Oral Signs of Systemic Disease Nasim Fazel Editor 123 Oral Signs of Systemic Disease www.ajlobby.com Nasim Fazel Editor Oral Signs of Systemic Disease www.ajlobby.com Editor Nasim Fazel Department of Dermatology University of California, Davis Sacramento, CA USA ISBN 978-3-030-10861-8 ISBN 978-3-030-10863-2 (eBook) https://doi.org/10.1007/978-3-030-10863-2 © Springer Nature Switzerland AG 2019 This work is subject to copyright All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed The use of general descriptive names, registered names, trademarks, service marks, etc in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland www.ajlobby.com To my beautiful children, Sina and Monah, who inspire me to persevere through any challenge and bring so much love and joy into my life www.ajlobby.com Foreword Oral Signs of Systemic Disease provides valuable insight into solving diagnostic challenges Careful oral examination can provide such clues to the learned observer However, the medical education and training of most physicians and other healthcare providers does not emphasize the oral cavity This results in a lack of familiarity with normal and abnormal features and the diagnostic clues they can provide By contrast, dental education provides the necessary skills to distinguish between normal and abnormal findings However, the dental clinician may not be skilled in placing abnormal features in the context of a mucocutaneous or systemic condition Thus, this contribution serves to educate both physicians and dentists Dr Nasim Fazel is an eminently well-suited editor for Oral Signs of Systemic Disease She is a DDS graduate of Northwestern University Dental School and an MD graduate of the University of Michigan Medical School This education was followed by residency training in dermatology at Henry Ford Hospital in Detroit, Michigan Dr Fazel has been a faculty member at the University of California, Davis in the Department of Dermatology for more than a decade and a half, where she has developed an oral dermatology practice and has served as Dermatology Residency Program Director As a dual-trained dentist and dermatologist, as well as a consummate educator, Dr Fazel brings great knowledge and experience to bear on the topic The contributors to this textbook are scholars from broad areas of expertise including dentistry, pediatric dermatology, oral dermatology, oral medicine, oral pathology, microbiology, and medical genetics Each brings a depth of knowledge and understanding of the relevance of their topics to the problem-solving algorithm for the patient with troublesome oral lesions Dermatologists know that the skin is often a mirror of systemic diseases Those skilled in the diagnosis of challenging oral diseases know that the mouth is, similarly, a mirror of systemic diseases This book will be a valuable addition to medical libraries as a reference volume and to the personal library of the clinician whose patient has a vexing oral disease Roy S. Rogers Professor of Dermatology Mayo Clinic College of Medicine Scottsdale, AZ, USA vii www.ajlobby.com Preface The conception of Oral Signs of Systemic Disease came about with the intention to provide a practical reference for the day-to-day practice of clinicians in the fields of dermatology, dentistry, oral medicine, and otolaryngology In addition, the comprehensive nature of this textbook can serve as an educational tool for students, residents, and fellows in training with the goal of learning the fundamentals of oral mucosal disease Oral Signs of Systemic Disease provides descriptive clinical and oral manifestations and differential diagnoses, including principles of therapy of major entities, while maintaining a uniform chapter format I am greatly appreciative of the authors for their contributions, without whom this textbook would not have been possible I would also like to thank Portia Wong, Diane Lamsback, and Rebekah Collins at Springer Publishing for their time and support Sacramento, CA, USA Nasim Fazel ix www.ajlobby.com Contents 1 Introduction�������������������������������������������������������������������������������������� 1 Parastoo Davari and Nasim Fazel 2 Oral Signs of Gastrointestinal Disease ������������������������������������������ 9 John C Steele 3 Oral Signs of Hematologic Disease������������������������������������������������ 25 Diana V Messadi and Ginat W Mirowski 4 Oral Signs of Endocrine and Metabolic Diseases������������������������� 45 Jaisri R Thoppay, Thomas P Sollecito, and Scott S De Rossi 5 Oral Signs of Nutritional Disease �������������������������������������������������� 63 Stanislav N Tolkachjov and Alison J Bruce 6 Oral Signs of Connective Tissue Disease���������������������������������������� 91 Kenisha R Heath and Nasim Fazel 7 Oral Signs of Vesiculobullous and Autoimmune Disease�������������� 113 Michael Z Wang, Julia S Lehman, and Roy Steele Rogers III 8 Oral Signs of Viral Disease�������������������������������������������������������������� 145 Danielle N Brown, Ramya Kollipara, and Stephen Tyring 9 Oral Signs of Bacterial Disease������������������������������������������������������ 169 Emily W Shelley and Rochelle R Torgerson 10 Oral Signs of Tropical, Fungal, and Parasitic Diseases���������������� 193 Ricardo Pérez-Alfonzo, Silvio Alencar-Marques, Elda Giansante, and Antonio Guzmán-Fawcett 11 Oral Signs of Genetic Disease �������������������������������������������������������� 227 Julio C Sartori-Valinotti and Jennifer L Hand Index���������������������������������������������������������������������������������������������������������� 253 xi www.ajlobby.com Contributors Silvio Alencar-Marques, MD, PhD Department of Dermatology and Radiotherapy, Botucatu School of Medicine – São Paulo State University (UNESP), Botucatu, Brazil Danielle N. Brown, MD Department of Pediatrics, Massachusetts General Hospital for Children, Boston, MA, USA Alison J. Bruce, MD Department of Dermatology, Mayo Clinic, Jacksonville, FL, USA Parastoo Davari, MD Department of Dermatology, University of California, Davis, Sacramento, CA, USA Scott S. De Rossi, DMD Department of Oral Medicine, Augusta University Dental College of Georgia, Augusta, GA, USA Nasim Fazel, MD, DDS Department of Dermatology, University of California, Davis, Sacramento, CA, USA Elda Giansante, MD University Hospital Caracas, Central University of Venezuela, Caracas, Venezuela Antonio Guzmán-Fawcett, MD Pierre Fauchard Faculty of Dentistry, Autonomous University of Paraguay, Asunción, Paraguay Jennifer L. Hand, MD Department of Dermatology, Mayo Clinic and Foundation, Rochester, MN, USA Kenisha R. Heath Air Force Institute of Technology, Wright-Patterson AFB, OH, USA Ramya Kollipara, MD Dermatology, Cosmetic Laser Dermatology, San Diego, CA, USA Julia S. Lehman, MD Departments of Dermatology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA Diana V. Messadi, DDS, MMSc, DMSc Section of Oral Medicine and Orofacial Pain, Division of Oral Biology and Medicine, UCLA School of Dentistry, Los Angeles, CA, USA xiii www.ajlobby.com Contributors xiv Ginat W. Mirowski, DMD, MD Department of Oral Pathology Medicine, Radiology, Indiana University School of Dentistry, Indianapolis, IN, USA Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN, USA Ricardo Pérez-Alfonzo, MD Dermatologic Program, “Jacinto Convit” Institute of Biomedicine, Central University of Venezuela, Caracas, Venezuela Roy Steele Rogers III, MD Department of Dermatology, Mayo Clinic Arizona, Scottsdale, AZ, USA Julio C. Sartori-Valinotti, MD Department of Dermatology, Mayo Clinic and Foundation, Rochester, MN, USA Emily W. Shelley, DO Cleveland Medical Center, University Hospitals, Cleveland, OH, USA Thomas P. Sollecito, DMD, FDS, RCS The Robert Schattner Center, University of Pennsylvania School of Dental Medicine, Philadelphia, PA, USA John C. Steele, MB ChB, BDS, FDS(OM)RCSEd, FHEA The Leeds Teaching Hospitals NHS Trust, Leeds, UK Faculty of Medicine and Health, University of Leeds, Leeds, UK Jaisri R. Thoppay, BDS, MBA, MS Department of Oral and Maxillofacial Surgery, VCU School of Dentistry at VCU Medical Center, Virginia Commonwealth University, Richmond, VA, USA Stanislav N. Tolkachjov, MD Surgical Dermatology Group, Birmingham, AL, USA Rochelle R. Torgerson, MD, PhD Departments of Dermatology and Obstetrics and Gynecology, Mayo Clinic, Rochester, MN, USA Stephen Tyring, MD Department of Dermatology, University of Texas Health Sciences Center at Houston, Houston, TX, USA Michael Z. Wang, MD Department of Dermatology, Mayo Clinic, Rochester, MN, USA www.ajlobby.com 252 patients with Ehlers-Danlos syndrome Am J Orthod Dentofac Orthop 1997;111(1):75–84 172 Sherman SL, Allen EG, Bean LH, Freeman SB. Epidemiology of Down syndrome Ment Retard Dev Disabil Res Rev 2007;13(3):221–7 173 Sekerci AE, Cantekin K, Aydinbelge M, Ucar Fİ Prevalence of dental anomalies in the permanent dentition of children with Down syndrome J Dent Child 2014;81(2):78–83 174 Abeleira MT, Outumuro M, Ramos I, Limeres J, Diniz M, Diz P. Dimensions of central incisors, canines, and first molars in subjects with Down syndrome measured on cone-beam computed tomographs Am J Orthod Dentofac Orthop 2014;146(6):765–75 175 Oredugba FA, Eigbobo JO, Temisanren OT. Tooth crown dimensions in a selected population of Nigerians with Down syndrome West Afr J Med 2014;33(2):146–50 176 Rao D, Hegde S, Naik S, Shetty P. Malocclusion in individuals with mental subnormality – a review Oral Health Dent Manag 2014;13(3):786–91 177 de Miguel-Diez J, Villa-Asensi JR, Alvarez-Sala JL. Prevalence of sleep-disordered breathing in children with Down syndrome: polygraphic findings in 108 children Sleep 2003;26(8):1006–9 178 Guimaraes CV, Donnelly LF, Shott SR, Amin RS, Kalra M. Relative rather than absolute macroglossia in patients with Down syndrome: implications for treatment of obstructive sleep apnea Pediatr Radiol 2008;38(10):1062–7 179 Anuthama K, Prasad H, Ramani P, Premkumar P, Natesan A, Sherlin HJ. Genetic alterations in syndromes with oral manifestations Dent Res J 2013;10(6):713–22 180 Al-Sufyani GA, Al-Maweri SA, Al-Ghashm AA, Al-Soneidar WA. Oral hygiene and gingival health status of children with Down syndrome in Yemen: a cross-sectional study J Int Soc Prevent Commun Dent 2014;4(2):82–6 181 Chin CJ, Khami MM, Husein M. A general review of the otolaryngologic manifestations of Down syndrome Int J Pediatr Otorhinolaryngol 2014;78(6):899–904 182 Ribeiro CG, Siqueira AF, Bez L, Cardoso AC, Ferreira CF. Dental implant rehabilitation of a patient with Down syndrome: a case report J Oral Implantol 2011;37(4):481–7 183 Lehmann AR, McGibbon D, Stefanini M. Xeroderma pigmentosum Orphanet J Rare Dis 2011;6:70 184 Kraemer KH, Lee MM, Andrews AD, Lambert WC. The role of sunlight and DNA repair in melanoma and nonmelanoma skin cancer The xeroderma pigmentosum paradigm Arch Dermatol 1994;130(8):1018–21 185 Li C, Hu Z, Liu Z, Wang LE, Strom SS, Gershenwald JE, et al Polymorphisms in the DNA repair genes XPC, XPD, and XPG and risk of cutaneous mela- J C Sartori-Valinotti and J L Hand noma: a case-control analysis Cancer Epidemiol Biomark Prev 2006;15(12):2526–32 186 Anttinen A, Koulu L, Nikoskelainen E, Portin R, Kurki T, Erkinjuntti M, et al Neurological symptoms and natural course of xeroderma pigmentosum Brain 2008;131(Pt 8):1979–89 187 Wayli HA. Xeroderma pigmentosum and its dental implications Eur J Dent 2015;9(1):145–8 188 Lopes-Cardoso C, Paes da Silva Ramos Fernandes LM, Ferreira-Rocha J, Teixeira-Soares C, Antônio- Barreto J, Humberto-Damante J. Xeroderma Pigmentosum – a case report with oral implications J Clin Exp Dent 2012;4(4):e248–51 189 Gorlin RJ, Sedano H, Anderson VE. The syndrome of palmar-plantar hyperkeratosis and premature periodontal destruction of the teeth A clinical and genetic analysis of the Papillon-Lefevre syndrome J Pediatr 1964;65:895–908 190 Cury VF, Costa JE, Gomez RS, Boson WL, Loures CG, De ML. A novel mutation of the cathepsin C gene in Papillon-Lefevre syndrome J Periodontol 2002;73(3):307–12 191 Zhang Y, Lundgren T, Renvert S, Tatakis DN, Firatli E, Uygur C, et al Evidence of a founder effect for four cathepsin C gene mutations in Papillon-Lefevre syndrome patients J Med Genet 2001;38(2): 96–101 192 Wiebe CB, Häkkinen L, Putnins EE, Walsh P, Larjava HS. Successful periodontal maintenance of a case with Papillon-Lefevre syndrome: 12-year follow-up and review of the literature J Periodontol 2001;72(6):824–30 193 Ragunatha S, Ramesh M, Anupama P, Kapoor M, Bhat M. Papillon-Lefevre syndrome with homozygous nonsense mutation of cathepsin C gene presenting with late-onset periodontitis Pediatr Dermatol 2015;32(2):292–4 194 Eick S, Puklo M, Adamowicz K, Kantyka T, Hiemstra P, Stennicke H, et al Lack of cathelicidin processing in Papillon-Lefevre syndrome patients reveals essential role of LL-37 in periodontal homeostasis Orphanet J Rare Dis 2014;9:148 195 Hart TC, Hart PS, Michalec MD, Zhang Y, Firatli E, Van Dyke TE, et al Haim-Munk syndrome and Papillon-Lefevre syndrome are allelic mutations in cathepsin C. J Med Genet 2000;37(2):88–94 196 Ahmed B. Prosthodontic rehabilitation of Papillon- Lefevre syndrome J Coll Phys Surg Pak JCPSP 2014;24(Suppl 2):S132–4 197 Nickles K, Schacher B, Ratka-Krüger P, Krebs M, Eickholz P. Long-term results after treatment of periodontitis in patients with Papillon-Lefevre syndrome: success and failure J Clin Periodontol 2013;40(8):789–98 198 Sarma N, Ghosh C, Kar S, Bazmi BA. Low-dose acitretin in Papillon-Lefevre syndrome: treatment and 1-year follow-up Dermatol Ther 2015;28(1): 28–31 Index A Acid reflux, 21 Acrodermatitis enteropathica (AE), 78, 79 Actinomycosis, 199, 200 Actinomyces israeli, 198 clinical manifestations, 198 differential diagnosis, 198, 199 epidemiology, 198 etiopathogenesis, 198 histopathology, 199 treatment, 199 Acute atrophic candidiasis, 195, 196 Acute gonococcal urethritis in men, 172 Acute leukemias, 25 Acute lymphocytic leukemia (ALL), 25 Acute myelogenous leukemia (AML), 25, 26 Acute necrotizing ulcerative gingitivis (ANUG), 176 antibacterial therapy, 176 diagnosis, 176 differential diagnosis, 176 epidemiology, 175 etiopathogenesis, 175 excessive corticosteroids, 175 gingival manifestations, 175 leukocyte function, 175 norepinephrine, 175 oral hygiene, 175, 176 oral penicillin V and metronidazole, 176 psychological stress and impaired immune function, 175 signs and symptoms, 175 smoking, 175 spirochetes in gingival tissue, 175 stages, 175, 176 tetracycline, 176 Addison’s disease, 45 clinical features of, 47 clinical manifestations, 46 differential diagnosis, 47, 48 epidemiology, 45 etiopathogenesis, 45 oral pigmentation in, 47 oral signs and symptoms, 46 treatment recommendations, 48 Adrenal insufficiency, 45 etiopathogenesis of, 46 Advanced oral hairy leukoplakia, 157 Age-related sicca syndrome, 94 AIDS-associated lymphoma, 27 ALA synthase-2 (ALAS 2), 57 Alcoholics, 72 Alezzandrini syndrome, 136 Amyloidosis, 29, 56 chronic ulceration and macroglossia in, 56 clinical manifestations, 31, 56 differential diagnosis, 31, 32, 57 epidemiology, 56 etiopathogenesis, 31, 56 oral signs and symptoms, 31, 56 treatment recommendations, 32, 57 Anemia, 19 Aneurin See Thiamine Angina bullosa haemorrhagica (ABH) clinical manifestations, 135 differential diagnosis, 135 epidemiology, 135 etiopathogenesis, 135 oral signs and symptoms, 135 treatment recommendations, 135 Angular cheilitis/perleche, 67, 72, 78, 79, 195, 197 and median rhomboid glossitis, 230 predisposing factors, 196 Angular stomatitis, 67 Antimalarials, 104 Antimicrobials, 13 Aphthous ulcers, 16, 157, 158 Aphthous-like ulcers, 17 Arthralgias, 92, 106 Arthritis, 99 Ascorbic acid, 74 Aspergillosis amphotericin B, 210 clinical manifestations, 209 differential diagnosis, 210 epidemiology, 209 etiopathogenesis, 209 © Springer Nature Switzerland AG 2019 N Fazel (ed.), Oral Signs of Systemic Disease, https://doi.org/10.1007/978-3-030-10863-2 253 Index 254 Aspergillosis (cont.) immunological tests, 210 PCR analysis, 210 treatment, 210 types, 209 voriconazole, 210 Atrophic “bald” tongue, 38 Atrophic glossitis, 72 Atypical mycobacteria, 219, 220 clinical manifestations, 219 differential diagnosis, 220, 221 epidemiology, 218, 219 etiopathogenesis, 219 M fortuitum infection, 220 odontogenic infection, 220 oral ulcerations, 219 treatment, 221 Autoimmune disorders, 45 Avidin, 73 Azathioprine, 95 B Bacterial disease ANUG (see Acute necrotizing ulcerative gingivitis (ANUG)) CSD (see Cat scratch disease (CSD)) diphtheria (see Diphtheria) gingivitis (see Gingivitis) gonorrhea (see Gonorrhea) granuloma inguinale (see Granuloma inguinale) leprosy (see Leprosy) nonspecific ulcers, 169 periodontitis, 169 Scarlet fever (see Scarlet fever) SSSS (see Staphylococcal scalded skin syndrome (SSSS)) syphilis (see Syphilis) TB (see Tuberculosis (TB)) tularemia (see Tularemia) Bacterial endocarditis, 233 Bandler syndrome, 236 Basal cell nevus syndrome (BCNS), 240, 241 B cell lymphoma, 27 Bejel (endemic syphilis), 218 Benzoates, 13 Biliary stasis, 72 Biotin deficiency, 73 Black hairy tongue (lingua villosa nigra), 197 Blastomycosis clinical manifestations, 208 diagnosis, 208 differential diagnosis, 208, 209 epidemiology, 208 etiopathogenesis, 208 itraconazole, 209 liposomal amphotericin B, 209 in medicine, 208 mucosal involvement, 208 treatment, 209 voriconazole, 209 Bone marrow failure, 230 Breastfeeding, 229 Bullous pemphigoid (BP) clinical manifestations, 118 differential diagnosis, 118, 119 epidemiology, 117 etiopathogenesis, 118 oral signs and symptoms, 118 treatment recommendations, 119 Burkitt’s lymphoma, 27, 28 Burning mouth syndrome (BMS), 80–82 C Calcineurin inhibitors, 137 Calcipotriol, 102 Calcium channel blockers, 102 Candida septicemia, 194 Candidiasis, 195 antibiotic therapy, 193 azoles, 197 C albicans, 193 classification, 194 clinical manifestations, 194 clinical presentation, 197 environmental conditions, 193 epidemiology, 194 etiopathogenesis, 194 fluconazole, 197 gastrointestinal and genital mucous membranes, 193 Gomori-Grocott stain, 197 HIV/AIDS patients with, 195, 198 ketoconazole, 197 microscopic examination, 197 posaconazole, 198 predisposing factors, 194 prophylactic systemic therapy, 198 sodium bicarbonate mouthwash, 197 topical imidazoles, 197 topical therapies, 198 treatment, 197 voriconazole, 198 Cardiovascular disease (CVD), 69, 97 Cat scratch disease (CSD) antibiotics, 187 clinical manifestations, 186 diagnosis, 187 differential diagnosis, 187 epidemiology, 186 etiopathogenesis, 186 facial nerve palsy, 187 primary, 187 regional lymph nodes, 186 treatment, 187 Celiac disease, 15 environmental, 15 clinical manifestations, 15, 16 differential diagnosis, 16 oral signs and symptoms, 16 Index treatment recommendations, 16, 17 epidemiology, 15 etiopathogenesis, 15 genetic, 15 Centrofacial lentiginosis, 236 Cervicofacial actinomycosis, 198, 199 Cevimeline hydrochloride, 95 Cheilitis, 170 Cheilitis granulomatosa, Cheilosis, 69 Chemotherapy, leukemia, 26 Chlorpromazine, 69 Chronic hyperplastic candidiasis, 196 Chronic lymphocytic leukemia (CLL), 25 Chronic mucocutaneous candidiasis, 197 Chronic myelocytic leukemia (CML), 25 Chronic ulceration, 56 Cinnamaldehyde, 13 Circumvallate papillae, Coccidioidomycosis amphotericin B, 213 clinical manifestations, 213 differential diagnosis, 213 epidemiology, 212, 213 etiopathogenesis, 212 posaconazole, 213 salvage therapy, 213 voriconazole, 213 Common warts, 154 Competence, Condyloma acuminata, 154, 155 Congenital erythropoietic porphyria (CEP), 58 Congenital syphilis, 171 Connective tissue disease dermatomyositis clinical manifestations, 105, 106 differential diagnosis, 107 epidemiology, 105 etiopathogenesis, 105 oral signs and symptoms, 106, 107 treatment, 107, 108 MCTD clinical manifestations, 102, 103 differential diagnosis, 104 epidemiology, 102 etiopathogenesis, 102 oral signs and symptoms, 104 treatment, 104 Sjögren’s syndrome clinical manifestations, 92 differential diagnosis, 94, 95 epidemiology, 91, 92 etiopathogenesis, 92 non-visceral manifestations, 92, 93 oral signs and symptoms, 93, 94 treatment, 95, 96 visceral manifestations, 93 SLE, 96 clinical manifestations, 96, 97 differential diagnosis, 98, 99 255 etiopathogenesis, 96 oral signs and symptoms, 97, 98 treatment, 99 systemic sclerosis, 100 clinical manifestations, 100, 101 differential diagnosis, 101, 102 etiopathogenesis, 100 oral signs and symptoms, 101 treatment, 102 Corticosteroids, sjs/ten, 128 Corticotroph adenomas, 53 Cowden syndrome, 236 Crohn’s disease, epidemiology, genetic, hypersensitivity/allergy, 10 inflammatory/immunological, 10 clinical manifestations, 10 differential diagnosis, 11, 12 oral signs and symptoms, 10, 11 treatment, 12, 13 microbiological/infection, 10 oral manifestations, Cronkhite-Canada syndrome, 236 Crown, 3, Cryptococcal meningoencephalitis, 211 Cryptococcosis clinical manifestations, 211 clinical subtypes, 212 differential diagnosis, 211, 212 epidemiology, 210, 211 etiopathogenesis, 210 oral mucosal lesions, 211 treatment, 212 ulcerative/necrotic lesions, 211 Cutaneous cryptococcosis, 211 Cutaneous leishmaniasis (CL), 214 Cutaneous molluscum contagiosum, 153 Cutaneous T-cell lymphoma, 28 Cutaneous vasculitis, 93 Cyanocobalamin, 70–72 Cyclic neutropenia, 29 clinical manifestation, 28 differential diagnosis, 29 etiopathogenesis, 28 oral signs and symptoms, 28, 29 treatment recommendations, 29 Cyclophosphamide, 99 Cyclosporine, 95, 126 Cytomegalovirus (CMV), 160, 161 D Dapsone, 129, 130 Darier disease (DD), 242, 243 Dental care practice recommendations, 234 Dental erosion, differential diagnosis, 21 Dental surgery, 220 Dentinogenesis imperfecta (DI), 239 Dentition, oral cavity, Index 256 Dermatitis herpetiformis, 15, 16 clinical manifestations, 129 differential diagnosis, 129 epidemiology, 128 etiopathogenesis, 128, 129 oral signs and symptoms, 129 treatment recommendations, 129, 130 Dermatomyositis (DM), 99 clinical manifestations, 105, 106 differential diagnosis, 107 epidemiology, 105 etiopathogenesis, 105 oral signs and symptoms, 106, 107 treatment, 107, 108 Desquamative gingivitis, 123 Diffuse cutaneous systemic sclerosis (DCSS), 101 Diffuse gingival hyperplasia, 26 Diffuse stomatitis, 72 Dimorphous leprosy, 181 Diphtheria, 178, 179 characteristic sign, 178 diagnosis, 179 differential diagnosis, 178 epidemiology, 178 etiopathogenesis, 178 exotoxin, 178 treatment, 179 vaccination programs for children, 178 Direct immunofluorescence (DIF) testing, 118, 120 Disseminated histoplasmosis, 201 Donovanosis, 184 Dorsal tongue, thick exudative pseudomembrane, 80 Down syndrome (DS), 244, 245 Dry beriberi, 68 Dyskeratosis congenital (DC), 231 clinical manifestations, 230 CO2 laser therapy, 231 dental and periodontal care, 231 differential diagnosis, 231 epidemiology, 230 etiopathogenesis, 230 leukoplakia, 230, 231 photodynamic therapy, 231 Dystrophic EB (DEB), 132 E Early-periodontal disease, 232 EB simplex (EBS), 132 Eczematous dermatitis, 79 Ehlers-Danlos syndrome (EDS), 243, 244 Enamel, defects, 16, 129 differential diagnosis for, 16 Endemic syphilis, 218 Endemic treponemal diseases clinical manifestations, 217 differential diagnosis, 218 endemic syphilis, 218 epidemiology, 217 etiopathogenesis, 217 Pinta, 218 treatment, 218 yaws, 217 Endocrine and metabolic diseases Addison’s disease, 45 clinical manifestations, 46 differential diagnosis, 47, 48 epidemiology, 45 etiopathogenesis, 45 oral signs and symptoms, 46 treatment recommendations, 48 amyloidosis, 56 clinical manifestations, 56 differential diagnosis, 57 epidemiology, 56 etiopathogenesis, 56 oral signs and symptoms, 56 treatment recommendations, 57 hemochromatosis, 58 clinical manifestations, 59 differential diagnosis, 59 epidemiology, 58 etiopathogenesis, 59 oral signs and symptoms, 59 treatment recommendations, 59, 60 hyperparathyroidism, 54 clinical manifestations, 55 differential diagnosis, 55 epidemiology, 54 etiopathogenesis, 54 oral signs and symptoms, 55 treatment recommendations, 55 hyperpituitarism, 53 clinical manifestations, 53 epidemiology, 53 etiopathogenesis, 53 oral signs and symptoms, 53 treatment recommendations, 53 hyperthyroidism, 51 clinical manifestations, 51 differential diagnosis, 51 epidemiology, 51 etiopathogenesis, 51 oral signs and symptoms, 51 treatment recommendations, 51, 52 hypothyroidism, 48 clinical manifestations, 48 differential diagnosis, 50 epidemiology, 48 etiopathogenesis, 48 oral signs and symptoms, 48, 50 treatment recommendations, 50 porphyrias, 57 clinical manifestations, 57 differential diagnosis, 58 epidemiology, 57 etiopathogenesis, 57 Index oral signs and symptoms, 58 treatment recommendations, 58 Endocrinologic insufficiencies, 35 Eosinophilic granuloma, 32 Epidermolysis bullosa clinical manifestations, 132 differential diagnosis, 134 etiopathogenesis, 132 oral signs and symptoms, 132–134 treatment recommendations, 131, 132, 134 Epididymitis, 173 Epstein-Barr virus (EBV), 155, 156 Erosive glossitis, 67 Erosive oral lichen planus, 122 Erythema, 74 Erythema multiforme (EM) clinical manifestations, 124 differential diagnosis, 125 epidemiology, 124 etiopathogenesis, 124 malignancies, 124 oral presentations, 134 oral signs and symptoms, 124, 125 subtypes, and associated proteins and genes, 133 treatment recommendations, 125, 126 Erythema nodosum leprosum (ENL), 181 Esophageal disease, 106 Esophageal dysfunction, 103 Esophageal dysmotility, 101 Excimer laser, 137 Extrapulmonary TB, 174 F Familial adenomatous polyposis (FAP), 238, 239 Fat-soluble vitamin deficiencies BMS, 80, 81 niacin deficiency, 79, 80 recurrent aphthous stomatitis, 81, 82 vitamin A, 74, 75 vitamin D, 75–77 vitamin K, 77, 78 zinc deficiency, 78 Fever, 96 Fiery scarlet hue, 79–80 Filiform papillae, Focal epithelial hyperplasia (FEH) (Heck’s disease), 155 Folate, 36 Foliate papillae, Folic acid, 72 Follicular hyperkeratosis, 229 Fungal rhinosinusitis, 209 G Gardner’s syndrome, 17 clinical manifestations, 17 differential diagnosis, 17, 18 257 epidemiology, 17 etiopathogenesis, 17 oral signs and symptoms, 17 orofacial signs of, 17 treatment recommendations, 18 Gastroesophageal reflux disease (GERD), 20, 101 clinical manifestations, 20 differential diagnosis, 21 epidemiology, 20 etiopathogenesis, 20 oral signs and symptoms, 21 treatment recommendations, 21 Gastrointestinal disease, IBD, see Inflammatory bowel diseases (IBD) Gastrointestinal involvement, MCTD, 103 Gene therapy, 134 Genodermatoses, 228, 231 German measles, 161 Gingival bleeding, 34 Gingival hyperplasia, 26, 241 Gingivitis antibiotic therapy, 183 antibiotics, 183 bone resorption, 182 clinical manifestations, 183 diagnosis, 183 differential diagnosis, 183 epidemiology, 182 etiopathogenesis, 182 gingival discoloration, 183 plaque formation, 183 risk factors, 183 surgical reconstruction, 183 tooth destruction and premature loss, 183 treatment, 183 Glossitis, 67, 71, 79 Glucocorticoids, 99 Gluten, 15 Gonorrhea cervicitis in women, 172 contact with fomites, 172 differential diagnosis, 173 doxycycline, 173 epidemiology, 172 etiopathogenesis, 172 extragenital infection, 172 histopathological examination, 173 incidence, 172 penicillin allergic, 173 primary mode of transmission, 172 rectal and oropharyngeal mucosa, 172 severe pharyngitis, 173 symptomatology, 172 tissue damage, 172 treatment guidelines, 173 urethritis in men, 172 vertical transmission, 172 virulence factors, 172 Gonorrheal conjunctivitis, 172 Index 258 Gorlin syndrome See Basal cell nevus syndrome (BCNS) Gottron’s papules, 105 Granulocyte colony-stimulating factor (G-CSF), 29 Granuloma inguinale aminoglycoside, 184 antibiotics, 184 definitive diagnosis, 184 differential diagnosis, 184 disseminated disease, 184 epidemiology, 183, 184 etiopathogenesis, 184 genital and anal lesions, 184 histological examination, 184 HIV co-infection, 184 treatment, 184 ulcer progression, 184 Group A beta-hemolytic streptococcus (GAS), 176 Growth hormone secreting adenomas, 53 H Haematinic deficiencies, 20 Hand foot and mouth disease (HFMD), 151, 152 Hand-Schüller-Christian disease, 32 Hansen’s disease See Leprosy Hashimoto's disease, 48 Headaches, 93 Healing linear ‘slit-like’ ulcer, 12 Heck’s disease, 155 Heliotrope rash, 105 Hematinic deficiencies, malabsorptive causes, 20 Hematologic disorders amyloidosis clinical manifestations, 31 differential diagnosis, 31, 32 etiopathogenesis, 31 oral signs and symptoms, 31 treatment recommendations, 32 cyclic neutropenia clinical manifestation, 28 differential diagnosis, 29 etiopathogenesis, 28 oral signs and symptoms, 28, 29 treatment recommendations, 29 iron deficiency anemia, 37 clinical manifestations, 37 differential diagnosis, 38 epidemiology, 37 etiopathogenesis, 37 oral signs and symptoms, 38 treatment recommendations, 38 LCH clinical manifestations, 32 differential diagnosis, 33 etiopathogenesis, 32 oral signs and symptoms, 32, 33 treatment recommendations, 33 leukemia clinical manifestations, 26 differential diagnosis, 26 etiology, 25 etiopathogenesis, 25 oral signs and symptoms, 26 treatment, 26 lymphomas clinical manifestations, 27 differential diagnosis, 28 etiology, 26 etiopathogenesis, 26, 27 oral signs and symptoms, 27, 28 treatment recommendations, 28 megaloblastic anemia, 35 clinical manifestations, 36 differential diagnosis, 36 etiopathogenesis, 36 oral signs and symptoms, 36 treatment recommendations, 36 multiple myeloma clinical manifestations, 29, 30 differential diagnosis, 30 etiopathogenesis, 29 oral signs and symptoms, 30 treatment recommendations, 31 pernicious anemia, 36 clinical manifestations, 37 differential diagnosis, 37 epidemiology, 36 etiopathogenesis, 37 oral signs and symptoms, 37 treatment recommendations, 37 plasma cell dyscrasias, 29 platelet disorders, 33 clinical manifestations, 34 differential diagnosis, 34 epidemiology, 33 etiopathogenesis, 34 oral signs and symptoms, 34 treatment recommendations, 34, 35 polycythemia vera, 38 clinical manifestations, 39 differential diagnosis, 39 epidemiology, 38 etiopathogenesis, 38 oral signs and symptoms, 39 treatment recommendations, 39 red blood cell disorders, 33 SCD, 39 clinical manifestations, 39 differential diagnosis, 40 epidemiology, 39 etiopathogenesis, 39 oral signs and symptoms, 40 treatment recommendations, 40 thalassemias, 35 clinical manifestations, 35 epidemiology, 35 etiopathogenesis, 35 oral signs and symptoms, 35 treatment recommendations, 35 Hemochromatosis, 58 clinical features of, 59 clinical manifestations, 59 Index differential diagnosis, 59 epidemiology, 58 etiopathogenesis, 59 oral signs and symptoms, 59 treatment recommendations, 59, 60 Hemoglobin, 35 Hemorrhage, 74 Hemorrhagic stomatitis, 116 Hemostasis, 50 Hepcidin, 59 Hereditary hemorrhagic telangiectasia (HHT) brain abscess, 233 clinical manifestations, 233 differential diagnosis, 233 epidemiology, 232 etiopathogenesis, 232, 233 treatment, 233 vascular malformations, 233 Hermansky-Pudlak syndrome (HPS) bleeding tendency, 234 clinical manifestations, 234 differential diagnosis, 234 epidemiology, 233, 234 etiopathogenesis, 234 treatment, 234 Herpangina, 152, 153 Herpes Labialis, 149 Herpes simplex lesion, 149 Herpes simplex virus (HSV) infection HSV-1, 145 HSV-2, 145 Herpes zoster, 150, 151 Histoplasmosis, 201, 202, 205 amphotericin B, 202 clinical manifestations, 200, 201 definitive diagnosis, 202 differential diagnosis, 202 etiopathogenesis, 200 extra-pulmonary manifestations, 201 Histoplasma capsulatum, 200 itraconazole, 202 non-healing ulcerations, 202 therapies, 202 HIV, 160 HIV-associated salivary gland disease, 158 Hodgkin Lymphoma (HL), 26, 27 Human herpes virus (HHV-3), 150 Human immunodeficiency virus (HIV), 156–160 Human papillomavirus (HPV), 153–155 Hydrops fetalis, 35 Hydroxychloroquine, 95, 104, 107 Hyperkeratosis on buccal mucosa, 237 Hypermobility of temporomandibular joint (TMJ), 243 Hyperparathyroidism, 54 clinical features of, 55 clinical manifestations, 55 differential diagnosis, 55 epidemiology, 54 etiopathogenesis, 54 oral signs and symptoms, 55 259 treatment recommendations, 55 Hyperpituitarism, 53 clinical features of, 54 clinical manifestations, 53 epidemiology, 53 etiopathogenesis, 53 oral signs and symptoms, 53 treatment recommendations, 53 Hypersegmented neutrophils, 71 Hyperthyroidism, 51 clinical features of, 52 clinical manifestations, 51 differential diagnosis, 51 epidemiology, 51 etiopathogenesis, 51 oral signs and symptoms, 51 treatment recommendations, 51, 52 Hypothyroidism, 48 clinical features of, 49 clinical manifestations, 48 differential diagnosis, 50 epidemiology, 48 etiopathogenesis, 48 oral signs and symptoms, 48, 50 treatment recommendations, 50 I Iatrogenic candidemia, 194 Idiopathic inflammatory myopathies (IIM), 105 Immune thrombocytopenic purpura (ITP), 158 Immunomodulators, 119 Immunosuppressants, 12 Incontinentia pigmenti (IP), 227, 228 clinical manifestations, 227, 228 dental decay, 228 diagnostic criteria, 228 differential diagnosis, 228 epidemiology, 227 etiopathogenesis, 227 loss of dentition, 228 oral and dental manifestations, 228 salivary secretion, 228 treatment, 228 Indirect immunofluorescence testing (IIF), 114 MMP, 120 Infectious mononucleosis, 155, 156 Inflamed, bleeding gingiva, 158 Inflammatory bowel diseases (IBD), Celiac disease, 15 environmental, 15–17 epidemiology, 15 etiopathogenesis, 15 genetic, 15 Crohn’s disease and OFG, epidemiology, etiopathogenesis, genetic, hypersensitivity/allergy, 10 Index 260 Inflammatory bowel diseases (IBD) (cont.) inflammatory/immunological, 10–13 microbiological/infection, 10 Gardner’s syndrome, 17 clinical manifestations, 17 differential diagnosis, 17, 18 epidemiology, 17 etiopathogenesis, 17 oral signs and symptoms, 17 treatment recommendations, 18 GERD, 20 clinical manifestations, 20 differential diagnosis, 21 epidemiology, 20 etiopathogenesis, 20 oral signs and symptoms, 21 treatment recommendations, 21 malabsorption, 19 clinical manifestations, 19 differential diagnosis, 20 oral signs and symptoms, 20 treatment recommendations, 20 PJS, 18 clinical manifestations, 18 diagnosis, 18 differential diagnosis, 19 epidemiology, 18 etiopathogenesis, 18 oral signs and symptoms, 18, 19 treatment recommendations, 19 Pyostomatitis vegetans, 14 clinical manifestations, 14 differential diagnosis, 14 epidemiology, 14 etiopathogenesis, 14 oral signs and symptoms, 14 treatment recommendations, 14, 15 ulcerative colitis, 13 clinical manifestations, 13 differential diagnosis, 13 epidemiology, 13 etiopathogenesis, 13 oral signs and symptoms, 13 treatment recommendations, 13 Inherited patterned lentiginosis, 236 Interdental papilla, Intertriginous candidiasis, 194 Intestinal polyposis, 18 Intraoral linear IgA bullous dermatosis, 131 Intrinsic factor (IF), 36 Iron deficiency anemia (IDA), 37, 63, 66, 67, 81 clinical manifestations, 37 differential diagnosis, 38 epidemiology, 37 etiopathogenesis, 37 oral signs and symptoms, 38 treatment recommendations, 38 Irritant dermatitis, 194 Isoniazid, 70 J Jaundice, 40 Junctional epithelium, 132, 133 Juvenile rheumatoid arthritis, 177 K Kaposi’s sarcoma (KS), 159, 160 Kawasaki disease, 177 Keratinization, Keratoconjunctivitis sicca, 92, 97 Keratocyst odontogenic tumors (KCOT), 240 Kindler syndrome, 132, 134 Koebner phenomenon, 137 Korsakoff’s psychosis, 68 L Langerhans cell histiocytosis (LCH) clinical manifestations, 32 differential diagnosis, 33 etiopathogenesis, 32 oral signs and symptoms, 32, 33 treatment recommendations, 33 Laryngeal cryptococcosis, 211 Laugier-Hunziker syndrome, 236 Leishmaniasis, 205 antimonial treatment, 217 clinical manifestations, 214 definitive diagnosis, 216 differential diagnosis, 216 epidemiology, 214, 215 etiopathogenesis, 214 mucosal involvement, 214 oral miltefosine, 216 species identification, 216 therapy in, 216 tissue biopsy/aspiration material, 216 treatments, 216 Lentigines, 19, 236 Leonine facies, 181 Leprosy, 181 bacilli isolation, 182 classification system, 180 clinical manifestations, 181 diagnosis, 182 differential diagnosis, 182 early detection and treatment, 180 epidemiology, 180 etiopathogenesis, 180 multidrug therapy, 182 oral manifestations, 181 peripheral nerve enlargement, 182 treatment, 182 Lesch-Nyhan syndrome (LNS) clinical manifestations, 235 differential diagnosis, 235 epidemiology, 234 etiopathogenesis, 234 Index prevalence, 234 primary dentition, 235 psychologic therapy and uric acid reduction, 235 treatment, 235 Letterer-Siwe disease, 32 Leukemia clinical manifestations, 26 differential diagnosis, 26 etiology, 25 etiopathogenesis, 25 oral signs and symptoms, 26 treatment, 26 Leukokeratosis, 230 Leukopenia, 103 Leukoplakia, 231 Limited cutaneous systemic sclerosis (LCSS), 101 Linear IgA bullous dermatosis (LABD), 131 clinical manifestations, 130 differential diagnosis, 131 epidemiology, 130 etiopathogenesis, 130 oral signs and symptoms, 130, 131 treatment recommendations, 131 Lingual papillae, Localized amyloidosis, 31 Lower labial mucosa, ulceration of, 98 Lumbar lordosis, 106 Lupus nephritis, 97 Lymphomas clinical manifestations, 27 differential diagnosis, 28 etiology, 26 etiopathogenesis, 26, 27 oral signs and symptoms, 27 treatment recommendations, 28 M Macroglossia, 31, 50, 56, 57 Magenta tongue, 69 Malabsorption, 19 clinical manifestations, 19 differential diagnosis, 20 oral signs and symptoms, 20 treatment recommendations, 20 Measles, 162, 163 Median sulcus, Megaloblastic anemia, 35, 72 clinical manifestations, 36 differential diagnosis, 36 etiopathogenesis, 36 oral signs and symptoms, 36 treatment recommendations, 36 Megaloblasts, 71 Meischer’s cheilitis, Melanocytes, Melanotic macules, 18 Merkel cells, Methotrexate, 104 261 Microbiome, 5, Microcytic anemia, 33 Milk, 69 Mixed connective tissue disease (MCTD), 98 clinical manifestations, 102, 103 differential diagnosis, 104 epidemiology, 102 etiopathogenesis, 102 oral signs and symptoms, 104 treatment, 104 Molar teeth, Molluscum contagiosum lesions, 153 Montenegro’s skin test, 216 Motor deficits, 71 Mucocutaneous candidiasis, 197 Mucocutaneous leishmaniasis, 214–216 Mucocutaneous oral pigmentation, differential diagnosis, 19 Mucogingival line, Mucormycosis, 203 antifungal therapy, 204 clinical manifestations, 203 clinical recognition, 203 diagnosis, 203 epidemiology, 202 etiopathogenesis, 202 incidence, 202 inflammatory response, 203 predisposing factors, 203 salvage therapy with deferasirox, 204 treatment strategy, 204 Mucosal pallor, 67 Mucosal petechia, 74 Mucosal telangiectasias, 233 Mucosal ulcerations, 98, 157 Mucosa, oral cavity, 1, Mucous membrane pemphigoid (MMP), 120 clinical manifestations, 119 differential diagnosis, 121 epidemiology, 119 etiopathogenesis, 119 oral signs and symptoms, 119–121 treatment recommendations, 121 Mucous membranes, 63 Multiple myeloma (MM), 30 clinical manifestations, 29, 30 differential diagnosis, 30 etiopathogenesis, 29 oral signs and symptoms, 30 treatment recommendations, 31 Multiple petechiae, 34 Mumps, 162 Myalgias, 92 Myelopathy, 36 N National Foundation for Ectodermal Dysplasias, 228 Neonatal candidiasis, 194 Index 262 Neutrophilic microabscesses, 129 Neutrophils, 28 Niacin deficiency, 79, 80 Nitrate reducing bacteria, Non-Hodgkin Lymphoma (NHL), 26, 27 Non-steroidal anti-inflammatory drugs (NSAIDs), 99 Non-venereal endemic treponemal infection, see Endemic treponemal diseases Nucleic acid amplification tests (NAATs), 173 Nutritional disease clinical and oral manifestations, workup and treatment, 64–66 fat-soluble vitamin deficiencies BMS, 80, 81 niacin deficiency, 79, 80 recurrent aphthous stomatitis, 81, 82 vitamin A, 74, 75 vitamin D, 75–77 vitamin K, 77, 78 zinc deficiency, 78 iron deficiency anemia, 63, 66, 67 water-soluble vitamin deficiencies biotin, 73 cyanocobalamin, 70–72 folic acid, 72 pyridoxine, 69, 70 riboflavin, 69 thiamine, 67, 68 vitamin B deficiencies, 67 vitamin C, 73, 74 O Odontogenic keratocysts, 240 Oligodontia with agenesis, 237 Oral actinomycosis, 198 Oral burning sensation, 20 Oral candidiasis, 197 Oral cavity, dentition, microbiome, 5, mucosa, 1, oral examination, principles of, inspection, palpation, physiology, 4, regions, salivary glands, tongue, 2, Oral contraceptives (OCP), 70 Oral donovanosis, 184 Oral gonorrhea, 173 Oral hairy leukoplakia (OHL), 157 Oral leukokeratosis, 229 Oral lichen planus (OLP), 123 clinical manifestations, 122 differential diagnosis, 123 epidemiology, 121 etiopathogenesis, 121, 122 signs and symptoms, 122, 123 treatment recommendations, 123, 124 Oral pemphigus vulgaris clinical manifestations, 113 differential diagnosis, 114 epidemiology, 113 etiopathogenesis, 113 signs and symptoms, 114 treatment recommendations, 114, 115 Oral phytonadione, 78 Oral squamous cell carcinoma, 46 Oral thrush in newborns, 195 Oral verruca vulgaris, 154 Orofacial granulomatosis (OFG), buccal mucosa, cobblestoning of, 11 clinical features, 11 epidemiology, etiopathogenesis, genetic, gingivitis and mucosal tags, 11 healing linear ‘slit-like’ ulcer, 12 hypersensitivity/allergy, 10 inflammatory/immunological, 10 clinical manifestations, 10 differential diagnosis, 11, 12 oral signs and symptoms, 10, 11 treatment, 12, 13 management/treatment options, 12 microbiological/infection, 10 swelling, 10 tongue, ventral surfaces of, 11 Orofacial swelling, differential diagnosis, 12 Oropharyngeal ulcers, 152 Orthopedic/orthodontic repositioning, teeth malpositioning and bone expansion, 228 Osler-Weber-Rendu syndrome, see Hereditary hemorrhagic telangiectasia (HHT) Osteogenesis imperfecta (OI), 239, 240 Osteomas, 17, 18 Osteoporosis, 51 P Pachyonychia congenital (PC), 229 clinical genetic test, 229 clinical manifestations, 229 differential diagnosis, 230 epidemiology, 228, 229 etiopathogenesis, 229 oral leukokeratosis, 230 oral rehabilitation, 230 systemic retinoids, 230 treatment, 230 Papillon-Lefevre syndrome (PLS), 246 Paracoccidioidomycosis, 205–207 amphotericin B desoxycholate, 206 clinical manifestations, 204, 205 clinical prognosis, 207 diagnosis, 205 differential diagnosis, 205 Index epidemiology, 204 etiopathogenesis, 204 serological testing, 206, 208 tissue infiltration, 205 treatment, 206–208 trimethoprim-sulfamethoxazole, 206 Paraneoplastic autoimmune multiorgan syndrome (PAMS), 116 clinical manifestations, 116 differential diagnosis, 117 epidemiology, 115 etiopathogenesis, 115 oral signs and symptoms, 116, 117 treatment recommendations, 117 Paraneoplastic pemphigus (PNP), 116 clinical manifestations, 116 differential diagnosis, 117 epidemiology, 115 etiopathogenesis, 115 oral signs and symptoms, 116, 117 treatment recommendations, 117 Parathyroid hormone (PTH), 54 Parenteral nutrition, 194 Parotitis, 162 Pellagra, 80 Pelvic inflammatory disease, 173 Pemphigus vulgaris, 114 Perineal dermatitis, 69 Periodontal disease, 183 Periodontitis, 29 Pernicious anemia (PA), 19, 36 clinical manifestations, 37 differential diagnosis, 37 epidemiology, 36 etiopathogenesis, 37 oral signs and symptoms, 37 treatment recommendations, 37 Petechiae on soft palate and oral pharynx, 156 Peutz-Jeghers syndrome (PJS), 18, 235, 236 cancer surveillance, 236 clinical manifestations, 18, 235, 236 diagnosis, 18 differential diagnosis, 19, 236 epidemiology, 18, 235 etiopathogenesis, 18, 235 oral signs and symptoms, 18, 19 periorificial distribution, 236 pigmented macules, 236 prevalence, 235 treatment, 236 treatment recommendations, 19 Phlebotomy, 39 Pilocarpine, 95 Pinta, 218 Pituitary adenomas, 53 Plaque-induced gingivitis, 183 Plasma cell dyscrasias, 29 Platelet disorders, 33 clinical manifestations, 34 differential diagnosis, 34 263 epidemiology, 33 etiopathogenesis, 34 oral signs and symptoms, 34 treatment recommendations, 34, 35 Pleuritis, 97 Plummer-Vinson syndrome (PVS), 67 Poikiloderma, 106 Poikiloderma and atrophy of skin and lips, 232 Polyarthralgia, 103 Polycythemia vera (PV), 38 clinical manifestations, 39 differential diagnosis, 39 epidemiology, 38 etiopathogenesis, 38 oral signs and symptoms, 39 treatment recommendations, 39 Polymorphonuclear leukocytes (PMNs), 28 Polymyositis (PM), 99 Polypectomy, 19 Porphyria cutanea tarda, 57 Porphyrias, 57 clinical features of, 58 clinical manifestations, 57 differential diagnosis, 58 epidemiology, 57 etiopathogenesis, 57 oral signs and symptoms, 58 treatment recommendations, 58 Posterior oropharnynx, Prednisone, 107 Primary acute herpetic gingivostomatitis, 149 Primary cutaneous cryptococcosis, 211 Primary herpetic gingivostomatitis (PHGS), 145, 149, 150 Primary pulmonary coccidioidomycosis, 213 Progressive nephropathy, 56 Prophylactic antiviral therapy, 126 Pseudomembranous candidiasis, 195–197 Pseudothrombocytopenia, 33 Pseudo-vitamin D deficiency rickets (PDDR), 76 Pseudoxanthoma elasticum (PXE), 237 ABCC6 gene, 236 clinical manifestations, 236 differential diagnosis, 237 differential treatment, 237 epidemiology, 236 etiopathogenesis, 236 meticulous oral hygiene, 237 mucosal lesions, 237 Pulmonary cryptococcosis, 211 Pulmonary disease, 106 Pulmonary TB, 174 Punctate telangiectasias, 233 Pyostomatitis vegetans (PV), 11 clinical manifestations, 14 differential diagnosis, 14 epidemiology, 14 etiopathogenesis, 14 oral signs and symptoms, 14 treatment recommendations, 14, 15 Pyridoxine deficiency, 70 264 R Rachitic rosary, 76 RAPADILINO syndrome, 232 Raynaud’s phenomenon, 100, 101, 103 Recurrent aphthous stomatitis (RAS), 29, 68, 81, 82 Recurrent gingivitis, 29 Recurrent herpes labialis (RHL), 149 Recurrent intraoral herpes (RIH), 149 Red blood cell disorders, 33 Renal involvement, MCTD, 103 Reticular oral lichen planus, 122 Rheumatic fever, 178 Rheumatoid arthritis (RA), 98 Riboflavin deficiency, 69 Ridley-Jopling classification, leprosy, 180 Rituximab, 96, 115, 119, 121 Root, 3, Rothmund-Thompson syndrome (RTS), 232 clinical manifestations, 232 dental malformations, 232 differential diagnosis, 232 epidemiology, 231 etiopathogenesis, 231 treatment, 232 Rubella, 161 S Salivary gland disease with xerostomia, 158, 159 Salivary glands, oral cavity, Scarlet fever, 177 allergic drug reaction, 177 antistreptolysin O titers, 177 circumoral pallor, 176 clinical signs, 177 differential diagnosis, 177 enlarged uvula and tonsils with erythema, 177 epidemiology, 176 etiopathogenesis, 176 incidence, 176 by Pastia’s lines, 176 penicillin, 177 treatment, 177 Sclerosis, 101 Scrotal dermatitis, 69 Scurvy, 73, 74 Seborrheic dermatitis-like rash, 69 Serositis, 99 Severe congenital neutropenia (SCN), 28 Severity of Illness Score for Toxic Epidermal Necrolysis (SCORTEN), 127 Sickle cell disease (SCD) clinical manifestations, 39 differential diagnosis, 40 epidemiology, 39 etiopathogenesis, 39 oral signs and symptoms, 40 treatment recommendations, 40 Sickle cell trait (SCT), 39 Sjögren’s syndrome (SS) Index clinical manifestations, 92 differential diagnosis, 94, 95 epidemiology, 91–93 etiopathogenesis, 92 non-visceral manifestations, 92, 93 oral signs and symptoms, 93, 94 treatment, 95, 96 visceral manifestations, 93 Small aphthous ulcerations, 70 Splenic sequestration, 34 Squamous cell carcinoma, 205 Staphylococcal scalded skin syndrome (SSSS) bullous variants, 180 clindamycin-resistant strains, 180 clinical manifestations, 179, 180 diagnosis, 180 differential diagnosis, 180 epidemiology, 179 etiopathogenesis, 179 hyperemia, 180 oral involvement, 180 oxacillin-sensitive, 180 penicillinase-resistant antibiotics, 180 temperature regulation and volume resuscitation, 180 vancomycin, 180 Stevens-Johnson syndrome clinical manifestations, 126 differential diagnosis, 127 epidemiology, 126 etiopathogenesis, 126 oral signs and symptoms, 126, 127 treatment recommendations, 127, 128 String of pearls, 131 Subacute/chronic mycosis, 208 Sublingual gland, Submandibular ducts, 12 Submandibular gland, Sulcular gingiva lines, Sulcus terminalis, Suprabasal acantholysis, 116 Syphilis antiretroviral therapy, 169 blistering mucositis, 170 cardiovascular involvement, 170 CDC guidelines, 172 clinical manifestations, 170 congenital, 171 dark field microscopy/serologic testing, 169 diagnosis, 170 differential diagnosis, 171, 172 epidemiology, 169 etiopathogenesis, 169, 170 gummas, 170 histopathological examination, 171 necrosis of dorsum of tongue, 170 nonspecific ulcers, 170 penicillin G, 172 primary, 170 safety, sex practices, 169 secondary, 170, 171 Index sexual intercourse, 169 skin manifestations, 170 stages, 170 tertiary, 171 treatment, 172 Treponema pallidum, 169 Syphilitic perlèche, 170 Systemic amyloidosis, 31 Systemic candidiasis, 194 Systemic lupus erythematosus (SLE), 96 clinical manifestations, 96, 97 differential diagnosis, 98, 99 etiopathogenesis, 96 oral signs and symptoms, 97, 98 treatment, 99 Systemic sclerosis (SSc), 99, 100 clinical manifestations, 100, 101 differential diagnosis, 101, 102 etiopathogenesis, 100 oral signs and symptoms, 101 treatment, 102 Systemic vasculitis, 95 T Taste buds, Taurodontism, 53 Telangiectasias, 231 of lip, 233 Tertiary hyperparathyroidism, 54 Thalassemias, 35 clinical manifestations, 35 epidemiology, 35 etiopathogenesis, 35 oral signs and symptoms, 35 treatment recommendations, 35 Thiamine, 67, 68 Thrombocytopenia, 33 Thyroid hormone (TH) production, 48 Tissue transglutaminase (tTG) antibody test, 15 Tongue, oral cavity, 2, Toxic epidermal necrolysis clinical manifestations, 126 differential diagnosis, 127 epidemiology, 126 etiopathogenesis, 126 oral signs and symptoms, 126, 127 treatment recommendations, 127, 128 Trendelenburg gait, 106 Trigeminal neuralgia, 103 Tuberculosis (TB) clinical manifestations, 174 differential diagnosis, 174 of dorsal tongue, 174 epidemiology, 173 etiopathogenesis, 173, 174 inhalation of droplets, 173 intact oral and pharyngeal mucosa, 174 primary progressive, 174 secondary lesions, 174 265 treatment, 174, 175 Tuberous sclerosis complex (TSC), 241, 242 Tularemia, 185 aminoglycosides, 186 clinical manifestations, 185 definitive diagnosis, 186 dermatologic signs, 185 direct fluorescent antibody, 186 epidemiology, 184, 185 etiopathogenesis, 185 features of, 186 fluoroquinolones, 186 oral manifestations, 185 parapharyngeal abscess formation, 185 PCR methods, 186 tetracyclines, 186 zoonotic infection, 186 Type lepra reaction, 181–182 Type VII collagen, 132 U Ulcerative colitis (UC) clinical manifestations, 13 differential diagnosis, 13 epidemiology, 13 etiopathogenesis, 13 oral manifestations, 13 oral signs and symptoms, 13 treatment recommendations, 13 Ulcerative lesions on soft palate and oral pharynx, 153 Ultraviolet light (UVB), 76 Undifferentiated connective tissue disease (UCTD), 99 Uroporphyrinogen decarboxylase (UROD), 57 V Varicella (chickenpox), 151 Varicella-zoster virus (VZV), 150, 151 Vermillion border, 18 Verruca vulgaris, 154 Verrucous cutaneous blastomycosis, 208 Vesiculobullous and autoimmune diseases ABH clinical manifestations, 135 differential diagnosis, 135 epidemiology, 135 etiopathogenesis, 135 oral signs and symptoms, 135 treatment recommendations, 135 bullous pemphigoid clinical manifestations, 118 differential diagnosis, 118, 119 epidemiology, 117 etiopathogenesis, 118 oral signs and symptoms, 118 treatment recommendations, 119 dermatitis herpetiformis clinical manifestations, 129 differential diagnosis, 129 Index 266 Vesiculobullous and autoimmune diseases (cont.) epidemiology, 128 etiopathogenesis, 128, 129 oral signs and symptoms, 129 treatment recommendations, 129, 130 epidermolysis bullosa clinical manifestations, 132 differential diagnosis, 134 etiopathogenesis, 132 oral signs and symptoms, 132–134 treatment recommendations, 131, 132, 134 erythema multiforme clinical manifestations, 124 differential diagnosis, 125 epidemiology, 124 etiopathogenesis, 124 oral signs and symptoms, 124, 125 treatment recommendations, 125, 126 LABD clinical manifestations, 130 differential diagnosis, 131 epidemiology, 130 etiopathogenesis, 130 oral signs and symptoms, 130, 131 treatment recommendations, 131 MMP clinical manifestations, 119 differential diagnosis, 121 epidemiology, 119 etiopathogenesis, 119 oral signs and symptoms, 119–121 treatment recommendations, 121 OLP clinical manifestations, 122 differential diagnosis, 123 epidemiology, 121 etiopathogenesis, 121, 122 signs and symptoms, 122, 123 treatment recommendations, 123, 124 oral pemphigus vulgaris clinical manifestations, 113 differential diagnosis, 114 epidemiology, 113 etiopathogenesis, 113 signs and symptoms, 114 treatment recommendations, 114, 115 PNP/PAMS clinical manifestations, 116 differential diagnosis, 117 epidemiology, 115 etiopathogenesis, 115, 116 oral signs and symptoms, 116, 117 treatment recommendations, 117 SJS/TEN clinical manifestations, 126 differential diagnosis, 127 epidemiology, 126 etiopathogenesis, 126 oral signs and symptoms, 126, 127 treatment recommendations, 127, 128 vitiligo clinical manifestations, 136 differential diagnosis, 137 epidemiology, 135 etiopathogenesis, 136 oral signs and symptoms, 136, 137 treatment recommendations, 137 Vincent infection and trench mouth, see Acute necrotizing ulcerative gingivitis (ANUG) Viral diseases, 146–148 clinical manifestations and implications, 145 Vitamin A, 74, 75 Vitamin A deficiency (VAD), 74 Vitamin B deficiencies, 67 Vitamin B1, see Thiamine Vitamin B12, 36 See Cyanocobalamin Vitamin B2, see Riboflavin deficiency Vitamin B3, see Niacin deficiency Vitamin B6, see Pyridoxine Vitamin B6 deficiency, see Pyridoxine deficiency Vitamin B12 deficiency, 36, 71 Vitamin C deficiency, 73, 74 Vitamin D, 75–77 Vitamin D-dependent rickets (VDDR), 77 Vitamin D-resistant rickets (VDRR), 77 Vitamin K deficiency (VKD), 77, 78 Vitiligo, 136 clinical manifestations, 136 differential diagnosis, 137 epidemiology, 135 etiopathogenesis, 136 oral signs and symptoms, 136, 137 treatment recommendations, 137 W Waldeyer’s ring, 27, 28 Water-soluble vitamin deficiencies biotin, 73 cyanocobalamin, 70–72 folic acid, 72 pyridoxine, 69, 70 riboflavin, 69 thiamine, 67, 68 vitamin B deficiencies, 67 vitamin C, 73, 74 Wernicke-Korsakoff syndrome (WKS), 68 Wernicke’s encephalopathy (WE), 68 Wet beriberi, 68 Wood's lamp, 136 X Xeroderma pigmentosum (XP), 245, 246 Xerostomia, 75, 93 Y Yaws, 217, 218 Z Zinc deficiency, 73, 78, 79 .. .Oral Signs of Systemic Disease www.ajlobby.com Nasim Fazel Editor Oral Signs of Systemic Disease www.ajlobby.com Editor Nasim Fazel Department of Dermatology University of California,... goal of learning the fundamentals of oral mucosal disease Oral Signs of Systemic Disease provides descriptive clinical and oral manifestations and differential diagnoses, including principles of. .. disease Roy S. Rogers Professor of Dermatology Mayo Clinic College of Medicine Scottsdale, AZ, USA vii www.ajlobby.com Preface The conception of Oral Signs of Systemic Disease came about with