Question 1. Describe oral findings in lupus erythematosus.
Answer:
Table of Contents
- Lupus erythematosus (LE) is an auto¬immune disease (in Latin, lupus = wolf, erythematosus = red). It has two types of involvement: Systemic and discoid. The pathognomonic finding is the facial butterfly rash, most frequently seen in systemic lupus erythematosus.
- The typical oral lesion in both systemic and discoid lupus comprises a central atrophic, red mucosa surrounded by a slightly raised, border. This border is composed of white paint-brush-like lines that run in the vertical direction. The various sites affected in the oral cavity are the palate, buccal, and vestibular mucosa.
Read And Learn More: Oral Medicine and Radiology Question And Answers
- In chronic cases of LE, the oral lesion appears as round, well-circumscribed, scaly, atrophic plaques. The long-standing oral lupus gives a characteristic honeycomb pattern to buccal mucosa due to mucosal scaring with keratotic lesions.
- Lip lesions are a well-circumscribed discoid pattern with diffuse cheilitis. The lesions spread out from the vermilion border to the adjacent skin.
- In systemic lupus erythematosus, xerosto¬mia secondary to Sjogren’s syndrome is also common.
- In the acute form, palatal erythema, bullous eruption with subsequent ulceration is common.
Lupus erythematosus Treatment:
- Interdisciplinary management is necessary for the prognosis.
- For oral lesions, intralesional injection of 3 mg/mL triamcinolone acetonide is effective.
- Systemic steroids are reserved for more severe cases.
- Methotrexate or azathioprine can be used as a steroid-sparing drug.
Question 2. Oral manifestations of scleroderma.
Answer:
- Rigid lips with a narrow oral aperture (microstomia) give the appearance of a fish mouth.
- Skin folds around the mouth disappear and gives a mask-like facial appearance.
- Telangiectasia on lips and hard palate.
- Pseudoankylosis of temporomandibular joints due to the involvement of surrounding soft tissues.
- The rigidity of the tongue makes speech and swallowing as difficult.
- Pseudoankylosis of TMJ
- Xerostomia
- Radiographic findings include uniform thickening of periodontal membrane especially around posterior teeth, involvement of facial muscles causing pressure- induced resorption on the mandible at an angle, coronoid process, and the condyle.
Question 3. Behcet’s syndrome.
Answer:
- Behcet’s disease or Behcet’s syndrome is a triple symptom complex characterized by recurrent oral, ocular, and genital ulcers and inflamed blood vessels.
- The underlying pathogenesis is immune-mediated systemic vasculitis and occurs in 3rd or 4th decade of life.
- The lesions are self-limiting with sequelae of remission and recurrence.
- Oral ulcers—Most common finding. Both major and minor aphthous ulcers occur in single crops. Healing of major ulcers takes place with mild scarring. Sympto¬matic management using topical analgesics and supportive medications may accelerate the prognosis.
- Genital ulcers—Both males and females develop genital ulcers. Usually, occur as superficial forms, but sometimes deep ulcers develop leading to fistulous tract formation with unfavorable prognosis.
- Ocular lesions—Retina and uvea are affected. Blurred vision, photophobia, and blindness are the complications.
- Apart from these classical traits, involvement of other systems like gastrointestinal, neurological, cutaneous, and cardiovascular are also common.
Lupus erythematosus Diagnosis:
- A positive pathergy test is associated with the active phase of Behcet’s syndrome. The test is performed by pricking a needle on the skin surface or intracutaneous injection of saline.
- Formation of a sterile pustule or small erythematous papule within 24-48 hours described the hypersensitivity reaction and was taken as definite diagnostic criteria for Behcet’s syndrome. The posi¬tivity did not correlate with the disease severity and related to geographic location (Mediterranean population).
- International study group criteria for diagnosis: Aphthous ulcerations which recur at least three times in a year in addition to any two of the following lesions will confirm the diagnosis.
- Genital ulceration, tender nodules on the skin, eye lesions or positive pathergy test.
- Treatment is aimed at reducing inflammation, relieving symptoms, and minimizing the recurrence.
- Combination therapy of corticosteroids and immunosuppressive drugs are used to reduce morbidity.
For oral lesions:
- Triamcinolone acetonide 0.1% cream 4 times/day is effective.
- For larger and widespread lesions, 0.5 mg/5 mL dexamethasone elixir 4 times rinse/day is useful.
- For major ulcers, intralesional 5-10 mg/ mL triamcinolone is indicated.
- Chlorhexidine or tetracycline mouth rinse (250 mg capsule dissolved in 5 mL of water) will reduce the pain and healing period.
- For severe or resistant oral lesions, systemic prednisone 40-60 mg/day for 2 weeks and then tapering over the next 4 weeks is considered.
Question 4. Sarcoidosis.
Answer:
- Sarcoidosis is an acquired granulomatous disease affecting multiple tissues and organs. The etiology is unknown. Pulmo¬nary and cutaneous involvements are more common, whereas head and neck involvement occurs in 10-15% of affected individuals.
- Bilateral enlargement of parotid glands and xerostomia are common findings.
- Oral lesions occur in chronic conditions and appear as non-tender, well-defined, brownish-red papules or as submucosal nodules. If superficial ulceration occurs, then these nodules or papules become symptomatic.
- Severe alveolar bone loss and mobility of teeth are also present. Gingiva appears erythematous and swollen in the interdental regions.
- The presence of typical noncaseating epithelioid granulomas on histopathological evaluation will support the diagnosis.
- Glucocorticosteroids with cytotoxic drugs (like methotrexate or cyclosporine) are the primary mode of treatment.
Question 5. Name the different types of immune reactions with examples.
Answer:
- Type 1 immediate hypersensitivity reactions— Anaphylactic shock, angioedema, urticaria, conjunctivitis, rhinorrhea, and rhinitis. It takes 15-20 minutes from exposure time to respond but sometimes may take 10-12 hours. It is mediated by IgE.
- Type 2 cytotoxic hypersensitivity reaction— Are mediated by IgG and IgM and takes minutes to hours for manifestation. Exam¬ples are drug-induced hemolytic anemia, granulocytopenia, and thrombocytopenia.
- Type 3 Immune complex hypersensitivity reaction—May be widespread (Serum sickness) or involve single organ like skin (Systemic lupus erythematosus, Arthus reaction), kidneys (Lupus nephritis), blood vessels (Polyarteritis), joints (Rheumatoid arthritis). IgM mediates it but IgG also plays a role. The reaction takes 3-10 hours for manifestation.
- Type 4 cell-mediated or delayed type hyper¬sensitivity reaction — Tuberculin (Mantoux) reaction is the example. Injecting the antigen PPD or old tuberculin results in the appearance of erythema and induration after 48 hours in hypersensitive individuals.
Immunological Diseases Multiple Choice Questions
Question 1. The mask-like appearance of the face is seen in.
- Facial paralysis
- Trigeminal neuralgia
- Scleroderma
- Myofascial pain syndrome
Answer: 3. Scleroderma
Question 2. Pseudoankylosis of TMJ is common in.
- Osteoarthritis
- Rheumatoid arthritis
- Scleroderma
- Sarcoidosis
Answer: 3. Scleroderma
Question 3. Fish-mouth appearance is the finding in.
- Scleroderma
- Submucous fibrosis
- Plummer-Vinson syndrome
- Angular cheilitis
Answer: 1. Sclero¬derma
Immunological Diseases Viva Voce
Question 1. Name the primary immunodeficiency diseases.
Answer: Defects in cellular immunity lead to DiGeorge syndrome, severe combined immunodeficiency disease, and Wiskott-Aldrich syndrome.
Question 2. Name the secondary immunodeficiency diseases.
Answer:
- Secondary immunodeficiencies are caused by HIV infection, immunosuppressive drug therapy, malignancy or granulomatous disorders of the lymphoid system, and protein-depending disorders.
- It includes Leukemia, Hodgkin’s disease, non-Hodgkin’s lymphoma, multiple myelomas, sarcoidosis, and acquired immunodeficiency syndrome (AIDS).
Question 3. Give examples of connective tissue diseases.
Answer: Also known as, collagen disease, collagen vascular disease, hyperimmune disease, and autoimmune disease. It includes sys¬temic lupus erythematosus, rheumatoid arthritis, scleroderma, dermatomyositis, and polyarthritis nodosa.
Question 4. What is a hypersensitivity reaction?
Answer: The occurrence of intense and disagreeable (Discomfort and sometimes fatal) responses in a pre-sensitized (Immune) host is known as a hypersensitivity reaction.
Question 5. Discuss Wiskott-Aldrich syndrome.
Answer:
- It is an X-linked recessive disorder characterized by eczema, thrombocytopenia, pyogenic and opportunistic infections, and bloody diarrhea. It is due to altered cell surface glycoprotein in lymphocytes and platelets.
- T-cell abnormalities include herpes simplex infection and B-cell abnormalities include recurrent bacterial infection in the oral cavity. Neutropenia and neutrophil dysfunction syndrome cause oral ulcers. Maxillary sinusitis, severe asthma, recurrent otitis media, and pneumonia are also common.
Question 6. What is Raynaud’s phenomenon?
Answer: Paroxysmal vasospasm (reduced blood flow) of the fingers results in a color change of the fingertips in response to cold and emotion.
Question 7. What is DiGeorge syndrome
(or)
What is a velocardiofacial syndrome?
Answer:
- Results from chromosomal deletion 22q11 resulted in defective embryonic growth in the thymus, parathyroid glands, and greater vessels of the heart. Features include variable immunodeficiency, neonatal hypocalcemia, and congenital cardiac defects.
- Abnormal ear, cleft palate, short palpebral fissures, smallmouth, prominent forehead, and micrognathia give a characteristic facial appearance.
Question 8. What is autoimmune disease?
Answer:
- Autoimmunity is the state where the immune system misinterprets the own cells of the body as foreign invaders and sends signals to the immune cells to attack them.
- Diseases that result from such unusual immune response are called as autoimmune diseases, for example lichen planus, lupus erythematosus, and psoriasis
Immunological Diseases Highlights
- The immune response consists of an innate system (first-line defense) and an acquired or adaptive system (specific response to each infectious agent).
- The innate (natural) immune response lacks memory hence the recognition of previous exposure of the pathogens (no antigen-specific recognition) whereas the acquired immune system is dependent on prior contact of the pathogen to respond.
- This memory is advantageous for preventing the same pathogenic agent from causing later infections.
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