Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management

Cover......Page 1
Autoimmune Diseases of the Skin, Third Edition......Page 3
ISBN 9783211992241......Page 4
Foreword......Page 6
Preface to the Second Edition......Page 9
Preface to the First Edition......Page 10
Table of Contents......Page 14
List of Contributors......Page 18
T and B cells......Page 25
Thymic maturation and selection of T cells......Page 26
Central tolerance and peripheral tolerance......Page 27
Mechanisms of peripheral tolerance......Page 28
Activation and differentiation of T cells......Page 30
Activation of self-reactive T and B cells......Page 33
Breaking T and B cell tolerance......Page 34
T cells......Page 36
Tissue damage, Th1, and Th17 cells......Page 37
Therapeutic induction of functional tolerance......Page 39
References......Page 41
Introduction......Page 47
Workplace......Page 48
Processing of Samples......Page 49
Controls......Page 51
Evaluation and Interpretation......Page 53
References......Page 55
Pathogenesis......Page 57
Autoantibody profile and clinical appearance......Page 59
Clinical presentation......Page 62
Differential diagnosis......Page 67
Pemphigus foliaceus......Page 69
Paraneoplastic pemphigus......Page 70
IgA pemphigus......Page 72
Diagnosis......Page 73
Immunoserological diagnosis with autoantigens......Page 74
Therapy......Page 76
References......Page 83
Clinical features......Page 89
Presentation and clinical Setting......Page 91
Diagnosis......Page 93
Differential diagnosis......Page 96
Therapy and Prognosis......Page 98
Disease activity and relapse markers......Page 99
BP180 and BP230 are components of hemidesmosomes, junctional adhesion complexes......Page 100
BP, an example of organ-specific autoimmune disease......Page 102
Humoral immune response......Page 103
Cellular immune response and inflammatory cascade......Page 104
Pathogenic mechanism of blister formation......Page 105
Unresolved issues and perspectives......Page 107
References......Page 108
Introduction......Page 121
Clinical Appearance / Classification......Page 122
Direct Immunofluorescence Microscopy......Page 125
Histopathology......Page 126
Serological Findings......Page 128
Therapy......Page 130
Summary......Page 131
References......Page 132
Introduction......Page 137
Pathogenesis......Page 138
Clinical Manifestations......Page 140
CP-like Presentation......Page 142
LABD-like Presentation......Page 143
The Relationship between EBA and other Systemic Diseases......Page 144
Direct Immunofluorescence (DIF)......Page 145
Immunoelectron Microscopy (IEM)......Page 146
Direct Salt Split Skin Immunofluorescence (SSSI)......Page 147
Indirect SSSI......Page 148
Enzyme-Linked Immunosorbent Assay (ELISA)......Page 149
IIF Microscopy Using Substrate Deficient in Basement Membrane Molecules......Page 150
Fluorescent Overlay Antigen Mapping (FOAM)......Page 151
Immunosuppressive Agents......Page 152
Rituximab......Page 153
References......Page 154
Epidemiology......Page 161
Histopathology......Page 162
Vascular activation in morphea......Page 164
Immune system activation in morphea......Page 165
Morphea en plaque......Page 166
Keloid Nodular Morphea......Page 168
Acral Linear morphea......Page 169
Deep Morphea......Page 170
Disabling pansclerotic morphea of childhood......Page 171
Laboratory abnormalities......Page 172
Diagnosis and Measurement of Disease Activity......Page 173
Ultraviolet A......Page 174
Vitamin D derivatives......Page 178
Methotrexate......Page 179
Antimalarials......Page 182
Other therapies......Page 183
References......Page 184
Immune Dysregulation......Page 197
Dysregulation of Extracellular Matrix Synthesis......Page 198
Clinical Appearence / Classification......Page 200
Limited Cutaneous SSc (lSSc)......Page 201
Environmentally Related Scleroderma-Like Syndromes......Page 202
Clinical Presentation......Page 203
Immunosuppressive Agents......Page 206
Skin Involvement......Page 207
Renal Involvement......Page 208
Gastrointestinal Involvement......Page 209
Summary......Page 210
References......Page 211
Classification......Page 217
Pathogenesis......Page 218
Clinical Appearance / Classification......Page 222
Diagnosis......Page 225
Direct Immunofluorescence......Page 226
Serological Tests......Page 227
Therapy......Page 228
Summary......Page 231
References......Page 232
Epidemiology......Page 239
Immunogenetics......Page 240
Environmental Factors......Page 242
Cutaneous Manifestations......Page 244
Systemic Disease......Page 246
Differential Diagnosis......Page 247
Laboratory Findings......Page 248
Histopathology......Page 249
Evaluation and management......Page 250
Local Therapy......Page 251
Systemic Therapy......Page 252
Prognosis......Page 255
Summary......Page 256
References......Page 257
Introduction......Page 267
Cutaneous Manifestations......Page 268
Muscle Disease......Page 272
Systemic Features......Page 273
Amyopathic Dermatomyositis......Page 274
Myositis and Malignancy......Page 275
Drug-Induced Dermatomyositis......Page 276
Diagnosis and Evaluation of the Patient with Dermatomyositis......Page 277
Course and Therapy......Page 278
Additional Therapeutic Options for Muscle and / or Systemic Involvement......Page 279
Therapeutic Options for Cutaneous Disease......Page 280
Summary......Page 281
References......Page 282
Anti-U1snRNP Autoantibodies......Page 291
Other Autoantibodies......Page 292
Clinical Appearance......Page 294
Diagnosis......Page 297
Therapy......Page 301
References......Page 302
Introduction......Page 307
Criteria for Diagnosis......Page 308
Pitfalls in Diagnosis and Methodology......Page 312
Overview of Pathogenesis and Correlation with Clinical Symptoms......Page 314
Oral Symptoms and Signs in the SS Patient......Page 316
Cutaneous Symptoms and Signs in the SS Patient......Page 319
Systemic Findings in the SS Patient......Page 322
Differential Diagnosis......Page 328
A. Cutaneous Therapy......Page 330
Punctal occlusion......Page 331
Topical cyclosporine......Page 332
C. Oral Therapy......Page 333
D. Secretagogues......Page 335
E. Systemic Therapy for Extraglandular manifestations of SS......Page 336
Summary......Page 337
References......Page 338
Epidemiology and genetics of psoriasis......Page 349
Histopathology......Page 351
Environmental factors......Page 352
T cell activation in psoriasis......Page 353
Modification by site......Page 356
Clinical variants......Page 357
Course of psoriasis......Page 359
Management of psoriasis......Page 360
Topical therapy......Page 361
Phototherapy and photochemotherapy......Page 362
Systemic therapy......Page 363
Summary......Page 365
References......Page 366
Classification of Chronic Urticaria......Page 373
Symptomatic Dermographism......Page 374
Cholinergic Urticaria......Page 375
Cold Urticaria......Page 377
Food Additive-Evoked Chronic Urticaria......Page 378
Treatment......Page 379
Etiology......Page 380
Clinical Features and Investigation......Page 381
Treatment......Page 382
Pathomechanisms......Page 383
Diagnosis......Page 386
Treatment of Autoimmune Urticaria......Page 389
References......Page 392
Introduction......Page 397
Immune Pathogenesis......Page 398
Lichen planus, lichenoid eruptions......Page 401
Clinical variants......Page 402
Cutaneous graft-versus-host-reaction (cGVHR)......Page 404
Lichen planus, lichenoid eruptions......Page 406
Summary......Page 408
References......Page 409
ANCA-associated vasculitides......Page 413
Granulomatous lesions in WG and CSS......Page 415
Immune complex-mediated vasculitides......Page 416
Skin manifestations......Page 417
Systemic vasculitis......Page 418
Laboratory tests......Page 420
ANCA-associated vasculitides (WG, MPA, CSS)......Page 421
Cryoglobulinemic vasculitis......Page 422
Summary......Page 423
References......Page 424
Skin Manifestations in Systemic Vasculitis......Page 429
Clinical features......Page 430
Extravascular necrotizing granuloma......Page 433
Pyoderma gangrenosum......Page 434
Raynaud’s phenomenon......Page 435
Henoch-Schönlein purpura......Page 436
Polyarteritis nodosa (PAN)......Page 438
Churg and Strauss syndrome......Page 440
Wegener’s granulomatosis......Page 441
Takayasu arteritis (TA) (Perniciaro et al., 1987, Francès et al., 1990, Skaria et al., 2000, Pascual-Lopez et al. 204)......Page 443
Giant cell arteritis (GCA)......Page 444
Rheumatoid nodules......Page 445
Rheumatoid Vasculitis (RV)......Page 446
Pyoderma gangrenosum (PG)......Page 447
Non-steroidal anti-inflammatory drugs (NSAID)......Page 448
Local reactions......Page 449
Inflammatory skin eruptions......Page 450
Cutaneous Manifestations In Subsets Of Rheumatoid Arthritis......Page 451
Dermatologic Manifestations of Relapsing Polychondritis (Francès et al., 2001)......Page 452
References......Page 454
Introduction......Page 459
Clinical picture and classification......Page 460
I. ROS and RNS induced stress in vitiligo......Page 462
H2O2 affects the essential cofactor (6R)-L-erythro 5,6,7,8 tetrahydrobiopterin (6BH4) and consequently cofactor dependent mechanisms......Page 464
H2O2 affects epidermal calcium homeostasis in vitiligo......Page 466
Increased DNA damage does not induce skin cancer in patients with vitiligo despite the presence of high H2O2- and ONOO– levels......Page 467
II. The neural hypothesis......Page 468
IV. Genetic factors in vitiligo......Page 469
Could formation of neoantigens through ROS / RNS mediated oxidation and failing degradation / turnover foster production of autoantibodies in vitiligo?......Page 470
ROS / RNS and induction of T-cell response – the possible link to auto immunity in vitiligo?......Page 472
Newer treatment concepts......Page 474
What is the overall take home message?......Page 475
References......Page 478
Classification / Clinical appearance......Page 487
Rodent models......Page 490
Human epidemiology......Page 492
HLA genes......Page 493
Non HLA genes......Page 495
Circumstantial evidence for AA as an autoimmune disease......Page 496
Immune system targets......Page 497
The humoral immune system in AA......Page 498
Mechanisms of cellular action on the hair follicle......Page 499
Disease modulating factors......Page 500
Disease initiation......Page 502
Therapy......Page 503
Intralesional corticosteroids......Page 504
Photochemotherapy (PUVA)......Page 505
Treatment protocol......Page 506
Side effects......Page 507
Irritant contact dermatitis – anthralin......Page 508
Summary......Page 510
References......Page 511
Introduction......Page 521
Molecular background of IgE autoreactivity......Page 522
Sensitization to self antigens in AD patients is already detectable in early infancy......Page 523
Hom s 2......Page 524
Malassezia sympodialis and “Head and Neck Dermatitis”......Page 525
Summary......Page 526
References......Page 527
Eosinophil infiltration in autoimmune bullous diseases......Page 529
Eosinophil activation and mediator release......Page 530
The distinct functions of eosinophils......Page 531
Eosinophils in bullous pemphigoid......Page 533
Eosinophils as therapeutic targets......Page 534
References......Page 535
What are paraneoplasias?......Page 541
1. “Hyperkeratotic” SP.......Page 544
2. Inflammatory paraneoplasias of the skin......Page 547
3. Collagen vascular diseases......Page 549
4. Autoimmune bullous diseases......Page 550
5. Neutrophilic dermatoses......Page 552
6. Paraneoplasias associated with plasma cell dyscrasia and monoclonal gammopathy......Page 553
7. Miscellaneous......Page 556
References......Page 558
Antigen Specific Approaches for the Therapy of Autoimmune Diseases......Page 561
Therapy by Autoantigen Administration......Page 562
B cell targeted strategies......Page 563
Anti-CD20 antibody therapy......Page 564
Second generation anti-CD20 monoclonal antibodies and small modular immunopharmaceutical proteins......Page 566
Anti-CD22 antibody (epratuzumab)......Page 567
Inhibitors of B cell survival and signaling factors......Page 568
Therapeutic approaches targeting BAFF and APRIL......Page 569
Strategies of blocking T-B cell interactions and germinal center reactions......Page 570
T cell targeted strategies......Page 571
Hematopoietic stem cell transplantation......Page 574
Clinical experience with HSCT in autoimmune diseases......Page 575
Mesenchymal stem cells for autoimmune diseases......Page 577
Targeting the fibrotic pathway in systemic sclerosis......Page 579
Targeting cytokines and cytokine receptors......Page 580
Anti-Il-6 receptor antibody (tocilizumab)......Page 581
Anti-IL-12/23 monoclonal antibody (ustekinumab)......Page 582
Summary and Conclusions......Page 583
References......Page 584
Subject Index......Page 595