial bones frequently associated with soft tissue swelling & tenderness; causes facial symmetry.
  • Classically involves the flat bones of the skull, ribs, pelvis, and scapula
  • Diabetes insipidus affects 5 to 50% of patients.
  • Skin involvement in the form of erythematous scaly rash or papular or nodular lesions which is seen in only 30% of patients.
  • Poor sexual development and retarted growth is another feature.
  • Vision loss or strabismus caused by optic nerve or orbital muscle involvement occurs rarely.
  • Chronic otitis media and otitis externa due to involvement of the mastoid and petrous portions of the temporal bone with partial obstruction of the auditory canal are fairly common

Oral manifestations:-

  • Earliest signs of the disease; may be present in 5 – 75% of cases.
  • Often non specific; sore mouth, halitosis, gingivitis, ulcerations, loose teeth, suppuration, failure of healing of extraction sockets
  • Loss of supporting alveolar bone mimicking advanced periodontal disease is characteristic.

Radiological features:-

  • Skull lesions sharply outlined; jaw lesions may be diffuse.
  • Jaw lesions manifested as bone destruction with tooth displacement.
  • Panoramic may show remarkable atrophy of the alveolar ridge and severe parodontitis.
  • Remaining teeth abnormally sited in an extra alveolar position

 

Histological features:-

  • Manifesting in four stages during progression of characteristic lesion
  • Proliferative histiocytic phase with accumulation of collections of eosinophilic leukocytes scattered throughout sheets of histiocytes.
  • Vascular – granulomatous phase with persistence of histiocytes & eosinophils, sometimes with aggregation of lipid laden (cholesterol) macrophages.
  • Diffuse xanthomatous phase with abundance of ‘foam cells’
  • Fibrous healing phase.
  • Infiltration with CD1 positive histiocytes disclosing intracytoplasmic Birbek granules at the electron microscopic examination .
  • Histological diagnosis is based on the presence of a histiocytic infiltrate in the upper and middle dermis.
  • The optical microscope examination reveals in the papillary dermis an important oedema, large cells with an indented nucleus and abundant eosinophilic cytoplasm
  • Histochemical colouring, showing positiveness for S-100 protein, the presence of CD1, CD4 and HLA-DR surface antigens confirm the diagnosis

Treatment & prognosis:-

  • Therapy of the Hand-Schuller-Christian disease varies according to the age of the patient, the severity and extent of the clinical picture.
  • Approximately half of the patients undergo spontaneous remission over a period of years.
  • Treatment of choice is curettage or excision of lesions.
  • Inaccessible lesions may be irradiated.
  • Some patients may benefit from chemo – therapeutic drugs like Prednisone, Vinblastine & Cyclophosphamide.

Reference:

1.Faculty notes

2.Google