Syndromes Related To Oral Diseases Pathology Question And Answers

Syndromes Related To Oral Diseases oral Pathology Question And Answers

Question 1. What is a syndrome?
Answer. A syndrome can be defined as a set of symptoms, which collectively indicate or characterize a disease, a psychological disorder or other abnormal conditions.

Question 2. Describe the features of Albright’s syndrome.
Answer.Polyostotic firous dysplasia of bone, café-au-lait spots on the skin, endocrine disturbances like precocious puberty, etc.

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Question 3. Describe the features of Apert’s syndrome.
Answer.Early synostosis of cranial sutures, triangular facial defects, high arched palate, syndactyly and mandibular prognathism. Shovel-shaped incisors, malocclusion, parrotbeak type nose.

Question 4. Describe the features of Ascher’s syndrome.
Answer.Acquired double lip, blepharochalasis and non-toxic thyroid enlargement and recurrent edema of both eyelids.

Question 5. Describe the features of Beckwith’s hypoglycemic syndrome.
Answer. Macroglossia, neonatal hypoglycemia, umbilical hernia and post-natal gigantism.

Question 6. Describe the features of Behcet’s syndrome.
Answer. Oral ulceration, genital ulceration, photophobia, conjunctivitis, uveitis, pyoderma and arthralgia.

Question 7. Describe the features of Caffey-Silverman’s syndrome.
Answer. Development of a tender, deeply placed-soft tissue swelling in bone especially mandible,increased cortical thickening of bone, severe malocclusion fever, pseudoparalysis,dysphagia, anemia and leukocytosis.

Question 8. Describe the features of Chediak-Higashi syndrome.
Answer. It is a progressive systemic disorder with oculo-cutaneous albinism, photophobia,nystagmus, severe gingivitis, oral ulcer, glossitis, pancytopenia and histiocytic inclusions in different organs.

Question 9. Describe the features of Costen’s syndrome.
Answer. Intermittent or continuous impairment of hearing, stuffy sensation in the ear, tinnitus,otalgia, dizziness, headache and glossodynia.

Question 10. Describe the features of Cowden’s syndrome.
Answer. Multiple papules on the lips and gingiva, papillomatosis of the oral mucosa resulting in a cobblestone appearance, follicular keratosis of the perioral, perinasal and periorbital skin, variety of neoplastic changes in many organs.

Question 11. Describe the features of CREST syndrome.
Answer. This phenomenon is a variant of progressive systemic sclerosis and is characterized by calcinosis cutis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly and telangiectasia.

Question 12. Describe the features of Cushing’s syndrome.
Answer. It occurs due to hyperadrenocortism with development of adiposity, mooning of the face,buffalo hump, abnormal hair distribution in the body, vascular hypertension, glycosuria,osteoporosis and hypertrichosis, etc.

Question 13. Describe the features of Crouzon syndrome.
Answer. Craniosynostosis without syndactyly, early synostosis of bone, triangular frontal defect,mandibular prognathism and high-arched palate, nasomaxillary retrusion with mouth breathing.

Question 14. Describe the features of Down’s syndrome.
Answer. Small slanting eyes with epicanthal folds, mongoloid facies, flt face, large anterior fontanel, open sutures, sexual underdevelopment, open mouth with macroglossia,mental retardation and cardiac abnormalities.

Question14a. How the Down’s syndrome can be suspected by looking at the skull?
Answer. Patients with Down’s syndrome always have a brachycephalic skull (branchy means small or short).

Question 14b. Which type of malocclusion is seen in Down’s syndrome?
Answer.Maxillary retrognathism.

Question 15. Describe the features of Eagle’s syndrome.
Answer. Elongation of the styloid process of temporal bone or ossifiaton of styloid ligament causing dysphagia, glossodynia, sore throat and pain along the distribution of the external carotid artery. The pain starts at the tonsillar area during mandibular movements,radiates to the TMJ or base of the tongue and interestingly it subsides as the jaws are closed.

Question 16. Describe the features of Ehlers Danlos syndrome.
Answer. Hyperelasticity of skin, hyperextensibility of joints, increased capillary fragility, easy bruising of the skin and mucosa, delayed wound healing, increased bleeding tendency.

Question 17. Describe the features of Fanconi’s syndrome.
Answer. Aplastic anemia, bone abnormality, microcephaly, hypogenitalism and generalized olive brown pigmentation of skin.

Question 18. Describe the features of flppy infant syndrome.
Answer. Hypotonia, generalized muscle weakness, reduced tendon reflxes; inability to sit, stand or walk due to generalized weakness.

Question 19. Describe the features of focal dermal hypoplasia.
Answer. Focal absence of dermis with herniation of the subcutaneous syndrome. fat into the defect, atrophy and pigmentation of skin, telangiectasias, papillomatosis, syndactyly,microdontia, enamel hypoplasia, cleft lip and palate.

Question 20. Describe the features of Frey’s syndrome.
Answer. It occurs as a result of damage to the auriculotemporal nerve with subsequent reinnervations of sweat glands by parasympathetic salivary fiers. The features include flshing and sweating of the temporal area during taking foods.

Question 21. Describe the features of Gardner’s syndrome.
Answer. Multiple polyposis of large intestine (colon), osteomas of bone, multiple epidermoid or sebaceous cysts of the skin, desmoid tumor, multiple impacted supernumerary and permanent teeth.

Question 22. Describe the features of Gorlin-Goltz syndrome
Answer. Basal cell carcinoma of the skin, multiple odontogenic kerocysts. bifi-ribs, hypertelorism,mental retardation, hypogonadism and multiple basal cell nevi.

Question 23. Describe the features of Goldenhar syndrome.
Answer. Unilateral microstomia, mental retardation, hypoplastic zygomatic arch, downward slanting of palpebral fisures, malformed pinna, high-arched palate, cleft-palate and uvula.

Question 24. Describe the features of Grinspan’s syndrome.
Answer. It occurs in association with lichen planus and consists of lichen planus of skin and mucosa, vascular hypertension and diabetes mellitus.

Question 25. Describe the features of Heerfordt’s syndrome.
Answer. Painless, fim enlargement of the parotid and other glands due to sarcoidosis, uveitis of the eye, fever, vomiting, facial palsy, xerostomia, malaise and GI disturbances.

Question 26. Describe the features of Horner’s syndrome.
Answer. It occurs due to ipsilateral brainstem lesion causing miosis or contraction of pupil, ptosis of the upper eye-lid, anhidrosis and vasodilatation over the facial or periorbital skin causing flshing.

Question 27. Describe the features of Horton’s syndrome.
Answer. Unilateral paroxysm of intense pain in the eye, maxilla, ear mastoid region, base of the nose and beneath the zygoma. Absence of trigger zone, occurrence of pain everyday exactly at the same time.

Question 28. Describe the features of Hurler’s syndrome.
Answer. Inherited mucopolysaccharide metabolism defect characterized by prominent forehead,saddle nose, hypertelorism, macroglossia, puffy eyelids, corneal clouding, short-broad mandible, gingival hyperplasias, multiple cyst-like osteolytic lesions of the jaws, dwarfim deafness.

Question 29. Describe the features of Hunter’s syndrome.
Answer. Mild but similar features like Hurler’s syndrome but without corneal clouding, death usually occurs before age of 15 years.

Question 30. Describe the features of hyoid syndrome.
Answer. Elongation of the greater cornu of hyoid bone with impingement on adjacent laryngeal tissue, pain in the lateral neck and carotid area when the neck is turned to one side, pain during swallowing. Ipsilateral referred pain in the ear, syncope, patient always feels as if a foreign body is lodged in the throat.

Question 31. Describe the features of happy puppet syndrome.
Answer. Jerky puppet-like movements of the body, peculiar open-mouthed appearance, frequent laughter, mental and motor retardation and seizures.

Question 32. Describe the features of Jaffe-Lichtenstein syndrome.
Answer. Multiple bone lesions of firous dysplasia, skin pigmentations.

Question 33. Describe the features of Jugular foramen syndrome.
Answer. Dysphagia, hoarseness of voice, glossopharyngeal neuralgia-like pain, palatal weakness, vocal cord paralysis.

Question 34. Describe the features of Jadassohn-Lewandowsky syndrome.
Answer. Bilateral oral white lesions involving the tongue and buccal mucosa, laminated thickening of the figer and toe nails.

Question 35. Describe the features of jaw-winking syndrome.
Answer. Exaggerated opening of the eye on moving of the mandible in a contralateral direction;ptosis of the affected eye at rest and normal pupilar reflxes.

Question 36. Describe the features of Klinefelter’s syndrome.
Answer. This syndrome occurs in males whose sex chromosome constitution includes one or more extra chromosomes. The patient may develop taurodontism, small testes,azoospermia and infertility.

Question 37. Describe the features of lacrimal-auricular-dental and digital syndrome.
Answer. Lacrimal duct obstruction with overflw of tears, cup-shaped deformed ear with loss of hearing, peg-shaped teeth, enamel hypoplasia and medial or lateral deviation of figers.

Question 38. Describe the features of Marfan’s syndrome.
Answer. Disproportionately long thin extremities, spidery figers, long and narrow face,hyperextensibility of joints, kyphosis or scoliosis and recurrant joint dislocations.

Question 39. Describe the features of Morquio’s syndrome.
Answer. Severe enamel hypoplasia with grey and pitted enamel, severe bone changes, mild corneal clouding, aortic regurgitation and pigeon breast.

Question 40. Describe the features of myofacial pain dysfunction syndrome.
Answer. This condition occurs in relation to the temporomandibular joint and is characterized by the following features—pain, muscle tenderness, clicking sound in TMJ, limitation of jaw movements, but no pain in the joint when palpated through the external auditory meatus.

Question 41. Describe the features of median cleft face syndrome.
Answer. Hereditary defect of abnormal midline development of face and head characterized by hypertelorism, median cleft of the lip, premaxilla and palate, cranium bifium occultum and malocclusion.

Question 42. Describe the features of Melkersson-Rosenthal syndrome.
Answer. Cheilitis granulomatosa, facial paralysis and scrotal tongue, persistant unilateral edema of the orbit and eyelid.

Question 43. Describe the features of multiple endocrine neoplasia syndrome (MEN-I).
Answer. Tumors or hyperplasia of the pituitary, parathyroids, adrenal cortex and pancreatic islets, peptic ulcer or gastric over secretion.

Question 44. Describe the features of Meniere’s syndrome.
Answer. Tinnitus, vertigo, deafness, nausea and vomiting.

Question 45. Describe the features of migraine syndrome.
Answer. Severe periodic headache, irritability, nausea, sleep disturbance and photophobia.

Question 46. Describe the features of Miescher’s syndrome.
Answer. Diffuse swelling of the lip, scaling, fisuring, vesicle or pustule formation on the vermillion border, it is a monosymptomatic form of Melkersson-Rosenthal syndrome.

Question 47. Describe the features of Mikulicz’s syndrome.
Answer. Painless, chronic bilateral hypertrophy of the parotid or lacrimal glands accompanied by enlargement of the lymph nodes, xerostomia.

Question 48. Describe the features of mebius syndrome.
Answer. Congenital facial diplegia, expressionless face due to facial paralysis, inability to close the eyes due to paralysis of the abducens, swollen lips, deafness, epilepsy, etc.

Question 49. Describe the features of mucocutaneous lymph node syndrome.
Answer. Fever, bilateral congestion of ocular conjunctiva, edema of the extremities, dryness and fisuring of the lips, strawberry-like redness and swelling of the tongue, acute nonpurulent swelling of the lymph node.

Question 50. Describe the features of myxoma syndrome.
Answer. Perioral pigmented macules, soft tissue myxomas and endocrinopathies.

Question 51. Describe the features of Maffucci’s syndrome.
Answer. Multiple hemangiomas of the skin and oral mucosa, multiple chondromas of the jaw bone.

Question 52. Describe the features of Nager’s syndrome.
Answer. Hypoplasia of malar bones, antimongoloid obliquity, palpebral fisures, defective hearing, cleft palate and micrognathia.

Question 53. Describe the features of neck-tongue syndrome.
Answer. Unilateral upper nuchal or occipital pain with or without numbness in the area,simultaneous numbness of the tongue on the same side and malocclusion.

Question 54. Describe the features of Noonan’s syndrome.
Answer. Congenital heart disease, chest deformity, mental retardation, short stature and facial bone anomalies.

Question 55. Describe the features of oral-facial-digital syndrome.
Answer. Cleft of the tongue, upper lip, palate and mandibular alveolar process, micrognathia,depressed nasal bridge, frontal bossing, digital malformation, thick firous bands in the lower mucobuccal fold eliminating the sulcus, supernumerary canines and premolars,and small hands and feet.

Question 56. Describe the features of oromandibular-limb hypogenesis syndrome.
Answer. Ocular hypotelorism, cranial nerve palsy, hypodactyly of hands and feet, hypoglossia,cleft palate, conical-shaped mandibular incisors.

Question 57. Describe the features of otopalatodigital syndrome.
Answer.Deafness, cleft palate, generalized bone dysplasia, prominent supraorbital ridge, frontal bossing.

Question 58. Describe the features of orbital syndrome.
Answer. It occurs due to malignant disease of the orbit, supraorbital pain and hypesthesia, blurred vision or diplopia, ptosis and ophthalmoplegia.

Question 59. Describe the features of paraneoplastic syndrome.
Answer.A symptom complex arising in a cancer bearing patient that can’t be explained by local or distant spread of the tumor.

Question 60. Describe the features of Papillon-Lefevre syndrome.
Answer. Hyperkeratosis palmoplantaris, generalized hyperhydrosis, fie body hair, peculiar dirtycolored skin, aggressive periodontitis with severe destruction of alveolar bone,gingival hyperplasia.

Question 61. Describe the features of paratrigeminal syndrome.
Answer. Severe pain or headache in the area of trigeminal distribution with sign of ocular sympathetic paralysis. Onset of pain is sudden, middle-aged males are frequently affected.

Question 62. Describe the features of Parry-Romberg syndrome.
Answer. Atrophy of the skin, subcutaneous tissue, bone and cartilage causing hemifacial deformity, trigeminal neuralgia, loss of hair and vitiligo on the affected side, contralateral jacksonian epilepsy.

Question 63. Describe the features of Plummer-Vinson’s syndrome (Paterson-Kelly syndrome).
Answer. Dysphagia, smooth, red, depapillated painful tongue, lemon tinted pallor of skin, cracks and fisures on the corner of the mouth, atrophy of the fiiform and fungiform papillae of tongue.

Question 64. Describe the features of PFAPA syndrome.
Answer. Periodic fever, aphthous-stomatitis, pharyngitis and adenitis.

Question 65. Describe the features of Pierre-Robin syndrome.
Answer. Cleft palate, micrognathia, glossoptosis and congenital heart disease, absent gag reflx,airway obstruction due to falling back of tongue on the posterior pharyngeal wall due to micrognathia.

Question 66. Describe the features of Peutz-Jeghers syndrome.
Answer. Recurrent abdominal pain due to familial intestinal polyposis, cutaneous pigmentation in perioral region, precocious puberty, GI bleeding and pigmentation of the buccal mucosa.

Question 67. Describe the features of Reiter’s syndrome.
Answer. Urethritis, arthritis, conjunctivitis, keratotic macules and papules on the skin.

Question 68. Describe the features of Ramsay-Hunt syndrome.
Answer. Facial paralysis, pain in the external auditory meatus and pinna of the ear, vasicular eruptions in the oral cavity and oropharynx, horseness of voice, tinnitus and vertigo.

Question 69. Describe the features of Rieger’s syndrome.
Answer. Hypodontia, enamel hypoplasia, protruding lower lip, maxillary hypoplasia, blue sclera,coloboma, malformed anterior teeth and microdontia.

Question 70. Describe the features of Rutherford’s syndrome.
Answer. Congenitally enlarged gingiva, delayed tooth eruption, curtain-like superior corneal opacities and mental retardation.

Question 71. Describe the features of Stevens-Johnson’s syndrome.
Answer. It’s asevere expression oferythemamultiforme characterized bywidespread vesiculation, denudation, sloughing and necrosis involving the skin, oral mucous membrane, eyes
and genitalia.

Question 72. Describe the features of Sturge-Weber syndrome.
Answer. Orofacial and meningeal angiomatosis with secondary mental defiiency, intracranial calcifiations, seizures, hemiplegia and nevus.

Question 73. Describe the features of Sanfiippo’s syndrome.
Answer. Severe CNS defects, mild somatic disturbances, enamel hypoplasia, excessive dentinogenesis with obliteration of pulp chambers and gingival enlargement.

Question 74. Describe the features of Sweat retention syndrome.
Answer. Extravasation of sweat or saliva in the tissue with subsequent inflmmation, keratin plug formation in sweat glands and accessory salivary glands.

Question 75. Describe the features of syndrome of crocodile tears.
Answer. This abnormal condition occurs following facial nerve paralysis, herpes zoster and operative trauma in the cranium. It seems to occur due to straying of the regenerating autonomic nerve fiers; some of those destined for salivary glands go to the lacrimal gland instead, resulting in a salivary-lacrimal reflex. It is characterized by spontaneous lacrimation occurring parallel with normal salivation during meals.

Question 76. Describe the features of Treacher Collins syndrome.
Answer. Anti-mongoloid palpebral fisures with coloboma. Hypoplasia of the facial, especially malar and mandibular bones, microstomia and oral fitulas, etc.

Question 77. Describe the features of Turner’s syndrome.
Answer. Short stature, cubitus valgus, webbed neck, sexual infantalism, renal disorders,micrognathia and premature eruption of teeth.

Question 78. Describe the features of trichodento-osseous syndrome.
Answer. Tightly curled and kinky hair, osteosclerosis, hypoplastic-hypocalcemia enamel, manyunerupted teeth and taurodontism, diastema and microdontia.

Question 79. Describe the features of Von-Recklinghausen’s syndrome.
Answer. Multiple neurofibromatosis in the body including oral cavity, pigmentation, and hirsutism,risk of malignant transformation of one or more lesions.

Question 80. Describe the features of Waterhouse-Friderichsen syndrome.
Answer. This syndrome occurs due to acute adrenal cortical insufficiency and is characterized by acute meningitis, septicemia, pronounced purpura, bilateral adrenal hemorrhage fever,coma etc, death usually occurs within 48-72 hours.

Question 81. Describe the features of whistling face syndrome.
Answer.Sunken eyes, true ocular hypertelorism, antimongoloid obliquity of palpebral fisures, small nose, micrognathia, protruding lips as seen during whistling and high-arched palate.