About the Book
The book covers all aspects of leprosy, including its history, epidemiology, immunogenetics, bacteriology, immunology, pathology of Mycobacterium leprae, mechanisms of nerve damage, disease classification, clinical and laboratory diagnosis, differential diagnosis, chemotherapy, lepra reactions and their management and National Leprosy Eradication Programme.
Includes chapters related to basics of history taking and clinical examination, techniques of nerve examination, ocular leprosy, vaccines (immunoprophylaxis and immunotherapy), chemoprophylaxis, deformities and their management, recording and assessment of nerve involvement and experimental leprosy.
Covers special situations, viz. leprosy in children, leprosy and pregnancy, leprosy associated with HIV and issues related to community-based rehabilitation, psychosocial aspects, stigma, and migration in leprosy.
Presents clear and comprehensive information that help in diagnosing leprosy in low endemic situations.
Discusses problems of drug resistance, challenges and scenario beyond 2020.
Highlights the role of NGOs in the eradication program, and the case histories for achieving the control over leprosy.
Contains the most up-to-date and comprehensive material from 84 acclaimed experts of national and international repute based on the wealth of their expertise.
Gives an idea of how the disease looks in different shades of skin color through photo gallery.
Improves the diagnostic accuracy with the help of nearly 450 figures that capture the presentation of leprosy in practice.
Enhances clinical and therapeutic knowledge with the latest guidance on diagnosis and other rapidly changing research areas in leprosy.
Ideal book for dermatologists, leprologists, internists, epidemiologists, social scientists, laboratory and research persons, teachers and students, national and international NGOs and the Health Ministers. About the Author
Bhushan Kumar MD FRCP (Edin) FRCP (London)
Hemanta Kumar Kar MD MAMS
Table of Contents:
Contents
Section 1: The Disease History and Epidemiology
1. History of Leprosy in India: A Historical Overview from Antiquity to the Introduction of Mdt 3
KV Desikan, Shubhada S Pandya
Historic Authentication of Leprosy in India 3; The Evolution of Modern Leprology 3; Pathology 8
The Era of Chaulmoogra/Hydnocarpus Oil Therapy in India 9; Leprosy as A Public Health Problem 11
Icbelra in Madras Province 13; The Bhore Committee 14; Legislation 15; The Era of New Drugs 17
Developments at Independence 17; Indian Social Initiatives and Gandhiji’s Support 19
The Indian Association of Leprologists 24; Surgeons as Trail Blazers 29
2. Epidemiology of Leprosy 33
PL Joshi
Leprosy and International Disease Classification 34; Definition of Leprosy 34
Geographical Distribution and Prevalence 34; Agent 37; Host Factors 37
Environmental Factors 39; Disability 39; Transmission 39; Incubation Period 40
Diagnosis 40; Classification of Leprosy 41; Epidemiological Indicators for Monitoring 41
Relapses 43
3. Global Leprosy Situation: Historical Perspective, Achievements, Challenges and Future Steps 45
Sumana Barua
Leprosy: A brief overview 45; History of the Disease 46
History of Treatment of Leprosy 46; Global Leprosy Situation 48
Evolution of Global Leprosy Strategies: To Ensure Relevant Technical Assistance from WHO 50
Measure for Disease Burden due to Leprosy 51
Remaining Challenges in Further Reducing the Disease Burden 51; International Leprosy Summit 52
Need to Change the Image of Leprosy in the Community 53
Policy Guidance by WHO for Member States National Leprosy Programs 53
Key Messages on Leprosy 54; Further Steps in Defining Global Leprosy Strategy 2016–2020 55
List of Publications on Leprosy by WHO Global Leprosy Programme 55
Technical Report Series: WHO Expert Committee on Leprosy 55
WHO Technical Advisory Group on Leprosy Control: Meeting Reports 55
Global Program Managers’ Meeting Reports 55
Sentinel Surveillance for Drug Resistance in Leprosy: Meeting Reports 55; Training Manuals 55
Weekly Epidemiological Record (WER) 56
4. Changing National Scenario, National Leprosy Control Programme, National Leprosy
Eradication Programme, and New Paradigms of Leprosy Control 59
CM Agrawal, BN Barkakaty
Preface 59; Vision 59; Mission 59; Progress Through Different Stages 59
Editors’ Views 68
Section 2: Basic Scientific Considerations and Pathology
5. Immunogenetics of Leprosy 73
Rajni Rani
Genes and Proteins of the Major Histocompatibility Complex 74
Nomenclature of the Human Leukocyte Antigen 76; Human Leukocyte Antigen and Leprosy 77
Other Genes Involved in Susceptibility to Leprosy 78; Genome Wide Association Studies 81
xviii Ial Textbook of Leprosy
6. Bacteriology of Leprosy 90
B Sekar
Mycobacteria 90; Pyridine Extraction 93; Clinical Spectrum of Leprosy 94
Immunological Properties of M. leprae 94: Pathogenesis of M. leprae 94
Metabolism of M. leprae 94; Biological Properties of M. leprae 95
Experimental Animal Models for M. leprae 97; Other Experimental Animals 98
Drug Resistance 98; Laboratory Diagnosis of Leprosy 99
7. Immunological Aspects 105
Indira Nath, Mehervani Chaduvula
General Immune Responses 105; Immunological Features of leprosy 106
Leprosy Reactions 111; Immunopathology of Dermal Lesions 112
Nerve Damage in Leprosy: The Immune Mechanisms 113; Immunogenetics of Leprosy 115
8. Biochemical Aspects of Leprosy 119
Krishnamurthy Venkatesan, Nirmala Deo
Mycobacterium leprae 119; Host-M. leprae Interactions 122
Hematological, Serological and Biochemical Changes in Patients with Leprosy 123
Serum Enzymes 127
9. Pathological Aspects of Leprosy 132
Dullobho Porichha, Mohan Natrajan
Need for Histopathology 132; Histology of Various Types of Leprosy 133
Regressing Lesions 141; Reactions in Leprosy 142; Relapse in Leprosy 145
Involvement of Other Organs 145
10. Structure, Electrophysiological and Ultrasonographic Studies of Peripheral Nerve 152
Sujai K Suneetha, P Narasimha Rao, Suman Jain
Structure and Function of Peripheral Nerve 152; Structure of Nerve 153
Types of Nerves 154; Nerve Supply to the Skin 155; Nerve Endings 155
Damage to Peripheral Nerves 156; Mycobacterium leprae and Neural Predilection 156
Electrophysiological Studies on Peripheral Nerves in Leprosy 157
Electrophysiological Study by Emg 157; Application of Electrophysiological Studies in Leprosy 159
High Resolution Ultrasonography as an Imaging Tool in Leprosy 160
High Resolution Ultrasonography 161
11. Pathomechanisms of Nerve Damage 170
Vanaja Prabhakar Shetty
General Features of Nerve Damage in Leprosy 170; Entry of M. leprae into the Nerves 172
Infection of Schwann Cells by M. leprae 173
Type of Degeneration and Regeneration Seen in Leprous Nerves 174
Immunological Aspects of Nerve Damage 174; Autoimmune Type of Damage 176
Role of Mycobacterial Antigens in Nerve Damage 176
Role of Inflammatory Cytokines in Nerve Damage 176; Insidious Nerve Damage 177
Neuropathic Pain 177; Leprosy Reactions and Nerve Damage 177
12. Naturally Occurring Leprosy: Mycobacterium leprae and Other Environmental Mycobacteria in Nature 182
UD Gupta, Partha Sarathi Mohanty
Molecular Biology of Mycobacterium leprae 182; Transmission 183
13. Experimental Leprosy: Contributions of Animal Models to Leprosy Research 186
Linda B Adams, Maria T Pena, Rahul Sharma, Ramanuj Lahiri, Richard W Truman
Mouse Model 186; Armadillo Model 192
Section 3: Clinical, Laboratory Diagnosis and Differential Diagnosis
14. History Taking and Clinical Examination 207
Aparna Palit, Ragunatha S, Arun C Inamadar
History Taking 207; Clinical Examination 211
Contents xix
15. Case Definition and Clinical Types of Leprosy 236
Bhushan Kumar, Sunil Dogra
Case Definition 236; Classification of Disease 238; Clinical Features 239; Rare Variants of Leprosy 248
Nerve Involvement 252; Systemic Involvement 252
16. Classification 254
Radhey Shyam Misra, Joginder Kumar Kataria
Need for Classification 255; Classification Criteria 255
Madrid Classification 256; Indian Classification 256; New Ial Classification 257
Ridley-Jopling Classification 257
World Health Organization Classification (1988) for Leprosy Control Programs 258
World Health Organization Classification (1998) 259
Classification Presently used in India for Treatment Purposes (who Classification as Modified Under Nlep) 259
Miscellaneous Criteria in use for Classification into Pb and Mb 259
Classification: Present Global Perspective 260; Correlation among Various Classifications 260
17. Methods of Nerve Examination 263
BK Girdhar
Nerve Examination 263; Palpation of Nerve Trunks 264; Palpation of Cutaneous Nerves 265
18. Histoid Leprosy 268
Virendra N Sehgal
Historical Landmarks 268; Definition 268; Epidemiology 268; Clinical Features 269
Bacteriology 269; Histopathology 271; Tuberculoid Contamination 272; Ultrastructure 272
Immunoprofile 273; Diagnosis 273; Differential Diagnosis 273; Treatment 274
Significance of Histoid Lepromas 275
19. Laboratory Diagnosis 278
Joyce Ponnaiya
Slit-Skin Smear Examination 278; Slit-Skin Smear—its Role and Indications 278
Bacterial Index 280; Morphological Index 281; Nasal Mucosal Smears 281
Skin Biopsy 282; Fixatives 283; Hematoxylin-Eosin Stain 283
Job-Chacko Modification of Fite-Faraco Stain for M. leprae 284
Gomori-Grocott Methenamine Silver Stain 284; Fluorescent Microscopy to Detect M. leprae 285
Immunochemical Staining to Demonstrate Mycobacterial Antigens 285; S-100 Stain for Schwann Cells 285
Nerve Biopsy 285; Histamine Test 286; The Sweat Test 286; Cytological Diagnosis of Leprosy 287
Fine Needle Aspiration Cytology for Cutaneous Lesions of Leprosy 287
20. Serological and Molecular Diagnosis of Leprosy 290
U Sengupta, VM Katoch
Serodiagnosis 290; Future Scope for Immunoassays 292
Genome of M. leprae 292; Detection of Drug Resistance 294; Future Perspective 294
21. Differential Diagnosis of Dermatological Disorders in Relation to Leprosy 298
NL Sharma, Vikram Mahajan
Pityriasis Alba 299; Polymorphic Light Eruption 299; Vitiligo 299
Nevus Achromicus/Nevus Anemicus 308; Acquired Hypomelanosis 308
Contact Leukoderma 308; Pityriasis Versicolor 308; Psoriasis 308; Cutaneous Sarcoidosis 310
Granuloma Annulare 310; Leishmaniasis 310; Cutaneous Tuberculosis 312
Lupus Miliaris Disseminative Faciei 312; Discoid Lupus Erythematosus 312
Malignancies 312; Miscellaneous Rare Conditions 313; Some General Points to Remember 313
22. Differential Diagnosis of Neurological Disorders in Relation to Leprosy 316
Bela J Shah
Patterns of Neuropathy 316; Epidemiology 317; Basic Anatomy 317; Pathophysiology 317
Basic Terminology 318; Diagnostic Approach 318; History 318
Physical Examination 320; Laboratory Testing 321
Various Neuropathic Disorders 321; Hereditary Neuropathy 326
xx Ial Textbook of Leprosy
Section 4: Systemic Involvement and Special Situations of Leprosy
23. Systemic Involvement in Leprosy 331
V Ramesh, Joginder Kumar Kataria
Bacillemia in Leprosy 331; Bones, Joints and Muscles 332; Nail Involvement 335
Eyes 335; Upper Respiratory Tract (Nose, Paranasal Sinuses, Oral Cavity, Oropharynx, and Larynx) 336
Lower Respiratory Tract 337; Gastrointestinal Tract 337; Reproductive System 337
Kidneys 337; Endocrine Glands 338; Lymph Nodes, Bone Marrow, and Spleen 338
Central Nervous System 339; Cardiovascular System 339; Hematology and Serology 339
24. Leprosy and Human Immunodeficiency Virus Coinfection 344
Archana Singal
Prevalence and Diagnosis 344
Predominance of Multibacillary or Paucibacillary Type and Clinical Features 345
Complications, Reactions, Relapse and Outcomes 345; Response to Treatment 346
Impact of Leprosy on Hiv Progression and Antiretroviral Therapy 346
Immunology and Histology in Leprosy-Hiv Coinfection 347
Immune Reconstitution Inflammatory Syndrome 348
25. Leprosy and Pregnancy 352
Neena Khanna
Effect of Leprosy on Female Reproductive System 352; Effect of Pregnancy on Leprosy 353
Presentation of Leprosy in Pregnant Women 354; Effect of Leprosy on Pregnancy 356
Postpartum Period 356; Impacts on Infant and Lactating Mother 357; Practical Tips 358
26. Childhood Leprosy 360
Archana Singal, Namrata Chhabra
Epidemiology 360; Transmission 361; Classification 361; Clinical Presentation 362
Diagnosis 366; Treatment 367; Prevention 367
27. Ocular Leprosy 370
Daniel Ebenezer
Ocular Involvement 371; Clinical Examination of the Eye in Leprosy 371
Lagophthalmos and Other Lid Deformities 378; Corneal Involvement 382; Ocular Inflammations 385
Cataract 387; Intraocular Pressure 389; Ocular Deformity Classification in Leprosy 390
Section 5: Disease Complications (Nerve Involvement, Neuritis and Reactions)
28. Neuritis: Definition, Clinicopathological Manifestations and
Proforma to Record Nerve Impairment in Leprosy 397
P Narasimha Rao, Sujai K Suneetha, Gigi J Ebenezer
Definitions 397; Pathological Basis of Neuritis 398
Mechanisms of Neuritis Across the Spectrum of Leprosy 399
Mechanism of Neuritis during Reactions 400; Clinical Presentations of Neuritis 400
Neuritis Associated with the Disease 401; Neuritis Associated with Reactions 401
Silent Neuritis or Quiet Nerve Paralysis 402; Clinical Manifestations of Neuritis 402
Methodology to Record Nerve Involvement 404; Clinical Grading of Nerve Thickening, Tenderness and Pain 404
Tools to Assess Sensory and Motor Nfi and Integrity of Peripheral Nerves 405
Tools to Assess Motor Nfi 409; Tools to Assess Autonomic Nfi 409; Examination of Eyes in Leprosy 410
Disability in Leprosy and its Grading 410; Proforma to Record Nerve Function Impairment 411
Motor Function Assessment of Important Nerves by Voluntary Muscle Testing 412
Proforma as for Recording Nfi in the Eye 412
Proforma to Record Clinical Grading of Nerve Involvement in Leprosy 413
Proforma to Record Disability, Deformities and Ulcers 413
xxi
29. Leprosy Reactions: Pathogenesis and Clinical Features 416
Hemanta Kumar Kar, Amrita Chauhan
Types of Leprosy Reactions 416; Epidemiology 417; Type 1 Reaction (Reversal Reaction) 418
Type 2 Reactions 424; Lucio Phenomenon 434
Section 6: Therapeutics (Medical and Surgical), Prophylaxis and Vaccines
30. Chemotherapy of Leprosy 441
Paul R Saunderson
Dapsone 442; Rifampicin 442; Clofazimine 443; Ethionamide/Prothionamide 444
Fluoroquinolones 444; Minocycline 444; Clarithromycin 445;
Bedaquiline 445; Persisters 445; Relapse 445; Future Regimens 446
31. Chemotherapy: Development and Evolution of WHO-MDT and Newer Treatment Regimens 448
VV Pai, RR Rao, V Halwai
Some Significant Landmarks in the Treatment of Leprosy and the Evolution of Mdt 448
Who Study Group on Chemotherapy of Leprosy 450; History of Mdt 450
Post-treatment Surveillance 452; Chemotherapy of Leprosy: Further Challenges 454
Chemotherapy and Related Clinical Issues 455; Recommendations for Research Trials 458
32. Management of Leprosy Reactions 465
Hemanta Kumar Kar, Ruchi Gupta
Early Diagnosis of Reaction 465; Management of Type 1 Reaction or Reversal Reaction 465
Treatment of Late Reversal Reaction 468; Management of Type 2 Reaction 468
Management of Lucio Phenomenon 475
33. Management of Neuritis and Neuropathic Pain 478
Bhushan Kumar, Sunil Dogra
Diagnosis and Monitoring 479; Management 480; Corticosteroids 481
Other Anti-inflammatory Agents 483; Clofazimine 483; Thalidomide 483
Azathioprine 483; Cyclosporine 484; Intraneural Administration of Drugs 484
Chronic Neuropathic Pain 484; Surgery 485; General Measures 486
Prevention 486; Newer Approaches of Treatment 487
Human Immunodeficiency Virus and Leprous Neuritis 487
34. Chemoprophylaxis in Leprosy 490
BK Girdhar
Who Needs Chemoprophylaxis? 490; Dapsone Chemoprophylaxis 491
Rifampicin for Protection Against Leprosy 493; Application of Chemoprophylaxis 494
35. Leprosy Vaccines: Immunoprophylaxis and Immunotherapy 496
Kiran Katoch
Immunoprophylaxis 496; Chemoprophylaxis 498; Immunotherapy 499
36. Nursing Care in Leprosy Patients 506
Vineet Kaur, Gurmohan Singh
Nursing Assessment of a Leprosy Patient 506; Assessment During Repeat Visits 509
Assessment of “Risk” Status 509; Assessment of Activities of Daily Living 509
Role in the Care of Anesthetic Limbs 510; Intervention in Deformity due to Leprosy 512
Intervention for Care of the Eyes in Leprosy 515; Role in the Care of Women with Leprosy 515
Role in the Care of Indoor Patients 516
37. Deformities of Face, Hands, Feet and Ulcers and their Management 517
Atul Shah, Neela Shah
Clinical Manifestations 517; Disabilities and Deformities in Leprosy: Terminology 518
Nerve Involvement 519; Testing for Nerve Damage 519; Deformities of Hands 522
Deformities of Feet 526; Deformities of Face 528
Contents
xxii Ial Textbook of Leprosy
Reconstructive Surgery (in Disability Prevention and Medical Rehabilitation)—A Government of India Initiative 529
Economic Rehabilitation 530; Health Education to a Person Affected with Leprosy 531
Numbness? No Sensation in your Hands or Feet? 536
Section 7: Miscellaneous
38. Deformity and Disability Prevention 541
GN Malaviya
Types of Deformities 541; Grading of Disabilities and Deformities 542
Factors Affecting the Onset and Progression of Disabilities 543
Causation of Deformities 544; Effects of Infiltration of Tissues by M. Leprae 544
Effects of Motor Paralysis 547; Effects of Anesthesia and Analgesia 547
Prevention of Disabilities 547; Anticipating Nerve Function Impairment 548
Role of Steroids 548; Role of Nerve Trunk Decompression 548
Care of the Eyes 548; Management of Reactions 548; Monitoring and Self-reporting 548
Evaluation of the Patient and Assessment of Disability 549; Role of Physiotherapy 549
Techniques of Physiotherapy as Applied to Leprosy Affected Persons 550
Prevention of Progression of Disabilities 556
Disabilities in Leprosy in the Context of Newer Concepts 558
39. Relapse in Leprosy 562
Devinder Mohan Thappa, Sowmya Kaimal, Divya Gupta
Definition 562; Relapse Rate 563; Microbiological Aspects 564
Immunological Tests for Relapse 564; Histopathology 565; Relapse Interval 565;
Classification of Relapse 566; Predisposing Factors for Relapse 566; Clinical Features 567
Differential Diagnosis 567; Diagnosis 569; Treatment 570; Failure to Respond to Therapy 570
40. Drug Resistance in Leprosy 573
Masanori Matsuoka
Chemotherapy 573; Method for Susceptibility Assay 576
Sentinel Surveillance by WHO 578; Relapse and Persisters 578
Viability in Various Conditions 579
41. Morbidity and Mortality in Leprosy 583
Ben Naafs, S Noto, PAM Schreuder
Morbidity 583; Mortality 584
42. Recording-Reporting and Monitoring MDT Services 587
Pratap Rai Manglani
Terms Used 587; Records 591; Reports 591
Validation Mechanism at Different Level 593; Performance Assessment 594
Section 8: Rehabilitation and Social Issues
43. Rehabilitation 619
D Kamaraj
Leprosy Rehabilitation 619; Community Based Rehabilitation 620; Sample Referral Format 626
Rehabilitation Services for Disabled Persons under the Ministry of Social Justice and Empowerment 626
Vocational and Employment Services/Opportunities for People with Disabilities under the Ministry of Labor 628
Services for Disabled Persons under the Ministry of Rural Development 628
44. Community-based Initiatives in Comprehensive Leprosy Work 630
PV Ranganadha Rao
Leprosy Program 631
Rationale and Purpose of Community-based Initiatives for Comprehensive Leprosy Work 632
xxiii
Community Participation in Primary Health Care 632; Opportunities for Community-based Initiatives 634
Scope of Community-based Initiatives in Leprosy 635
Experiences of Community-based Initiatives in Comprehensive Leprosy Work 635
45. Psychosocial Aspects in Leprosy 641
PK Gopal
Stigma 641; Rehabilitation 643; Categorization of Leprosy Affected Persons 644
Empowerment 645
46. Human Rights and Stigma in Leprosy 646
VV Dongre
In the Footsteps of Time... 646; Social Aspects 646; Medical Implications 647
Aspects of Discrimination: Human Rights Violations 647
Parsi Marriage Act of 1936 (No Divorce Granted on the Ground of Leprosy) 648
Employment Rules 649; Winds of Change 650; Voice of Awakening 650
47. Migration and Leprosy 652
Mohammad Aleem Arif
Effects of Migration on Leprosy Control 653
Migration, Stigma and Human Rights in Leprosy 655
48. Health Promotion, Education and Counseling 657
Manimozhi Natarajan
Perspectives 657; Health Promotion 658; Health Education 658
Information, Education and Communication 659; Interpersonal Communication 663
Case Study 666; Communication Skills 666; Programme Focus in Relation to Leprosy 668
Key Issues in Communication for Leprosy Programme 669
Activity Planning Versus Strategic Planning 670
49. Role of NGOs in National Leprosy Eradication Programme 672
Thomas Abraham, Pankaj Sharma, Atul Shah, VV Pai, Mohammad Aleem Arif
Major Ngos Working in Partnership with Nlep 672; Local Voluntary Organizations 684
Section 9: Future Prospects
50. Leprosy: Future Challenges 687
Diana NJ Lockwood
Immunoprophylaxis 687; Diagnosis 688; Immunotherapy 689; Immunosuppression 690
Erythema Nodosum Leprosum 690; Neuropathic Pain 690
51. Leprosy Scenario Beyond 2020 694
PS Sundar Rao
The Journey So Far 694; The Current Scenario 695; The Future 696
52. Leprosy Scenario Beyond its Elimination 704
Marcos Virmond
The Elimination Strategy 705; Leprosy Beyond Elimination 706; Clinical Expertise 708
Transmission and Vaccine 709; Research 710
53. Leprosy Recognition and Treatment in a Low Endemic Setting 713
David M Scollard
Background 713; What is Low Endemic? 713
Integration of Leprosy Services into General Health Care 714
Goals and Requirements of Leprosy Services in Low Endemic Situations 714
The United States Experience as a Model for Management of Leprosy in a Low Endemic Situation: Carville 2.0 714
Contents
xxiv Ial Textbook of Leprosy
54. Case Studies 718
Pankaj Sharma, Hemanta Kumar Kar, Ruchi Gupta
Childhood and Disease Detection 722; Public Pressure for Isolation 722
Shift Away from the Native Land 723; Choice of Profession 723
The Social Mobilization of LAPs 724; Family and the Children 724; Embracing Christianity 724
Social Implications of Disease 726; No Family, Alone in the World 727; The Children 727
The Mission Factor 727; The Religion 728; Indirect Pressure for Extradition 728
Treatment and Long Stays at Various Missionary Hospitals 729
Move to Delhi (and No to Begging) 730; Marriage and Children 731
A Social Server by Heart and a Philanthropist 731; The Changing Scenario 733; Message 734
Photo Gallery 735
Index 743