| Syndrome | Important Features |
|---|---|
| Parry Romberg's Syndrome |
Facial Hemiatrophy
|
| Vander Woude's Syndrome |
Pits of lower lip and cleft of palate
|
| Ascher's Syndrome |
Acquired double lip + Blepharochalasis + Non toxic thyroid enlargement
|
| Oro Facial Digital Syndrome |
Cleft tongue nd clefting of mandibular alveolar process
|
| Median Cleft Face Syndrome |
Hypertelorism + Median cleft of premaxilla, palate + Cranium bifidum occultum
|
| Meischer's Syndrome | |
| Melkerson-Rosenthal Syndrome |
Cheilitis granulomatosa + Facial Paralysis + Scrotal Tongue
|
| Peutz-Jeghers Syndrome |
Multiple Intestinal Polyposis + Pigmentation on face , oral cavity, and hands
|
| Beckwith's Hypoglycemic Syndrome OR Beckwith-Wiedeman syndrome |
Macroglossia + Neonatal Hypoglycemia + Microcephaly + Fetal Visceromegaly
|
| Rubinstein-Taybi Syndrome | Talon's Cusp + Developmental retardation + Broad thumbs + great toes + Delayed or incomplete descent of testes |
| Klinefelter Syndrome | Taurodontism, XXY genetic constitution |
| Gardner's Syndrome | Multiple polyposis of large intestine + Osteomas of bone + Multiple sebaceous cysts + Impacted supernumerary teeth |
| LADD syndrome |
|
2. Benign and Malignant Tumors
| Syndrome | Important Features |
|---|---|
| Cowden's Syndrome | Oral papillomatous lesions + Facial trichilemmomas associated with GIT, thyroid and CNS abnormalities |
| B-K mole syndrome | Large pigmented and high risk for development of melanoma |
| MEN - I ( Multiple Endocrine Neoplasia Syndrome ) | Hyperplasia of pituitary, parathyroid, adrenal cortex, pancreatic islets |
| MEN - II ( Sipple Syndrome ) | Parathyroid Hypoplasia + Pheochromocytoma + medullary carcinoma of thyroid |
3. Salivary gland tumors and Cysts and Tumors of odontogenic origin
| Syndrome | Features |
|---|---|
| Sjogren's Syndrome | Keratoconjunctivitis Sicca + Xerostomia + Rheumatoid arthritis |
| Gorlin - Goltz Syndrome or Jaw cyst - Cell Nevus - Bifid rib Syndrome |
Multiple odontogenic keratocysts + basal cell carcinoma + bifidd basal rib + Neurologic, ophthalmologic and sexual abnormalities
|
4. Bacterial, Viral, and Mycotic Infections
| Syndromes | Features |
|---|---|
| Heerfordt syndrome | Uveoparotid fever |
| Behcet's syndrome | Behcet's syndrome is characterized by triad of : Recurrent oral ulcers, recurrent genital ulcers, and ocular inflammation |
| Reiter's Syndrome | Tertad of urethritis + Arthritis + Conjunctivitis + Oral Ulcers |
| Ramsay Hunt Syndrome | Herpes Zoster infection on the geniculate ganglion, with involvement of the external ear and oral mucosa |
5. Diseases of Bones and Joints
| Syndrome | Important Features |
|---|---|
| Crouzon Syndrome / Craniofacial Dysostosis | Prognathic mandible, hypoplastic maxilla, high arched palate, parrot beak appearance and hypertelorism |
| Apert Syndrome | Syndactyly involving hands and feet (known as mitten hands and sock feet) with features similar to Crouzon Syndrome. |
| Treacher - Collins Syndrome / Mandibulofacial dysostosis (Franschetti Syndrome) | Hypoplasia of mandible and malar bone, macrostomia, OR malformation of external ear, high arched palate , bird or fish like face and antimongoloid slant with coloboma of lower eyelids |
| Pierre Robin Syndrome | Cleft palate + Micrognathia + Glossoptosis |
| Marfan Syndrome | Long thin extremeties, hyper extensibility of joints, spidery fingers (arachnodactyly), bifid uvula and CVS complications |
| Down Syndrome (Trisomy 21, Mongolism) |
Hypermobility, macroglossia, flat face, larger anterior fontannelle, sexual underdevelopment and cardiac abnormalities
|
| Van Buchem Syndrome | Generalised cortical hyperostosis |
| Gorham Syndrome | Massive osteolysis or vanishing bone |
| Albright syndrome | Precocious puberty + Polyostotic fibrous dysplasia + Cafe-au-lait pigmentation |
| Caffey - Silverman Syndrome | Infantile Hyperostosis, dysphagia, hyperirritability, increased alkaline phosphatase |
| Costen Syndrome | Tinnitus, Otalgia, headache, dizziness, burning tongue, and throat |
| Myofacial Pain Dysfunction Syndrome | Masticatory muscle tenderness + Pain + Limitation of motion + Clicking or popping noise |
6. Diseases of Blood
| Syndromes | Important Features |
|---|---|
| Fanconi Syndrome | Aplastic anemia, Microcephaly, Hypogenitalism and olive brown pigmentation |
| Plummer Vinson Syndrome | Characterized by triad of iron deficiency anemia, carcinoma of hypopharynx (post cricoid carcinoma) and koilonychia (spoon shaped nails) |
| Aldrich Syndrome | Thrombocytopenic purpura, eczema and increased susceptibility to infection |
| Chediak-Higashi Syndrome | Malignant lymphomas, albinism, nystagmus, recurrent infections, photophobia, gingivitis, glossitis |
| Kostmann Syndrome | Severe congenital neutropenia |
7. Disease of Periodontium
| Syndrome | Important Features |
|---|---|
| Papillon - Lefevre Syndrome | Juvenile Periodontitis + Palmar plantar keratosis + Calcification of falx cerebri |
8. Skin Diseases
| Syndromes | Important Features |
|---|---|
| Steven Johnson Syndrome | Severe bullous form of erythema multiformae involving skin, eyes, oral cavity and genitalia |
| CREST syndrome |
|
| Ehler Danlos Syndrome | Hyperextension of joints (Rubberman) , Hypermobility of TMJ |
| Goltz-Gorlin Syndrome (Focal Dermal Hypoplasia) | Multiple papillomas + Atrophy of skin + Polydactyly + Sunken eye + Sparse hair |
| Grinspan Syndrome | Characterized by triad of Lichen planus + Vascular hypertension + Diabetes Mellitus |
9. Diseases of Nerves and Muscles
| Syndromes | Important Features |
|---|---|
| Reader's Syndrome (Paratrigeminal Syndrome) | Severe headache + Signs of ocular sympathetic paralysis |
| Auriculotemporal syndrome (Frey's Syndrome) | Gustatory sweating |
| Horner's Syndrome | Ptosis + Miosis + Anhidrosis + Vasodilation |
| Jaw Winking Syndrome | Ptosis + Rapid elevation of the ptotic occuring on movement of the mandible to the contralateral side |
| Trotter's Syndrome | Tumors of the nasopharynx producing pain similar to trigeminal neuralgia and middle ear deafness |
| Eagles Syndrome | Dysphagia (Pain during mandibular movement and the pain is subsided when the jaws are closed) , sore throat, otalgia and glossodynia. |
| Floppy Infant Syndrome | Generalised weakness due to hypotonia, Inability to sit, stand and walk |
| Mobius Syndrome | Partial / Complete facial paralysis, drooling of saliva, difficulty in mastication and congenital facial diplegia |
| Horton's Syndrome | Vascular Headache |
10. Oral Aspects of Metabolic Disease
| Syndromes | Important Features |
|---|---|
| Cushing's Syndrome | Hyperadrenocorticism (excessive secretion of ACTH hormone) |
| Hurler's Syndrome / Gargoylism | Carbohydrste storage disease, Mucopolysaccharidosis (MPS) |
| Hunter syndrome | MPS-II |
| Waterhouse - Friderichsen Syndrome | Acute adrenal insufficiency in connection with acute septicemia |