Syndromes Short Question And Answers

Oral Medicine Syndromes Important Notes

1. Features Of Various Syndromes

Oral Medicine Syndromes Short Essays

Question 1. Steven Johnson Syndrome.

Answer:

Steven Johnson Syndrome

Steven Johnson Syndrome is a severe form of erythema multiform with wide-spread involvement, typically involving the skin, oral cavity, eyes, and genitalia

Steven Johnson Syndrome Clinical Features:

• Symptoms:
  • Fever, malaise
  • Photophobia
  • Eruptions on the oral mucosa, genital mucosa, and skin
• Skin lesions:
  • Hemorrhagic
  • Vesicle and bullae are present
• Eye lesions:
  • Photophobia
  • Conjunctivitis
  • Corneal ulcerations
  • Keratoconjunctivitis
• Genital lesions:
  • Non-specific urethritis
  • Balanitis
  • Vaginal ulcers
• Complications:
  • Trachea-bronchial ulceration
  • Pneumonia
• Oral manifestations:
  • Oral mucosa may be extremely painful
  • Mastication becomes impossible
  • Mucosal vesicles or bullae occur
  • They rupture and leave a surface covered with thick, white or yellow exudates
  • Erosions of the pharynx are also common
  • Lip may exhibit ulcerations with bloody crusting.

Steven Johnson Syndrome Management:

• ACTH, cortisone, and chlortetracycline are advised

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Question 2. Behcet’s syndrome.

Answer:

Behcet’s Syndrome

• Behcet’s Syndrome is a triad of recurring oral ulcers, recurring genital ulcers and eye lesions

Etiology:

• Immunological
  • It is caused by immune complexes
• Environmental antigen
  • It may be associated with streptococci, viruses, pesticides, and heavy metal

Behcet’s Syndrome Classification:

• Mucocutaneous – oral, genital, and skin lesions
• Arthritic
• Neuro-ocular

Behcet’s Syndrome Clinical Features:

• Age And Sex – Common in males with a mean age of 30 years
• Recurring oral lesions
  • It may be mild/deep large scarring
  • It may appear anywhere on the oral and pharyngeal mucosa
  • They are painful lesions

• • Size – varies from several mm to cm.
  • They have erythematous borders and are covered by exudates
• Recurrent Genital Lesions:
• Site: Scrotum and penis in males
  • Labia in females
• They are small and painful
• Eye lesions:
  • Uvetitis
  • Retinal internal edema
  • Vascular occultation
  • Optic atrophy
  • Conjunctivitis
  • Keratitis
• Skin Lesions: Small pustules/papules occur
• Arthritis:
• Neurologic degeneration:
• Others:
  • Thrombophlebitis
  • Intestinal ulceration
  • Venous thrombosis
  • Renal and pulmonary disease

Behcet’s Syndrome Management:

• Topical and intralesional steroids – Useful for oral mucosal lesion
• In severe cases – a combination of immune-suppressive drugs and systemic corticosteroids are used
• Cyclosporine and colchicines combination – useful in mucocutaneous and GIT manifestations
• Plasmapheresis – This is used in emergencies

Question 3. Gardener’s syndrome.

Answer:

Gardener’s Syndrome

• Gardener’s syndrome is inherited as an autosomal dominant trait

Gardener’s Syndrome Clinical Features:

• Intestinal polyposis
• Multiple osteomas
• Multiple impacted supernumerary teeth
• Connective tissue tumors
• Thyroid carcinomas
• Hypertrophy of pigmented epithelium of the retina
• Dental hypercementosis
• Congenital missing teeth
• Fused molar roots
• Long and tapered molar roots
• Hypodontia
• Compound odontomas
• Multiple carious lesions
• Difficulties in extraction due to ankylosis

Oral Medicine Syndromes Short Answers

Question 1. Trotter’s Syndrome.

Answer:

Trotter’s Syndrome

• The patient complains of neuralgic pain in the mandible, side of the head, tongue, and ear.
• It is associated with middle ear deafness and defective mobility of the soft palate
• Trismus

Trotter’s Syndrome Cause:

• Tumor of the nasopharynx, starting in the pharyngeal wall involving the Eustachian tube.

Question 2. Grinspan syndrome.

Answer:

Grinspan Syndrome

Grinspan Syndrome is a triad of oral lichen planus, diabetes mellitus, and hypertension

Question 3. Ramsay Hunt syndrome.

Answer:

Ramsay Hunt syndrome

• It is a zoster infection of the geniculate ganglion with involvement of external ear and oral mucosa
• Facial paralysis, pain of external auditory meatus, and pinna of the ear.
• Vesicular eruptions in the oral cavity and oropharynx
• Hoarseness of voice
• Tinnitus
• Vertigo

Question 4. Albright’s syndrome.

Answer:

Albright’s Syndrome

• Sex: It is found exclusively in females
• It is a severe form of fibrous dysplasia involving nearly all the bones in the body
• It is accompanied by pigmentations of skin along with endocrine disorders
• Endocrine disorders:
  • Precocious puberty
    • Pubic hair, axillary hair, and breast development occur at the age of 5 years
• Goiter
• Hyperthyroidism
• Hyperparathyroidism
• Cushing’s syndrome
• Acromegaly
• Skin pigmentations:
  • These are coffee with milk color spot
  • There is an irregular flat area of increased skin pigmentation
• Vaginal bleeding occurs
• Long bones are frequently affected
• Prognosis
  • The active period ceases in adult life
  • If growth continues the rate of activity is reduced
  • Calcification of fibrous tissues continues

Question 5. Behcet’s Syndrome

Answer:

Etiology:

• Immunologicalcaused by immune complexes formed by immunocompetent T lymphocytes and plasma cells
• Environmental: associated with bacteria such as streptococci, viruses, pesticides, and heavy metals

Behcet’s Syndrome Clinical Features:

• Age: 10-45 years
• Sex: Common in males

Behcet’s Syndrome Presentation:

• Genital lesions:
  • Consist of recurrent aphthae
  • Larger than mucosal lesions
• Site: in malespenis, inner thigh, scrotum
• In females: vulva, painful in females
  • Healing of these lesions may lead to severe scarring
• Skin lesions:
  • Small pustules or papules appear on the trunk or limbs
• Eye lesions:
  • Initially, ocular lesions develop
  • Consist of conjunctivitis, keratitis, uveitis, optic atrophy, retinal edema
  • This may lead to visual damage and eventually blindness
  • Photophobia may also occur
• Oral manifestation:
  • Recurrent aphthae ulcers develop
  • They vary in size
  • They are deep and painful
  • They are well-demarcated
  • Have an erythematous border
  • Covered by gray or yellow exudates
  • They can occur anywhere in the oral mucosa
  • Other features:
  • Neurological degeneration
  • Intestinal ulceration
  • Arthralgia
  • Visceral involvement

Behcet’s Syndrome Diagnosis:

• Presence of eye lesions
• Recurrent genital lesions
• Skin lesions
• Positive pathergy test

Behcet’s Syndrome Management:

• Administration of topical and intralesional steroids
• Use of immunosuppressor drugs like cyclosporine, azathioprine
• interferon alpha
• Plasmapheresis is used in emergency

Question 6. Eagle’s Syndrome.

Answer:

• Synonym: DISH Syndrome

Eagle’s Syndrome Types:

• Classic Type: Occurs after tonsillectomy
• Carotid Artery Syndrome: Results from calcification of stylohyoid ligament
• Traumatic Eagle’s Syndrome: Develops after fracturing of stylohyoid ligament

 Eagle’s Syndrome Clinical Features:

• Age: seen commonly in adults
• Presentation:
  • The elongated styloid process can be visualized
  • Pain in the lateral pharyngeal area and side of the lower face and neck
  • Difficulty in swallowing
  • Sore throat
  • Glossodynia
  • Headache
  • Dull to severe hemifacial pain
  • Blurred vision
  • Vertigo
  • Pan while opening of mouth wide or while swallowing

Eagle’s Syndrome Radiographic Features:

• Elongation of the styloid process is seen

Eagle’s Syndrome Management:

• Topical Anesthesia
  • Infiltration of anesthetic agent will relieve pain
  • Surgical treatment
  • Surgical resection or segmentation of elongated styloid process
• Corticosteroid Injection
  •  It provides relief

Question 7. Melkerson Rosenthel Syndrome.

Answer:

Melkerson Rosenthel Syndrome

• It is a triad of
• Chelitis granulomatosa
• Facial paralysis
• Scrotal tongue

Question 8. Heerfordt’s syndrome.

Answer:

Heerfordt’s Syndrome

• It is characterized by
  • Firm, painless, bilateral enlargement of the parotid gland
  • Inflammation of the uveal tract of the eye
  • Facial palsy

Question 9. Ascher’s syndrome.

Answer:

Ascher’s Syndrome

• It consists of
  • Double lip
  • Blepharochalasis – It is the drooping of the tissue between the eyebrow and the edge of the upper eyelid
  • Non-toxic thyroid enlargement

Question 10. Sturge Weber syndrome.

Answer:

Sturge Weber Syndrome

• Sturge-Weber Syndrome is a congenital disorder

Sturge Weber Syndrome Classification:

• Complete presymptomatic
• Incomplete symptomatic
• Incomplete monosymptomatic

Sturge Weber Syndrome Etiology:

• Dysplasia of the embryonal vascular system
• Development insult
• Aberrant migration

Sturge Weber Syndrome Clinical Features:

• Port wine stain present at the distribution of trigeminal nerve
• There is angioma
• The patient is present with seizures
• The patient may feel weakness
• Headaches can also occur
• Glaucoma may be present
• There is the presence of vascular hyperplasia of a purple color
• Gingival enlargement can also occur

Sturge Weber Syndrome Management:

• Anticonvulsant – To control seizures
• Anti – glaucoma
• Dentally managed

Question 11.Papillion Lefevre Syndrome.

Answer:

Papillion Lefevre Syndrome

• It is an autosomal recessive disorder
• It is a triad of
  • Hyperkeratosis of palms of the hand and soles of feet
  • Extensive prepubertal destruction of the periodontal bone supporting the dentition, usually extensive generalized horizontal bone loss
  • Calcification of dura

Oral Medicine Syndromes Viva Voce

1. Sturge-Weber syndrome is associated with vascular lesions
2. Hypopigmentation of skin and hair occurs in Che- diak- Higashi syndrome