Essentials of Oral Pathology (3rd Edition) PDF

Essentials of Oral Pathology  Essentials of Oral Pathology THIRD EDITION Swapan Kumar Purkait BDS (Cal) MDS (Cal) Professor and Head Department of Oral and Maxillofacial Pathology Purvanchal Ins...

Essentials of Oral Pathology  Essentials of Oral Pathology THIRD EDITION Swapan Kumar Purkait BDS (Cal) MDS (Cal) Professor and Head Department of Oral and Maxillofacial Pathology Purvanchal Institute of Dental Sciences Gorakhpur, Uttar Pradesh, India Forewords RR Paul Jay Gopal Ray Tamal Kanti Pal ® JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD New Delhi St Louis Panama City London Published by Jaypee Brothers Medical Publishers (P) Ltd Corporate Office 4838/24, Ansari Road, Daryaganj, New Delhi 110 002, India Phone: +91-11-43574357, Fax: +91-11-43574314 Offices in India Ahmedabad, e-mail: [email protected] Bengaluru, e-mail: [email protected] Chennai, e-mail: [email protected] Delhi, e-mail: [email protected] Hyderabad, e-mail: [email protected] Kochi, e-mail: [email protected] Kolkata, e-mail: [email protected] Lucknow, e-mail: [email protected] Mumbai, e-mail: [email protected] Nagpur, e-mail: [email protected] Overseas Offices North America Office, USA, Ph: 001-636-6279734 e-mail: [email protected], [email protected] Central America Office, Panama City, Panama, Ph: 001-507-317-0160 e-mail: [email protected], Website: www.jphmedical.com Europe Office, UK, Ph: +44 (0) 2031708910 e-mail: [email protected] Essentials of Oral Pathology © 2011, Jaypee Brothers Medical Publishers All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the author and the publisher. This book has been published in good faith that the material provided by author is original. Every effort is made to ensure accuracy of material, but the publisher, printer and author will not be held responsible for any inadvertent error(s). In case of any dispute, all legal matters are to be settled under Delhi jurisdiction only. First Edition: 1999 Second Edition: 2003 Reprint: 2004, 2007, 2008 Third Edition: 2011 ISBN 978-93-5025-214-7 Typeset at JPBMP typesetting unit Printed at Replika  To My children Prithu and Pubali and my wife Maitreyee Foreword................................... Today our society needs not just a dental surgeon, but a medically competent dental surgeon. Oral pathology is undoubtedly the most integral subject of teaching in dental curriculum which bridges the gap between the medical and dental sciences. But the available textbooks in this field of oral pathology are so voluminous that it becomes virtually impossible for any dental student to gather comprehensive knowledge out of those textbooks during their BDS course of studies. In view of this, the textbook titled Essentials of Oral Pathology written by Dr Swapan Kumar Purkait seems to fill this lacuna admirably. I am sure this book will be of immense importance to the general dental practitioners too. RR Paul MDS Head Department of Oral Pathology Guru Nanak Institute of Dental Sciences Kolkata, West Bengal, India Foreword................................... It gives me immense pleasure to introduce the third edition of the book on oral pathology titled Essentials of Oral Pathology by Dr Swapan Kumar Purkait to the budding graduates of dentistry. Oral and maxillofacial pathology is a specialty of dentistry and a very fundamental subject for all the students contemplating to practice clinical dentistry at large. The purpose of the latest edition of this book remains the same, i.e. to provide the reader with a comprehensive discussion of a wide variety of diseases that affects the maxillofacial region. The previous editions have been well accepted and so far thousands of students were benefitted by reading the same. The immense effort put in by the author in bringing out this edition is highly commendable. We all trust in the basic philosophy that education brings in knowledge with consistent effort and knowledge when properly nurtured brings in wisdom. Similarly, this book has also grown qualitatively and refined with every new edition. This book has twenty-three chapters, illustrations and photographs arranged systematically to fit in with the course and curriculum for undergraduate teaching; though much emphasis is not given on in-depth detail of molecular and biochemical aspects of modern diagnostic pathology, since this book has been written keeping in mind the need of undergraduate students only. Finally, I wish Dr Purkait all success in life and my deepest regards to him for sharing his knowledge with the students. I conclude by saying “Man is not immortal but remains immortal through his work”. I am sure all of us will be benefitted by this book. Jay Gopal Ray MDS (Oral Pathology) Postgraduate Teacher and Head Department of Oral Pathology Dr R Ahmed Dental College and Hospital Kolkata, West Bengal, India Foreword to the Second Edition There is an ongoing search for an improved approach to deliver oral healthcare to ail human across the globe. The gap between the reality and expectations in oral healthcare system has become wider and this disparity, however, lies with the failure of transforming fundamental biological science into own clinical practice. Oral pathology is one of the basic sciences in dentistry, the thorough understanding of which can certainly bring about qualitative changes in our clinical approaches towards amelioration of disease entity, providing both early intervention with better therapeutics and preventions. With this mission in mind, Dr Swapan Kumar Purkait, an eminent oral pathologist, has strived for new second edition of his book Essentials of Oral Pathology. It is with great pleasure and profound satisfaction, I have gone through the entire edition and found it to be very informative and useful for persuing undergraduate studies. This is a wonderful piece of work with simple language and lucid presentation also, easy for students to assimilate and reproduce. About 150 pages have been added to this new edition to incorporate current contents and references and other chapters are thoroughly updated throughout. New chapters like Syndromes Related to Oral Diseases, Classification of Oral Diseases, and Diseases of TM Joint, will certainly enrich the readers. The breakneck increase in information and indecent speed of expanding knowledge have paved the way for the second edition to come out. I am sure that this book will find its own place in dental profession and the readers shall have ready access to a comprehensive reference that enables rapid retrieval of integrated and relevant information on the subject. Tamal Kanti Pal MDS, PhD, Cert Implant, New York University, USA Head and Postgraduate Faculty Department of Periodontics Guru Nanak Institute of Dental Sciences Kolkata, West Bengal, India Preface to the Third Edition.. The second edition of Essentials of Oral Pathology was published seven years back in the year 2003. I am grateful to the students and teachers of oral pathology for their acceptance and appreciation of the book. The purpose of the third edition is primarily to make qualititative upliftment of the book and to provide the students with more elaborate discussions on oral and orofacial diseases. Moreover, some new topics have also been added in this version. The highlighting features of this edition are the introduction of some special aspects of various oral lesions and the addition of several tables to pinpoint the key features of many important diseases. This edition is also coming in multicolor version, which I hope will be beneficial to the students in understanding the subject better. I sincerely request the teachers and students of oral pathology to express their views and kindly provide me with constructive suggestions as to how this book can be further improved. Swapan Kumar Purkait Preface to the First Edition.... Oral Pathology is an important branch of dentistry and although, there are few good textbooks available in the subject, Essentials of Oral Pathology has been written with a view to present the subject to the students in a more simplified but comprehensive manner. I hope the book will fulfill the need of the students by giving them relevant guidance in their day-to-day learning process as well as during their preparation for examination and above all, with the book in hand, students will find the subject easy to handle. As no one is perfect in absolute sense, I also humbly accept my limitations regarding shortcomings in the book and therefore, I sincerely welcome the valuable suggestions from my senior colleagues and students regarding what further should be done to improve this book. Swapan Kumar Purkait Acknowledgments................... This book has been accomplished with the help of many people from all over the country and I am indebted to all of them. I wish to thank and express my sincere regards to my teachers Dr RR Pal, Dr Amit Roy, Dr (Mrs) Kabita Chatterjee, and Dr Tamal Kanti Pal for their constant encouragement and inspirations. Special thanks to Dr Jay Gopal Ray for his continuous encouragement and whole hearted support. Heartiest thanks are extended to Dr Alok Banerjee, Dr (Mrs) Mousumi Pal, Dr KP Das and Dr Madhumita Bhattacharjee for providing me with some brilliant clinical photographs for this book. Deepest thanks to my wife Maitreyee , my daughter Pubali and Mr Suman Guha who have done a great job in making the computer generated graphic illustrations and designing of the tables used in the book. I am thankful to my student Dr Sumanta Kumar Kolay (now an oral pathologist too) for his continuous support and encouragement extended as always during the course of writing the book. Heartiest thanks to my seniors, friends and colleagues Dr Tushar Deb, Dr Bijoy Das, Dr Sanjib Mitra, Dr Anjana Majumder, Dr S R Karmakar, Dr Arup Ghosh, Dr Neeta Singh, Dr Shila Dey, Dr Narendra Singh, Dr Jayanta Chatterjee, Dr Sumit Majumder, Dr A Mamud and Dr Sourav Bhattacharjee for their whole hearted support and encouragement. I am fortunate to have the constant encouragement and trust of Shri Jitendar P Vij, the Chairman and Managing Director of Jaypee Brothers Medical Publishers (P) Ltd along with his editorial and production staff. Last but not least, I sincerely thank the students and teachers of Oral and Maxillofacial Pathology, who have made the 1st and 2nd edition of my book successful and this 3rd edition would not have been possible without their acceptance and appreciation. If someone’s name is inadvertently not included in this column of acknowledgment, I sincerely apologize for that. Contents.................................... 1. Developmental Anomalies of Oral and Paraoral Structures.......................... 1–63 Introduction 1 Developmental anomalies of oral soft tissues 1 Anomalies of lips and palate 1 Lip pits and fistulas 1 Double lip 2 Frenal tag 2 Hereditary intestinal polyposis (Peutz-Jeghers) syndrome 3 Oral melanotic macule (ephelis) 4 Uvula elongata 4 Cheilitis glandularis 5 Cheilitis granulomatosa 6 Anomalies of oral mucosa 7 Fordyce’s granules 7 Focal epithelial hyperplasia 8 White sponge nevus 9 Developmental defects of the gingiva 10 Fibromatosis gingivae 10 Retrocuspid papilla 11 Developmental anomalies involving the jawbone 11 Agnathia 11 Micrognathia 11 Macrognathia 13 Facial hemihypertrophy 13 Facial hemiatrophy 15 Cleft lip and cleft palate 16 Developmental anomalies of the tongue 20 Aglossia 20 Microglossia 20 Macroglossia 20 Ankyloglossia 22 Cleft tongue 22 Fissured tongue (scrotal tongue) 23 Median rhomboid glossitis 23 Lingual varices 25 Geographic tongue 25 Hairy tongue 27 Lingual thyroid nodule 28 Thyroglossal tract cyst 29 Anomalies of oral lymphoid tissue 30 Reactive lymphoid aggregate 30 Lymphoepithelial cyst (branchial cyst) 30 Angiolymphoid hyperplasia with eosinophilia (ALHE) 31 Anomalies of the salivary gland 32 Developmental anomalies involving oral hard tissues 32 Abnormalities of teeth 32 Disturbance in size of teeth 32 Microdontia 32 Macrodontia 33 Disturbance in number of teeth 34 Anodontia 34 Complete or total anodontia 34 Partial anodontia 35 Supernumerary teeth 36 Disturbances in eruption of teeth 38 Premature eruption 38 Delayed eruption 38 Impacted teeth 39 Eruption sequestrum 41 Disturbances in the shape of teeth 41 Gemination (twinning) 41 Fusion 42 Concrescence 43 Dilaceration 44 Taurodontism 45 Dens-in-dente (dens-invaginatus) 45 Dens- evaginatus 46 Talon cusp 47 Enamel pearl 47 Disturbance in the structure of teeth 48 Disturbance in the structure of enamel 48 Acquired disturbances of enamel 48 Focal enamel hypoplasia 48 Idiopathic enamel opacities 49 Generalized enamel hypoplasia 49 Effect of individual systemic conditions on enamel hypoplasia 49 Nutritional deficiency 49 Congenital syphilis 49 Hypocalcemia 50 Exanthematous disease 50 Birth injuries and Lowbirth weight 50 Fluorides and mottling 51 Hereditary disturbance of enamel formation 51 Amelogenesis imperfecta 51 Syndrome associated enamel defects 53 Disturbances in structure of dentin 54 Dentinogenesis imperfecta (hereditary opalescent- dentin) 54 Dentinal abnormality due to systemic or environmental disturbances 57 Dentin dysplasia 58 Regional odontodysplasia (ghost teeth) 60 Disturbance in structure of cementum 61 Hypercementosis 61 Hypocementosis 62. 2. Benign and Malignant Neoplasms of the Oral Cavity................................. 64–174 Neoplasm (tumor) 64 Classification of oral non-odontogenic neoplasms 65 Neoplasms of epithelial tissue origin 65 Neoplasms of mesenchymal tissue origin 65 Benign neoplasms of the epithelial tissue origin 66 Papilloma 66 Keratoacanthoma 68 Pigmented cellular nevus 70 Intradermal (intramucosal) nevus 70 Junctional nevus 71 Compound nevus 71 Blue nevus 72 Malignant neoplasms of the epithelial tissue origin 72 Squamous cell carcinoma 72 Oral cancer in different intraoral locations or subsites 80 Carcinoma of the lip 80 Carcinoma of the tongue 81 Carcinoma of the floor of the mouth 83 Carcinoma of the palate 83 Carcinoma of the buccal mucosa 84 Carcinoma of the gingiva/alveolar ridge 84 Carcinoma of the maxillary antrum 85 Basal cell carcinoma (rodent ulcer) 94 Verrucous carcinoma 96 Malignant melanoma 98 Spindle cell carcinoma 101 Primary intra-alveolar carcinoma 102 Neoplasms of mesenchymal tissue origin 103 Benign neoplasms of fibrous connective tissue 103 Fibroma 103 Desmoplastic fibroma 104 Giant-cell fibroma 105 Myofibroma 106 Peripheral ossifying fibroma 107 Central ossifying fibroma 108 Peripheral giant cell granuloma 110 Central giant cell granuloma 113 Benign fibrous histiocytoma 116 Myxoma 117 Nodular fascitis 118 Benign neoplasm of adipose tissue origin 119 Lipoma 119 Benign neoplasm of vascular tissue origin 120 Hemangioma 120 Benign neoplasm of lymphatic vessels 124 Lymphangioma 124 Benign neoplasm of bone 127 Osteoma 127 Osteoid osteoma 129 Osteoblastoma 129 Benign neoplasm of cartiliage tissue 130 Chondroma 130 Benign chondroblastoma 131 Benign neoplasm of smooth muscles 131 Leiomyoma 131 Benign neoplasm ofstriated muscle 133 Rhabdomyoma 133 Granular cell myoblastoma 133 Benign neoplasms of neural tissue 135 Neurilemmoma (schwannoma) 135 Neurofibroma 137 Melanotic neuroectodermal tumor of infancy 139 Malignant neoplasms of mesenchymal xx Essentials of Oral Pathology tissue 140 Fibrosarcoma 140 Malignant fibrous histiocytoma 143 Liposarcoma 144 Hemangioendothelioma 144 Hemangiopericytoma 145 Kaposi’s sarcoma 147 Ewing’s sarcoma 148 Chondrosarcoma 150 Mesenchymal chondrosarcoma 152 Osteosarcoma 153 Lymphomas 157 Non- Hodgkin’s lymphoma (NHL) 157 Burkitt’s lymphoma 161 Hodgkin’s lymphoma 164 Multiple myeloma 165 Solitary plasmacytoma 167 Leiomyosarcoma 168 Rhabdomyosarcoma 169 Neurogenic sarcoma 169 Metastatic tumors of the jaws 171. 3. Oral Precancerous Lesions and Conditions.................................................. 175–197 Leukoplakia 175 Oral hairy leukoplakia 182 Leukoedema 183 Carcinoma in situ 184 Erythroplakia 185 Stomatitis nicotina 186 Oral submucous fibrosis (OSF) 187 Sideropenic dysphagia 191 Lichen planus 191. 4. Diseases of the Salivary Glands..................................................................... 198– 234 Classification of salivary gland diseases 198 Non-neoplastic disorders 198 Neoplastic disorders 199 Developmental anomalies of the salivary gland 199 Aplasia or agenesis of the salivary gland 199 Hypoplasia of the salivary glands 199 Ectopic salivary glands (aberrant) 200 Atresia 200 Accessory ducts 200 Diverticuli 200 Lingual mandibular salivary glands depression 201 Reactive lesions of the salivary gland 201 Salivary gland cysts 201 Sialolithiasis 201 Postradiation sialadenitis 205 Chronic sclerosing sialadenitis 205 Necrotizing sialometaplasia 206 Infective lesions (sialadenitis) 207 Bacterial sialadenitis 207 Acute bacterial sialadenitis 207 Chronic bacterial sialadenitis 208 Recurrent parotitis 209 Viral sialadenitis 209 Mumps (endemic parotitis) 209 Cytomegalic inclusion disease 209 Immune-mediated disease 209 Mikulicz’s disease 209 Sjogren’s syndrome 210 Miscellaneous disorders of salivary gland 213 Heerfordt’s syndrome 213 Sialosis 213 Ptyalism 213 Aptyalism (xerostomia) 214 Neoplasm of the salivary glands 215 Pleomorphic adenoma 216 Monomorphic adenoma 220 Myoepithelioma 221 Oncocytoma (oxyphilic adenoma) 222 Adenolymphoma (warthin’s tumor) 222 Malignant salivary gland neoplasms 224 Malignant pleomorphic adenoma (mixed tumor) 224 Adenoid cystic carcinoma (cylindroma) 225 Mucoepidermoid tumor 229 Acinic cell tumor 231 Adenocarcinoma 231. 5. Odontogenic Neoplasms.................................................................................... 235–267 Classification of odontogenic tumors (modified WHO classification) 236 Ameloblastoma 236 Unicystic ameloblastoma 242 Adenomatoid odontogenic tumor (AOT) 244 Calcifying epithelial odontogenic tumor (CEOT) 247 Squamous odontogenic tumor 250 Ameloblastic fibroma 251 Ameloblastic fibro- odontome 253 Odontomes 254 Odontogenic fibroma 257 Peripheral odontogenic fibroma 257 Central odontogenic fibroma 258 Odontogenic myxoma 259 Periapical cementral dysplasia (cementoma) 261 Familial gigantiform cementoma 262 Cementoblastoma 262 Malignant odontogenic neoplasms 264 Malignant ameloblastoma 264 Ameloblastic carcinoma 264 Odontogenic carcinoma 265 Odontogenic sarcomas 265 Clear cell odontogenic carcinoma 265 Primary intra-alveolar carcinoma 265. 6. Cysts of the Oral Regions.................................................................................. 268–305 Classification of cysts 268 Cysts associated with the maxillary antrum 269 Cyst of the tissue of the mouth, face and neck 269 Odontogenic cysts 270 Odontogenic keratocyst (primordial cyst) 270 Dentigerous cyst 276 Radicular cyst 281 Eruption cyst 286 Lateral periodontal cyst 287 Dental lamina cyst (gingival cyst) of the newborn 288 Gingival cysts of the adult 288 Sialo-odontogenic cysts (glandular odontogenic cyst) 289 Botryoid odontogenic cysts 290 Calcifying epithelial 290 Odontogenic cyst (CEOC) 290 Paradental cyst 292 Non-odontogenic cysts 293 Globulomaxillary cyst 293 Nasolabial cyst (kelstadt’s cyst) 294 Nasopalatine duct cyst (incisive canal cyst) 295 Solitary bone cyst (traumatic/hemorrhagic bone cyst) 297 Aneurysmal bone cyst 298 Cyst of the salivary gland 300 Ranula 302 Dermoid cyst 303 Surgical ciliated cyst of maxilla 303. 7. Regressive Alterations of Teeth...................................................................... 306–319 Attrition of teeth 306 Abrasion of teeth 307 Tooth abfraction 309 Erosion of teeth 309 Role of salivary function in the prevention of dental erosion 311 Resorption of teeth 311 External resorption of tooth 312 Internal resorption of tooth 314 Pulp calcification 315 Hypercementosis 316 Age changes in teeth 317 Cementicles 318 Dentinal sclerosis 318.  Contents xxi 8. Bacterial, Viral and Fungal Infections........................................................... 320–367 Specific bacterial infections 320 Tuberculosis 320 Syphilis 324 Gonorrhea 328 Actinomycosis 329 Scarlet fever 331 Diphtheria 332 Sarcoidosis 332 Leprosy 333 Tetanus 334 Midline lethal granuloma 335 Wegener’s granulomatosis 335 Noma (cancrum oris) 336 Pyogenic granuloma 337 Viral infections 339 Acquired immunodeficiency syndrome (AIDS) 339 Herpes virus infections 344 Herpes simplex virus type–I infections 345 Herpes simplex virus type-II infections 347 Varicella- zostervirus 348 Infections 348 Cytomegalovirus infection 350 Epstein-barr virus infections 351 Human papillomavirus infection 352 Paramyxovirus infection 352 Measles (rubeola) 352 Mumps 353 Coxsackie virus infections 353 Herpangina 353 Hand, foot and mouth disease 354 Aphthous ulcers 354 Behçet’s syndrome 357 Reiter’s syndrome 357 Rabies 357 Fungal infection 358 Candidiasis 358 Deep fungal infections 362 Coccidioidomycosis 362 Histoplasmosis 362 Cryptococcosis 363 North american blastomycosis 364 Mucormycosis 364. 9. Dental Caries........................................................................................................ 368–388 Dental caries 368 Acidogenic theory 369 Proteolytic theory 374 Proteolytic chelation theory 375 Sucrose chelation theory 375 Autoimmune theory 375 Contributing factors in dental caries 375 Clinical aspects of dental caries 378 Histopathological aspect of dental caries 382 Histology of dentinal caries (caries in dentin) 383 Protective responses of dentin and pulp against caries 384 Caries activity tests 385 Methods of caries prevention 386 Caries vaccine 386. 10. Diseases of Dentin-Pulp Complex and Periapical Tissues...................... 389–413 Pulpal diseases 389 Focal reversible pulpitis 391 Acute pulpitis 392 Chronic pulpitis 393 Aerodontalgia 395 Pulp necrosis 395 Diseases of the periapical tissues 398 Primary acute apical periodontitis 398 Periapical granuloma (Chronic apical periodontitis) 398 Acute exacerbation of chronic periapical granuloma (phoenix abscess) 400 Periapical abscess (dentoalveolar abscess) 400 Osteomyelitis 401 Acute suppurative osteomyelitis 403 Chronic suppurative osteomyelitis 406 Chronic focal sclerosing osteomyelitis (Condensing osteitis) 408 Diffuse sclerosing osteomyelitis 409 Chronic osteomyelitis with proliferative periostitis (Garre’s osteomyelitis) 410 Giant cell periostitis with hyaline change (Pulse granuloma) 411 Endodontic-periodontic lesions 412. 11. Spread of the Oral Infection............................................................................. 414–425 Space infections 414 Space infections related to maxilla 414 Space infections related to mandible 416 Cellulitis 419 Ludwig’s angina 420 Cavernous sinus thrombosis (thrombophlebitis) 421 Maxillary sinusitis 422 Focal infection 423. 12. Physical and Chemical Injuries of the Oral Cavity.................................... 426–444 Physical injuries 426 Fractures of teeth 426 Root fracture 426 Cemental tear 426 Bruxism 426 Ankylosis of teeth 428 Submerged teeth 428 Toothbrush injury 429 Toothpick injury 430 Traumatic atrophic glossitis 430 Chronic ulcers of the tongue 430 Traumatic ulcer 431 Factitious injuries (self-inflicted oral wounds) 432 Denture related injuries or lesions 433 Electrical burns in the mouth 434 Thermal burns in mouth 435 Radiation injuries 435 Various damaging effects of radiation on individual organs or tissues 436 Osteoradionecrosis 438 Chemical injuries 440 Congenital porphyria 440 Biliary atresia 440 Erythroblastosis fetalis 440 Fluorosis 441 Oral manifestations of various metal poisoning 441 Oral manifestations of cytotoxic drug therapy 442 Oral manifestations of tetracycline staining 442 Angioneurotic edema 442 Chemical burns 443 Chemical burns due to other medicaments 443. 13. Biopsy and Healing of Oral Wounds.............................................................. 445–455 Biopsy 445 Exfoliative cytology 447 Healing of oral wounds 448 Healing of biopsy wound 449 Healing of gingivectomy wound 450 Healing of the extraction wound 450 Dry socket (alveolar osteitis) 451 Healing of the fractured jawbone 451 Replantation of tooth 452 Transplantation of teeth 453 Healing around osteointegrated implants 454. xxii Essentials of Oral Pathology 14. Oral Aspects of Metabolic Disorders............................................................. 456–478 Disturbances in mineral metabolism 456 Calcium 456 Phosphorus 457 Iron 458 Magnesium 458 Zinc 458 Disturbance in vitamin metabolism 458 Vitamin D 458 Osteoporosis 459 Rickets 459 Vitamin A 460 Vitamin B complex 460 Vitamin C (ascorbic acid) 461 Vitamin K 462 Disturbances in protein metabolism 462 Amyloidosis 462 Porphyria 463 Disturbances in carbohydrate metabolism 463 Hurler’s syndrome 463 Disturbance in lipid metabolism 464 Hand- schuller-christian disease 464 Eosinophilic granuloma 465 Letterer-siwe disease 465 Gaucher’s disease 466 Niemann-pick disease 466 Disturbance in hormone metabolism 466 Hypopituitarism 466 Pituitary insufficiency in adults 467 Diabetes insipidus 467 Hyperpituitarism 467 Hypothyroidism 469 Hyperthyroidism 469 Hyperparathyroidism 470 Hypoparathyroidism 472 Adrenal hormones 473 Mineralocorticoids 474 Chronic adrenocortical insufficiency (addison’s disease) 474 Hyperfunction of adrenocortical hormone (cushing’s syndrome) 474 Pancreatic hormone (insulin) 475 Progeria 476 Imbalance of sex hormones 476. 15. Diseases of Bone.................................................................................................. 479–501 Paget’s disease of bone (osteitis deformans) 479 Fibrous dysplasia of bone 482 Cherubism 486 Osteogenesis imperfecta 489 Cleidocranial dysplasia 491 Osteopetrosis (marble bone disease) 492 Pierre robin syndrome 494 Gardner syndrome 494 Marfan’s syndrome 494 Down syndrome (trisomy 21) 495 Infantile cortical hyperostosis (caffey’s disease) 496 Mandibulofacial dysostosis (treacher-collins syndrome) 497 Achondroplasia 497 Massive osteolysis (vanishing bone disease) 498. 16. Diseases of Temporomandibular Joint.......................................................... 502–508 Developmental disorders 502 Hypoplasia of the mandibular condyle 502 Hyperplasia of the mandibular condyle 502 Traumatic disorders 502 Luxation and subluxation 502 Ankylosis of temporomandibular joint 503 Inflammatory disorders 505 Ankylosing spondylitis 505 Osteoarthritis 505 Rheumatoid arthritis 506 Acute traumatic arthritis 506 Myofacial pain dysfunction (MPD) syndrome 507 Neoplasia of temporomandibular joint 507. 17. Oral Aspects of Hematological Disorders..................................................... 509–527 Pernicious anemia 509 Iron deficiency anemia 510 Aplastic anemia 511 Hemolytic anemia 512 Thalassemias 513 Sickle cell anemia 515 Erythroblastosis fetalis 515 Polycythemia vera 516 Leukemias 517 Agranulocytosis (granulocytopenia) 521 Cyclic neutropenia 522 Purpura 522 Hemophilia 525. 18. Periodontal Disease............................................................................................. 528–542 Gingival hyperplasia 535 Desquamative gingivitis 537 Acute necrotizing ulcerative gingivitis (ANUG) 538 Lateral periodontal abscess 539 Pericoronitis 540 Staining of teeth 541. 19. Oral Aspects of Dermatological Disorders................................................... 543–568 Hereditary ectodermal dysplasia 543 Psoriasis 544 Pityriasis rosea 545 Incontinentia pigmenti 545 Erythema multiforme 546 Dermatitis herpetiformis 548 Keratosis follicularis 549 Acanthosis nigricans 549 Dyskeratosis congenita 550 White sponge nevus 550 Polymyositis 551 Pemphigus 552 Pemphigoid 555 epidermolysis bullosa 558 Lupus erythematosus 559 Discoid lupus erythematosus 561 Scleroderma 563 Ehlers-Danlos syndrome 566. 20. Diseases of the Nerves and Muscles.............................................................. 569–576 Diseases of the nerves 569 Trigeminal neuralgia 569 Sphenopalatine neuralgia 571 Glossodynia and glossopyrosis 571 Auriculotemporal syndrome (Frey’s syndrome) 572 Glossopharyngeal neuralgia 572 Bell’s palsy (facial nerve paralysis) 572 Causalgia 574 Eagle’s syndrome 574 Disease of the muscles 574 Generalized familial muscular dystrophy 574 Myasthenia gravis 575 Myositis ossificans 575. 21. Oral Manifestations of Generalized Diseases............................................. 577–586 Vitamin deficiencies 577 Important causes of lymphadenopathy 578 Blood dyscrasias 578 Metabolic disorders 579 Heavy metal poisoning 580 Endocrine disturbances 580  Contents xxiii Granulomatous diseases 581 Dermatological diseases 582 Bone diseases 582 Acute infective diseases 583 Helminthic diseases 583 Renal diseases 583 Neural diseases 584 Sexually transmitted diseases 584 Cardiovascular diseases 584 Genetic disorders 585 Allergic conditions 585 General manifestations of oral diseases 585. 22. Syndromes Related to Oral Diseases............................................................. 587–597 23. Important Classifications of Oral Diseases.................................................. 598–612 White lesions of the oral cavity 598 Red-blue lesions of the oral cavity 599 Pigmented lesions of the oral cavity 599 Classification of vesiculobullous diseases 600 Classification of ulcerative conditions 600 Classification of discoloration of tooth 601 Classification of cysts of the oral region 601 Classification of odontogenic neoplasms 602 Classification of giant cell lesions 602 Classification of verrucal–papillary lesions of oral cavity 602 Classification of diseases of salivary glands 602 Classification of fibro-osseous lesions 603 Classification of vascular tissue diseases 603 Classification of diseases of the hemopoietic tissues and lymphoreticular system 604 Classification of stomatitis 604 Classification of severe infections of the orofacial tissues 604 Classification of chronic orofacial pain 605 Classification of diseases of tongue 605 Classification of gingival enlargements 606 Classification of skin diseases 607 Classification of taste disorders 608 Classification of oral swellings 608 Classification of neck swellings 609 Classification of oral soft tissue 609 Classification of yellow conditions of oral mucosa 609 Anatomic radiolucencies of jawbones 610 Radiolucent lesions of the periapical region 610 Classification of pericoronal radiolucent lesions 610 classification of inter-radicular radiolucent lesions 610 Classification of multilocular radiolucent lesions of the jaws 611 Mixed radiolucent-radiopaque lesions associated with teeth 611 Mixed radiolucent-radiopaque lesions not necessarily associated with teeth 611 Multiple separate radiopaque lesions of the jaws 611 Generalized radiopacities of the jaws 612 Classification of causes of trismus 612. Index........................................................................................................................................613 INTRODUCTION Besides this, abnormalities in histodifferen- tiation may cause defective formation of dental Malformations or defects resulting from dis- hard tissues, resulting in the disturbance of tooth turbance of growth and development are known structure. as developmental anomalies. A large number of Disturbance in histodifferentiation often such developmental anomalies, which involve the occurs at a later stage of tooth development as body in general and oral structures in particular compared to the disturbance of morphodifferen- can occur during the embryonic life. tiation. Manifestations of such defects are evident either at birth or sometimes after birth. These anomalies often have some serious implications on the DEVELOPMENTAL ANOMALIES further growth and development of the involved OF ORAL SOFT TISSUES organ during the later stages of life. Moreover, developmental anomalies affecting ANOMALIES OF LIPS AND PALATE the teeth are seen more often then any other defects LIP PITS AND FISTULAS in the oral cavity. Disorders of development of teeth may be due DEFINITION to abnormalities in the differentiation of the dental Lip pits are congenital invaginations, which can lamina and tooth germs (abnormal morpho- involve either the paramedial portion of vermilion differentiation), which results in various defects border of lower and upper lips or the labial in the number, size and form of teeth. commissural area. Types of developmental anomalies Congenital anomalies The defects, which are present at birth or before birth during the intrauterine life, are known as congenital anomalies. Hereditary developmental When certain defects are inherited by the offspring from either of the anomalies parents, it is called hereditary anomaly. Such types of anomalies are always transmitted through genes. Acquired anomalies Acquired anomalies develop during intrauterine life due to some pathological environmental conditions. They are not transmitted through genes. Hamartomatous A hamartoma can be defined as an excessive, focal overgrowth of anomalies mature, normal calls and tissues, which are native to that particular anatomic location. Developmental abnormalities occurring due to such hamartomatous change in the tissue are known as hamartomatous developmental anomalies. Idiopathic anomalies Developmental abnormalities of unknown cause are called idiopathic anomalies.  2 Essentials of Oral Pathology ORIGIN In both lip and commissural pits, there are no The condition arises probably due to notching of signs of inflammation or ulceration and both the lip at the early stage of labial development, conditions are harmless. which causes fixation of tissue at the base of the notch. The condition may also arise due to failure TREATMENT of a complete union of the embryonic lateral sulci While commissural pits require no treatment, the of the lip, which persist in the later life. Both lip lip pits are sometimes surgically excised for pits and the commissural pits are developmental cosmetic reason. malformations, which appear to be inherited as autosomal dominant traits. DOUBLE LIP CLINICAL FEATURES Double lip is a developmental anomaly charac- Lip Pits terized by a horizontal fold of excess or redundant tissue, on the mucosal side of the lip. It is usually The lip pit is a small depression over the lip, located on the inner aspect of upper lip, although which can be either unilateral or bilateral and the lower lip can also be occasionally involved. are more commonly seen on the lower lip. These pits can be up to 3 to 4 mm in diameter CLINICAL FEATURES and may have a depth of up to 2 cm. Lip pit occur more commonly among females Double lip is an oral anomaly, which can be and their frequency ranges from 1: 75000 to 1: either a congenital or an acquired one, the 100000 among Caucasians. aquired type occurs mostly due to trauma. Congenital lip pits may occur either as an The condition clinically appears as a “cupid’s isolated condition or they may be associated bow” when the lip is tense but it is not visible with cleft lip and/or cleft palate (Van der when the lip is at rest. Woude’s syndrome). The defect can occur either alone or in The opening of a lip pit on the labial surface association with other anomalies. often appears as a circular or transverse slit, Double lip in association with blepharochalasis moreover a lip pit opening may be located at (dropping of the upper eye lid) and nontoxic the apex of a nipple like elevation. thyroid enlargement are known as Ascher’s Mucous secretion is visible at the opening of Syndrome. those pits, which communicate with an Clinically a double vermillion border is underlying minor underlying salivary gland. apparent with a transverse furrow between the Since the salivary gland orifices open into these two borders, when the patient smiles. pits, as a result saliva often exudates from them. However, exudation of mucus from lip pits onto TREATMENT the lower labial skin may cause embracement Although, it is excised sometimes for cosmetic to the patient. reasons, double lip mostly requires no treatment. Commissural Pits FRENAL TAG The commissural pits measure from 1 to 4 mm in diameter, are found either bilaterally or DEFINITION unilaterally and often they have a familial Frenal tag is a redundant piece of mucosal tendency. tissue, which projects from the maxillary labial Commissural pits can occur in association frenum. with multiple preauricular pits. Unlike lip pits, the commissural pits are more CLINICAL FEATURES frequent among males and black people are It is a familial condition and seems to be affected more often than whites. inherited as autosomal dominant trait.  Developmental Anomalies of Oral and Paraoral Structures 3 The shape and size of frenal tag varies from the oral mucosa (crosses vermillion border in patient to patient and is clinically asympto- about 94 percent cases). matic. Buccal mucosa is the most frequently involved Sometimes, the condition is mistaken for a intraoral site, followed by palate, gingiva, fibrous hyperplasia caused by local injury or tongue and floor of the mouth, etc. Sometimes irritation. these macules can be seen over the hands and feet as well. HISTOPATHOLOGY The skin pigmentations tend to fade away in Histologically, frenal tag consists of normal oral adult life, while the mucosal pigmentations epithelium and connective tissue. continue to persist. Intestinal polyposis is the other very important TREATMENT feature of Peutz-Jegher’s syndrome besides the melanotic pigmentations. Although the polyps No treatment is required. occur throughout the small intestine, colon and stomach are more commonly affected. HEREDITARY INTESTINAL POLYPOSIS Presence of these polyps can cause recurrent (PEUTZ-JEGHERS) SYNDROME abdominal pain (in patients younger than 25 years of age), unexplained rectal bleeding DEFINITION and prolapse of tissue from rectum with Peutz-Jegher’s syndrome is a hereditary condition diarrhea. characterized by gastrointestinal hamartomatous Occasionally intussusceptions and intestinal polyposis in association with mucocutaneous obstruction may cause even death. pigmentations. The syndrome can also cause precocious The disease is transmitted either through an puberty, menstrual disturbances in females, autosomal dominant gene or it can occur gynecomastia in males and development of spontaneously. It is a developmental condition testicular mass, etc. and although pigmentation is an important The polyps occur either as a hamartomatous feature of this disease, the primary disorder is growth or as an inflammatory lesion and they actually not of the melanocyte system. may have a very limited neoplastic potential. There can be growth acceleration in few Incidence rate: Approximately, one case per patients due to concomitant occurrence of 60,000 to 300,000 populations. sertoli cell tumor. CLINICAL FEATURES Key points of Peutz-Jegher’s syndrome Peutz-Jegher’s syndrome begins in infancy Melanin pigmentations of the vermillion border (2nd and 3rd decade of life) and there is no sex Multiple intestinal polyps predilection. Recurrent abdominal pain and obstruction Patients almost always have a positive history Precocious puberty in some cases. of the disease in the family. There is multifocal melanin pigmentations in HISTOPATHOLOGY the perioral locations, which often manifest as Histologic examination of the oral macular lesions discrete, brown to bluish-black or purpleblack exhibits excessive accumulation of melanin macules on the skin. granules in the basal cell layer. The size of the macule varies from 1 to 5 mm in diameter and these macules often group around the oral, nasal and orbital orifices. DIFFERENTIAL DIAGNOSIS The pigmentation is most intense at the Albright syndrome vermilion border of the lower lip and it often Addison’s disease extends both to the facial skin as well as into Oral melanotic macule.  4 Essentials of Oral Pathology DIAGNOSTIC ASSESSMENT HISTOPATHOLOGY Pigmentation in the perioral region is unique Microscopically, oral melanotic macule and often diagnostic for Peutz-Jegher’s presents diffuse accumulations of melanin syndrome. granules in the basal keratinocytes and the Radiographic examinations can be useful for lamina propria. detection of intestinal polyps. The lesions do not evolve from proliferation of Genetic counseling. melanocytes and there is no risk of malignant Familial history of the diseases also helps in transformation in them. making diagnosis. Occasionally, melanin incontinence is observed with pigmented granules being seen in TREATMENT subepithelial melanophages. No treatment is required for the oral and perioral Melanophagocytosis can also be seen. melanotic macules. However, surgical interven- tion may be required for the intestinal polyps DIFFERENTIAL DIAGNOSIS causing intussusceptions. Superficial melanoma Blue nevi ORAL MELANOTIC MACULE Amalgam tattoo (EPHELIS) Addison’s disease Peutz-Jegher’s syndrome. DEFINITION Oral melanotic macule is an idiopathic benign TREATMENT pigmented lesion of oral cavity; characterized by The persistent, innocuous looking lesions do not increased focal melanin pigmentations in the oral require any treatment, however biopsy is mucosa. mandatory for a definitive diagnosis of the condition as well as to rule out any possibility of CLINICAL FEATURES malignant melanoma. Oral melanotic macules present small, flat, well circumscribed, asymptomatic areas in the oral mucosa. UVULA ELONGATA These are seen commonly on the vermilion DEFINITION border of the lip (mostly lower lip) near the midline. Intraorally, the gingiva, buccal mucosa Uvula enlongata is a developmental anomaly and the palate are the most frequently involved characterized by abnormally long uvula, which touches or hangs lower than the base of the tongue. sites. Most of the lesions are less than one centimeter in diameter or sometimes little more and their CLINICAL FEATURES color ranges from brown or black or bluish The condition is usually seen at birth and some- green, etc. times it has a familial tendency for occurrence. There is no specific age group for this condi- It is seen more frequently among females than tion, however middle aged females are most males. often affected. Although it is mostly asymptomatic, some The oral lesions are painless, firm in sensitive patients may cough or gag when the consistency and elliptical in shape. elongated uvula touches the epiglottis or the Solitary lesion of oral melanotic macule on the base of the tongue. lip is called labial melanotic macule. Acquired cases of uvula elongata, may occur The conditions are asymptomatic and have no as a result of chronic pharyngitis due to cola malignant potential. nut chewing.  Developmental Anomalies of Oral and Paraoral Structures 5 DIFFERENTIAL DIAGNOSIS Lower lip is involved more often than the upper Neoplasms of the uvula. lip and the vermilion borders as well as the labial mucosa are of normal color. TREATMENT However in many cases, the lip shows diffuse keratosis with scaling of the surface. In most of the cases, no treatment is required. Patient sometimes complain of burning However, in symptomatic cases, astringents can discomfort or a feeling of rawness in the lip. be used which will contract the uvula. In more When the lip is everted due to swelling of the severe cases, amputation is recommended. glands, its surface often reveals multiple pits or fistulas representing dilated and inflamed CHEILITIS GLANDULARIS minor salivary duct openings. Externalization and chronic exposure of the DEFINITION delicate labial mucosa often result in erosion, Cheilitis Glandularis is an uncommon, funda- ulceration, crusting and infection, etc. mentally benign, developmental anomaly of the Few cases of cheilitis glandularis may undergo lips characterized by chronic, progressive malignant transformation and produce enlargement of the labial salivary glands. carcinoma of the lip. Von Volkmann first introduced the term in Key points of cheilitis glandularis 1870 and described it as a clinically distinct, deep suppurative, chronic inflammatory condition of Swelling of the lip due to enlargement of the the lower lip with mucopurulent discharge. minor salivary glands. Lower lip involved more frequently. ETIOLOGY Lip is everted with multiple fistulas found on the surface. Chronic exposure to sun (actinic damage), Exudation on the lip surface with occasional wind and dust erosion, ulceration and crusting. Factitial injury It is predominantly caused by sun and dust Infection, e.g. HIV exposure, stress and tobacco use. Neoplasm especially squamous cell carcinoma Increased risk of malignant transformation. Use of tobacco Emotional stress TYPES Heredity. Clinically cheilitis glandularis can be of three Recent investigations indicate that over- basic types: exposure to sun with superimposed bacterial A. The simple type infection is the more likely cause of this B. The superficial suppurative type and condition. C. The deep suppurative type. The simple type is the most common variant CLINICAL FEATURES of the disease and it presents multiple, painless, Cheilitis glandularis commonly occurs among pinhead size swellings on the lip with central middle aged or elderly adults and often there depression. is a male predilection. The superficial suppurative type of cheilitis Inflammatory enlargement of the superficial or glandularis presents painless swelling of the lip deep minor salivary glands of the lip often with induration, areas of shallow ulcerations and causes progressive, multinodular swelling. crusting. There may be secretion of clear, viscous The deep suppurative type is characterized exudates from the minor salivary duct by deep seated inflammation, abscess formation openings on the labial mucosa. in the lip with development of fistulas tracts. The Enlargement of the labial salivary glands often disease often heals by scarring. cause eversion and induration of the lower Many believe these subtypes probably represent lip. a continuation of the same disease process, i.e. if  6 Essentials of Oral Pathology the simple type of cheilitis glandularis is not believe it as a regional form of sarcoidosis or properly treated, it becomes secondarily infected Crohn’s disease, while others suggest it as a and progresses to the next type and then to the granulomatous lesion of allergic origin. next. The disease may also occur due to hyper- sensitivity to bacterial toxins from a chronic focus DIFFERENTIAL DIAGNOSIS of infection in another nearby location. Cheilitis granulomatosa Crohn’s disease CLINICAL FEATURES Bacterial infection (Elephantiasis nostras Children and young adults commonly develop verrucosa) this disease (median age 25 years) and usually Actinic cheilitis there is a female predominance. Squamous cell carcinoma Either lower or upper or both lips show a Eczematous cheilitis sudden diffuse, nontendered, nodular Chronic factitial injury. enlargement, which involves the entire lip. Generally, the lower lip is enlarged on a more HISTOPATHOLOGY regular basis. The surface epithelium can be either normal or In some rare cases, patients exhibit lip swelling along with swelling of cheeks, eyelids and hyperkeratotic. scalp, etc. The underlying salivary gland tissue shows The swelling is usually painless, firm and hypertrophy and inflammation with distention exhibits no pitting upon pressure. of acini. There is no sign of inflammation or ulceration Squamous metaplasia of the ductal epithelium on the surface of involved lips in the initial stage. may be seen. During the early stage, the disease is sometimes Dysplastic changes can be noted in some accompanied by fever, malaise and visual cases especially in type II and type III cases disturbance, etc. Regional lymph nodes are with increased risk of malignant transfor- enlarged in about 50 percent cases. mation. The initial swelling subsides within few hours Frank evidences of squamous cell carcinoma or days, however with more and more attacks are reported in about 18 to 25 percent cases in of the disease, the swelling tends to become relation to this disease. larger in size and persists longer, and eventually become permanent. Some lesions can TREATMENT even regress very slowly over the years. Biopsy is mandatory especially in suspected Enlarged lips often create some cosmetic cases, where the lip shows excessive keratosis or problems due to the presence of several cracks ulcerations. and fissures on the surface along with a Lesions with premalignant changes should be reddish-brown discoloration. treated by surgical stripping of the lip without in- Patient may also have difficulties during volving the vermillion border to save the esthetics. eating, drinking or talking. Few lesions may exhibit scaling, fissuring and CHEILITIS GRANULOMATOSA vesicle or pustule formation at the vermilion border. The fissured lip is often painful and is Cheilitis granulomatosa is an atypical granulo- firm, rubbery in consistency. matous disease of the lip, the origin of which is Patients sometime complain of decreased not clearly understood. salivary secretion and loss of taste sensation. Cheilitis granulomatosa in association with PATHOGENESIS facial paralysis and fissured tongue As mentioned above the exact cause of cheilitis constitutes the “ Melkersson-Rosenthal granulomatosa is not known. Some investigators Syndrome”.  Developmental Anomalies of Oral and Paraoral Structures 7 The facial paralysis is intermittent at first but Generalized edema and dilated blood vessels later on it becomes permanent in nature, the are present throughout the connective tissue. paralysis may be unilateral or bilateral, complete or incomplete. DIFFERENTIAL DIAGNOSIS Besides lip lesions, few other oral lesions can Sarcoidosis possibly occur in association with cheilitis Cheilitis glandularis. granulomatosa, these include nodular or Angioneurotic edema papillary tumor on the tongue, some Leprosy multinodular lesions and a reddish or bluish Crohn’s disease plaque on the buccal mucosa or palate. Traumatic injury Lymphoma Key points of cheilitis granulomatosa Edema and cheilitis subsequent to odontogenic Diffuse, firm painless swelling of the lip with infections. difficulty in eating, drinking and talking. Granulomatous inflammation or allergy is the TREATMENT suspected underlying cause. Intralesional injection of steroid (Triamci- Cheilitis granulomatosa, facial paralysis and nolone) may result in reduction in the size of the scrotal tongue constitute Melkersson-Rosenthal lesion. syndrome. Surgical excision of the granulomas may be effective but often there is recurrence. HISTOPATHOLOGICAL FEATURES Elimination of odontogenic and periodontal Microscopically, cheilitis granulomatosa (Fig. infections in the vicinity may produce reduc- 1.1) shows granulomatous inflammation of the tion in the signs and symptoms of the lip with infiltration by chronic inflammatory disease. cells, chiefly lymphocytes, plasma cells and histiocytes. ANOMALIES OF THE ORAL The multinodular, noncaseating granulomas MUCOSA are often located close to the blood vessels and these are composed of epitheloid cells and FORDYCE’S GRANULES swirled collagen fascicles with interspersed Langhans type of multinucleated giant DEFINITION cells. Fordyce’s granules are ectopic collections of These noncaseating granulomas often simulate numerous sebaceous glands, generally unasso- sarcoidosis and these lesions may replace the ciated with hair follicles and are found in various minor salivary glands of the lip. locations within the oral cavity (Fig. 1.2). Fig. 1.1: Photomicrograph of cheilitis granulomatosa Fig. 1.2: Fordyce’s granules  8 Essentials of Oral Pathology CLINICAL FEATURES These glands are located superficially, quite Fordyce’s granules are mostly seen in adult life close to the surface epithelium and are and there is often a male predilection. These composed of 1 to 5 lobules. are rarely found in children before puberty. They empty into a duct, which opens directly They commonly occur in a bilaterally onto the mucosal surface. The duct may show symmetrical pattern over the upper lip, buccal keratin plugging. mucosa, gingiva, anterior pillars of fauces and The peripheral cells of the Fordyce’s granules occasionally over the tongue. Fordyce’s are flat and darkly stained, whereas the inner granules are seen rarely in the lower lip. cells are lipid rich and pale in appearance. Fordyce’s granules can also be present in many Key points of Fordyce’s granules extraoral locations such as the esophagus, These are ectopic collections of sebaceous genitalia, nipples, palms and the parotid glands in the oral cavity (normal location is skin). glands, etc. Appear as multiple, yellowish, milia-like discrete Normally these glands are present in the 60 to granules on the buccal mucosa (bilaterally). 70% of adult population but their number Sebaceous cyst may develop from them. varies widely between individuals. The number of Fordyce’s granules in the upper DIFFERENTIAL DIAGNOSIS lip increases during puberty while there is an The smaller size, multiplicity and typical yellow increase in the number of these glands in the color are characteristic of the Fordyce’s granules buccal mucosa in the later stages of life. and are unlikely to be confused with any other Fordyce’s granules clinically appear as multiple, mucosal lesions. small, discrete, rice-like or milia-like yellowish- white bodies beneath the surface mucosa. They TREATMENT measure about 1 to 2 mm in diameter. No treatment is required for this condition. Those are completely asymptomatic conditions and in most of the cases patients are not aware However, on rare occasions, sebaceous cysts or of their presence in the mouth. adenomas may develop from the preexisting On occasions, clusters of numerous sebaceous Fordyce’s granules. glands may form slightly raised confluent plaques with a creamy appearance. FOCAL EPITHELIAL HYPERPLASIA DEFINITION HISTOPATHOLOGY Focal epithelial hyperplasia (commonly known Histologically, Fordyce’s granules of the oral as Heck’s disease) is a condition characterized mucosa are identical to those of sebaceous by multiple papillary or sessile hyperplastic areas glands, which are normally found in the skin, in the oral mucosa. except the fact that they are not associated with hair follicles (Fig. 1.3). ETIOLOGY AND PATHOGENESIS The disease is specifically found among American Indians, Northern native people and other ethnic groups in Europe and Africa. The exact etiology of Heck’s disease is not known but it is probably caused by the Human Papilloma Virus (HIV) type 13 and 32. Similar appearing lesions may also be encounte- red among HIVseropositive homosexual males. CLINICAL FEATURES The disease commonly occurs among children between the ages of 3 to 18 years and there is Fig. 1.3: Photomicrograph of Fordyce’s granules no sex predilection.  Developmental Anomalies of Oral and Paraoral Structures 9 Clinically, focal epithelial hyperplasia presents WHITE SPONGE NEVUS multiple, small, pedunculated, polypoid or nodular soft tissue growths in the oral cavity. DEFINITION They also can appear as well demarcated, White sponge nevus or Cannon‘s disease is a slightly raised plaques. congenital mucosal abnormality, which appears Sometimes several hyperplastic lesions may to follow an autosomal dominant hereditary cluster together to produce a typical pattern and manifests as a white lesion of the oral “Cobblestone” appearance. mucosa. Labial and buccal mucosa are the most common sites and lower lip is more frequently affected PATHOGENESIS than the upper. However, the disease can also This condition is transmitted as an autosomal involve the tongue, gingiva and anterior faucial dominant trait with incomplete penetrance and pillars, etc. variable expressivities. Individual lesions measure about 1 to 5 mm in Mutations in the genes coding for keratins 4 diameter and are either white or pink in color. and 13 (the pair of keratins expressed by Most of the lesions regress spontaneously after epithelial cells in the mucosa affected by the about 4 to 6 months and occasionally few disorder) have been identified, suggesting the lesions can recur. disorder as a hereditary keratin defect. The heaping up of cells on the surface of the epithelium also suggests the possibility of an DIFFERENTIAL DIAGNOSIS impaired normal desquamation process of the Leukoplakia superficial strata of cells. Psoriasis Keratoacanthoma CLINICAL FEATURES Veruciform Xanthoma. White sponge nevus has its onset mostly during childhood. Some lesions are congenital and are HISTOPATHOLOGY present at birth and few lesions may even Focal epithelial hyperplasia histologically initiate during the adolescent period. shows hyperparakeratosis of the covering There is no sex predilection, several members epithelium with extensive acanthosis of the same family are often affected. (increased thickening of the spinus cell layer). The intraoral lesions are almost always The epithelial cells of the upper spinus layer bilateral and are mostly found over the buccal show enlarged nuclei and vacuolated clear mucosa and tongue. Occasionally, the cytoplasms (koilocytes). vestibular mucosa, palate, gingiva and floor of Deeper layer of epithelium reveals thickening, the mouth, etc. are also affected. elongation and even fusion of the retepegs. Besides the oral mucosa, white sponge nevus Basal cell layer of the epithelium exhibit can be seen on the conjuctivital, esophageal, increased mitotic activity. nasal and anogenital mucosa as well. Occasionally focal areas of liquefaction Clinically, white sponge nevus presents a thick, degeneration of the basal layer may be found. bilateral, asymptomatic, deeply folded or The underlying connective tissue is loose, well corrugated white or gray lesion on the oral vascularized and exhibit variable infiltrates of mucosa. lymphocytes. The surface of the lesion is soft, uneven and has a shaggy appearance with a spongy TREATMENT consistency. Since focal epithelial hyperplasia is a harmless, The lesions can be either diffuse or patchy and self-regressing condition, it usually requires no have a translucent opalescence similar to that treatment. of leukodema.  10 Essentials of Oral Pathology The disease can sometimes present ragged Lichen planus white areas, which can be peeled off by gentle Candidiasis rubbing without any ensuing bleeding. Leukodema. It is a perfectly benign condition but is often mistaken for leukoplakia. TREATMENT No treatment is required for this disease. HISTOPATHOLOGY White sponge nevus histologically shows marked thickening of the epithelium with DEVELOPMENTAL DEFECTS OF mild to moderate hyperparakeratinization, THE GINGIVAE acanthosis and spongiosis. Marked intracellular edema of the spinus and FIBROMATOSIS GINGIVAE parakeratinized cell layer of the epithelium is an important characteristic feature of the DEFINITION disease. Fibromatosis gingivae are rare, benign, diffuse, The cells with intracellular edema show vacuo- noninflammatory hyperplasia of the gingival lated cytoplasms and shrunken (pyknotic) tissue, which sometimes cover the entire teeth. nuclei. It is a hereditary condition, which is trans- Intrestingly under microscope only the cell mitted as an autosomal dominant trait. The walls and the pyknotic nuclei at the centers of disease is probably the result of a diffuse infilt- the cells are visible, which often gives rise to a rative hyperplasic proliferation of the fibroblast so called “basket weave” appearance. cells and mature collagen fibers of the gingival Parakeratin plugging is another important tissue. finding in white sponge nevus, which runs from the surface and extends deep into the CLINICAL FEATURES spinus layer. Clinically the disease is characterized by dense, Individual cell keratinization may be seen in diffuse, smooth or nodular overgrowth of the spinus cell layer. gingival tissue. The basal cells are intact and the lamina Fibromatosis gingivae can be either genera- propria shows no inflammatory changes. There is no evidence that this lesion undergoes lized or localized in nature and it is similar in malignant transformation. appearance to the dilantin hyperplasia of gingiva. ELECTRON MICROSCOPIC STUDY The gingival enlargement mostly appears in young children or it may be present even at Ultra structural studies of white sponge nevus birth, however in some cases the swelling may reveal that some cells of the spinus layer not be noticed until the adult life. differentiate early and become enriched with tonofilaments. Both sexes are equally affected in gingival fibromatosis. DIFFERENTIAL DIAGNOSIS The gingiva shows multinodular enlargements Thick, corrugated, diffuse white lesion in the especially in the interdental papilla regions. mouth, with history of involvement of several The gingival tissue changes become obvious members of the same family is usually suggestive soon after the eruption of the deciduous teeth. of white sponge nevus. The hyperplastic tissue is firm, painless and However the following diseases are to be retains the normal coral pink color of gingiva. considered in the differential diagnosis of white Sometimes the markedly enlarged gingiva sponge nevus. may cover the entire crown of the erupted Leukoplakia teeth. The eruption process of teeth is however Hereditary intraepithelial dyskeratosis normal.  Developmental Anomalies of Oral and Paraoral Structures 11 Elimination of dental plaque does not make TREATMENT any significant reduction in the severity of the Periodic gingivectomy with placement of gingival disease. acrylic splints for cosmetic and functional reasons. Occasionally, the condition is associated with hypertrichosis, epilepsy and mental retar- dation, etc. RETROCUSPID PAPILLA Gingival fibromatosis can also be associated Retrocuspid papilla is a slightly raised area of with other syndromes, e.g. Cowden’s syndrome mandibular alveolar mucosa, which as the name and Rutherford’s Syndrome (features of implies, is commonly located lingual to the cuspids. individual syndrome are given in the relevant This structure measures about 2 to 4 mm and is chapter dealing with syndromes). often present bilaterally between the marginal Moreover, gingival fibromatosis can be a gingiva and the mucogingival junctions. feature of “Laband Syndrome”, which is Retrocuspid papilla is more common among characterized by splenomegaly, enlarged nasal children and it has structural resemblance to and external ear soft tissue, shortened terminal the incisive papilla. phalanges, hypermobility of joints and Histologically, the papilla represents a focus hypoplasia of nails. of fibrovascular tissue with an orthokera- Sometimes symmetrical fibrous overgrowths tinized or parakeratinized surface and it may occur bilaterally in the maxillary usually covers an osseous foramen of a nutrient tuberosity region and few of such cases are blood vessel. possibly related to gingival fibromatosis. HISTOPATHOLOGY DEVELOPMENTAL ANOMALIES Histopathologically gingival fibromatosis INVOLVING THE JAWBONE presents the following features: The covering epithelium is hyperplastic and often exhibits thin elongated retepegs. AGNATHIA The fibrous connective tissue consists mainly Agnathia refers to the complete failure of of course bundles of collagen fibers with development of jawbone, involving either maxilla scattered mature spindle shaped fibroblasts or mandible or even both the jaws. It is an extremely few of which are multinucleated. rare condition, however, often a portion of the Mucoid changes in the gingival connective jawbone, e.g. premaxilla, condyle, or ramus, etc. tissue may occur due to the accumulation of can be developmentally missing. excessive amount of ground substances. There are significant numbers of mast cells often associated with the fibroblastic prolifera- MICROGNATHIA tion. Micrognathia is an orofacial anomaly charac- Histologically, hereditary gingival fibromatosis terized by development of jaws, which is indistinguishable from other forms of are unusually smaller than normal (Figs 1.4 gingival enlargements including those which to 1.8). are drug-induced. CAUSES DIFFERENTIAL DIAGNOSIS Pierre-Robin syndrome Phenytonin (Dilatin) sodium induced gingival Hallerman-Steriff syndrome hyperplasia. Trisomy 13 Generalized hyperplastic gingivitis. Trisomy 18 Leukemic infiltration of the gingiva. Turner syndrome History of familial involvement is extremely Marfan syndrome important in making the diagnosis of the disease. Progeria  12 Essentials of Oral Pathology Fig. 1.4: Micrognathia (seen from front)-I Fig. 1.6: Hemifacial microstomia (seen from side)-I Fig. 1.5: Micrognathia (seen from side)-II Fig. 1.7: Hemifacial microstomia (seen from front)-II TYPES OF MICROGNATHIA Micrognathia can be of two types: A. Pseudomicrognathia B. True micrognathia Pseudomicrognathia: It is a condition where normal sized jawbone appears to look smaller when compared with the opposing jaw. A jawbone of standard size may appear smaller, if the opposite jaw is larger than normal or if it is positioned more posteriorly in relation to the skull. True micrognathia: It is the condition, where the jawbone is actually smaller than normal and it Fig. 1.8: Radiograph of hemifacial can be either a congenital or an acquired problem. microstomia  Developmental Anomalies of Oral and Paraoral Structures 13 The congenital micrognathia may follow a Paget’s disease of bone: Paget’s disease often hereditary pattern and it often occurs in association causes increase in the size of maxilla, which is with other congenital diseases such as Pierre-Robin directly related to the generalized overgrowth Syndrome or congenital heart disease, etc. of the cranium. Congenitally missing premaxilla often leads Acromegaly: Progressive increase in the size of to maxillary micrognathia and patients with this mandible occurs in cases of acromegally. anomaly show retracted middle third of the face. Leontasis ossea: It is a form of fibrous dysplasia Congenital mandibular micrognathia may of bone and the disease is often associated with occur due to posterior positioning of the condyle enlargement of maxilla. in relation to the skull. Hereditary causes: Mandibular prognathism Acquired micrognathia mostly occurs due to often occurs hereditarily. trauma or severe infections in the orofacial region Mandibular prognathism may occur due to especially in younger age. It may cause difficulty anterior positioning of the lower jaw in relation to in feeding the child. Ankylosis of the temporo- the cranium, even though the exact size of the jaw mandibular joint in young individuals often is normal. leads to mandibular micrognathia with retruded However, there are certain factors, which cause chin. mandibular prognathism and thereby create an appearance of mandibular macrognathia. CLINICAL FEATURES OF MICROGNATHIA These factors are as follows: Micrognathia often results in defective align- Increased height of the ramus. ment of teeth, crowding and malocclusion, etc. Increased length of the body of mandible. Retruded chin with small face. Increased gonian angle. Difficulty in feeding the children. Decreased maxillary length. Difficulty in proper articulation and speech. Posterior positioning of the maxilla in relation to the cranium. MACROGNATHIA Prominent chin button. Varying soft tissue contours. Macrognathia is a developmental anomaly characterized by an abnormally large jaw. The CLINICAL FEATURES OF MACROGNATHIA condition can affect both the jaws at a time but more Mandibular protrusion (when manduibular often it involves either maxilla or mandible. bone is affected). Gummy smile (mostly maxillary). TYPES OF MACROGNATHIA Ramus of mandible forms a less steep angle True macrognathia: When the jawbone is abnor- with body of the mandible. mally large in size in true sense, it is called true Excessive condylar growth. macrognathia. Prominent chin. Pseudomacrognathia: A normal sized jaw may look larger when the opposing jaw is smaller than TREATMENT normal in size. This condition is known as In case of macrognathia, surgical correction pseudomacrognathia. (osteotomy) of the abnormally large jaw is often performed for both functional and esthetic COMMON CAUSES OF TRUE reasons. MACROGNATHIA Pituitary gigantism: It is often associated with FACIAL HEMIHYPERTROPHY abnormally large jawbones. Both jaws are affected in gigantism and the bony overgrowth DEFINITION (Figs 1.9 and 1.10) is proportionate to the generalized increase in Facial hemihypertrophy is a developmental the size of the skeleton. condition characterized by disproportionate  14 Essentials of Oral Pathology appear to be vascular and neurogenic distur- bances that cause an increased neurovascular supply to the affected side of the face resulting in its overgrowth. Other possible factors include the following: Hormonal imbalance Incomplete twining Chromosomal abnormality Defective intrauterine development (due to asymmetric intrauterine pressure) Lymphatic abnormalities. CLINICAL FEATURES (FIGS 1.11 AND 1.12) Facial hemihypertrophy clinically exhibits the following features: Unilateral enlargement of the facial soft tissues, Fig. 1.9: Facial hemihypertrophy (seen from front)-I bones and teeth. A positive family history is reported in many of these cases. Either side of the face can be affected and there is a slight female predilection for this condition. The asymmetry is more specifically noticed in the frontal bone, maxilla, palate, mandible, alveolar process, condyles and the associated soft tissues. The skin is thick and coarse on the affected side and also there is presence of thick and abundant hair (hypertrichosis). Fig. 1.10: Facial hemihypertrophy (seen from side)-II unilateral enlargement of the face (Figs 1.9 and 1.10). Though the name is hemihypertrophy the actual underlying condition is a hyperplasia. Although most humans exhibit some degrees of facial asymmetry only few individuals actually develop clinically significant facial hemihyper- trophy. ETIOLOGY Although, a number of factors have been proposed to explain this condition, the most important ones Fig. 1.11: Facial hemihypertrophy-III  Developmental Anomalies of Oral and Paraoral Structures 15 DIFFERENTIAL DIAGNOSIS Differential diagnosis of facial hemihypertrophy includes the following: Neurofibromatosis Fibrous dysplasia is of bone Arteriovenous malformations of jaws. Key points of facial hemihypertrophy Unilateral enlargement of face including jaws, teeth, alveolar bone and soft tissues. Crown size of tooth and roots are larger on the Fig. 1.12: Radiograph of facial affected side. hemihypertrophy Teeth on the affected side erupt earlier, tongue The sebaceous and sweat glands on the affected is larger and malocclusion is common. side show excessive secretions. It’s a developmental defect but hormonal The ear and eye on the affected side may also imbalance and lymphatic abnormality, etc. are be enlarged. also important predisposing factors. Unilateral enlargement of the cerebral hemisphere may cause mental retardation and TREATMENT seizure in about 15 to 20% cases. No treatment is usually required for facial hemi- There can be an increased incidence of certain hypertrophy. However, selective surgical treat- systemic conditions in facial hemihypertrophy ments may be performed to improve functional such as Wilms, tumor of kidney, adrenocortical and cosmetic status wherever necessary. tumor and hepatoblastoma, etc. On rare occasions, the hypertrophy may extend FACIAL HEMIATROPHY beyond the face and include the limbs or even DEFINITION the entire one side of the body. Facial hemiatrophy also known as Parry- Romberg syndrome, is a developmental anomaly ORAL MANIFESTATIONS characterized by progressive decrease in the size Unilateral macroglossia with an increase in the of one side of the face due to atrophy of the facial size of the fungiform papilla is often seen. structures. Crowns and roots of teeth, especially of the ETIOLOGY permanent teeth are often enlarged on the The exact etiology of the disease is not known affected side. however certain possible factors have been The teeth on the affected side may also erupt identified which can precipitate the condition. prematurely. These factors include the following: There is often early shedding of deciduous teeth Peripheral nerve dysfunction on the affected side. Trauma The jawbone proper is larger and thicker on Heredity the affected side. Peripheral trigeminal neuritis Because of the osseous and dental asymmet- Infection ries in facial hemihypertrophy malocclusion Regional systematic sclerosis. often develops. Because of the overgrowth of soft tissue, buccal CLINICAL FEATURES mucosa on the affected side appears pendulus The condition usually begins in the first or and velvety, and folds of tissue may hang from second decade of life. Many cases may be there in the oral cavity. present since birth.  16 Essentials of Oral Pathology Initially a slightly depressed vertical furrow or lip to fuse together at the time of development. line is noticed at the midline of the forehead This defect is more commonly seen in relation to and eyebrow. the upper lip. As the condition progresses, facial tissues on Cleft palate: It is a developmental defect of palate the affected side including the skin, subcuta- characterized by lack of complete fusion of two neous tissue, muscle and bone, etc. become lateral halves of the palate resulting in a cleft. Cleft atrophic, resulting in facial deformity. in the palate leads to communication between oral Usually the left side of the face is involved more and the nasal cavity. often than the right side. Cleft of the maxillofacial skeleton are quiet Affected side of the face shows hyperpig- common entities and these can involve many mentation of skin with loss of hair. structures of the orofacial region. Severe cases may often result in hollowing of the cheek and depressed eye in the orbit. Congenital malformations causing Other features associated with facial clefts in the orofacial region hemiatrophy include trigeminal neuralgia, Cleft lip contralateral jacksonian epilepsy and ocular Clefts of the primary palate and hair changes. Cleft of the secondary palate The cartilage of the ear, nose and larynx, etc. Mandibular cleft may be affected. Oblique facial cleft Occasionally the disease may affect other parts Submucosal cleft palate of the body. Bifid uvula Pits of the lip. ORAL MANIFESTATIONS Intraoral tissues on the affected side exhibit an These developmental defects often have very overall atrophy. serious impacts on the growth, development and Delayed development of the jawbone. functions of the involved facial organ. Moreover, Tooth eruption on the affected side may also be such defects can jeopardize the appearance of the retarded. face and badly affect the personality of the patient Teeth on the affected side often have shorter as well. crowns and roots. Development of the roots of teeth on the affected ETIOLOGY side is also delayed. The etiology of cleft lip and cleft palate covers both hereditary and environmental factors. TREATMENT Hereditary Factors There is no effective treatment for facial Heredity is the most important single factor in the hemiatrophy. Progression of the condition ceases development of cleft lip and cleft palate. Different after certain age and it remains static thereafter studies indicate that nearly 40 percent of cleft lip for the remaining part of life. cases with or without cleft palate are hereditary in origin. Heredi