Blistering Diseases: Clinical Features, Pathogenesis, Treatment

Front Matter....Pages i-xxi
Front Matter....Pages 1-1
Biology of the Basement Membrane Zone....Pages 3-20
The Immune System in Normal Skin and in AIBD....Pages 21-33
Keratins and Their Role in EB Simplex....Pages 35-47
Desmosomal Proteins and Their Role in Epidermolysis Bullosa....Pages 49-54
Desmosomal Proteins as Autoantigens in Pemphigus....Pages 55-65
Collagen XVII and Its Role in Junctional Epidermolysis Bullosa....Pages 67-75
Plectin in Epidermolysis Bullosa and Autoimmune, Bullous Diseases....Pages 77-84
Integrins A6 and B4 and Their Role in Junctional Epidermolysis Bullosa and Recessive Epidermolysis Bullosa Simplex....Pages 85-90
Laminin 332 in Junctional Epidermolysis and as an Autoantigen in Mucous Membrane Pemphigoid....Pages 91-102
Kindlin-1 and Its Role in Kindler Syndrome....Pages 103-110
COL7A1 and Its Role in Dystrophic Epidermolysis Bullosa....Pages 111-120
Pathogenesis of Epidermolysis Bullosa Acquisita....Pages 121-130
Genetic and Environmental Risk Factors of Autoimmune Bullous Diseases....Pages 131-139
Apoptosis and Signaling in Bullous Diseases: Pemphigus....Pages 141-152
Front Matter....Pages 153-153
How to Take a Skin Biopsy Correctly to Diagnose Epidermolysis Bullosa and Autoimmune Bullous Diseases....Pages 155-159
Histological and Immunofluorescence Diagnosis of Autoimmune Blistering Diseases....Pages 161-191
ELISA Testing and Immunoblotting....Pages 193-201
Immunofluorescence Mapping....Pages 203-212
Electron Microscopy and Immunoelectron Microscopy....Pages 213-237
Prenatal Diagnosis of Epidermolysis Bullosa: Current Aspects and Perspectives....Pages 239-248
Front Matter....Pages 249-249
Epidemiology of Autoimmune Bullous Diseases....Pages 251-263
Epidermolysis Bullosa Registries and the Epidemiology of Epidermolysis Bullosa (EB)....Pages 265-274
Front Matter....Pages 275-275
Pemphigus Foliaceus and Endemic Forms....Pages 277-281
Pemphigus Vulgaris....Pages 283-288
Paraneoplastic Autoimmune Multiorgan Syndrome: Paraneoplastic Pemphigus Revisited....Pages 289-297
IgA Pemphigus....Pages 299-305
Ectodermal Dysplasia Skin Fragility Syndrome....Pages 307-312
Acantholytic Forms of Epidermolysis Bullosa....Pages 313-318
Erythema Multiforme, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis....Pages 319-326
Clinical Features of Epidermolysis Bullosa Simplex....Pages 327-332
Front Matter....Pages 333-333
Bullous Pemphigoid (Cutaneous Pemphigoid): The Spectrum of Clinical Presentations....Pages 335-342
Pemphigoid Gestationis....Pages 343-350
Linear IgA Disease....Pages 351-356
Clinical Features and Pathogenesis of Chronic Bullous Disease of Childhood....Pages 357-361
Mucous Membrane Pemphigoid....Pages 363-373
Generalized Severe Junctional Epidermolysis Bullosa....Pages 375-380
Junctional Epidermolysis Bullosa, Generalized Intermediate Type....Pages 381-391
Laryngo-Onycho-Cutaneous Syndrome....Pages 393-396
Junctional Epidermolysis Bullosa with Renal and Respiratory Involvement: Integrin α[Alpha]3 Mutations....Pages 397-401
Front Matter....Pages 403-403
Epidermolysis Bullosa Acquisita....Pages 405-412
Front Matter....Pages 403-403
Clinical Features of Bullous Systemic Lupus Erythematosus....Pages 413-418
Dystrophic Epidermolysis Bullosa....Pages 419-430
Front Matter....Pages 431-431
Kindler Syndrome....Pages 433-439
Dermatitis Herpetiformis....Pages 441-447
Ocular Involvement in Autoimmune Blistering Skin Diseases....Pages 449-455
Front Matter....Pages 457-457
Autoimmune Blistering Diseases and Corticosteroid Use: A Review of the Evidence....Pages 459-468
Azathioprine....Pages 469-481
Mycophenolate Mofetil for the Management of Autoimmune Bullous Diseases....Pages 483-491
Dapsone and Autoimmune Bullous Disorders....Pages 493-499
Tetracyclines and Their Use in Autoimmune Bullous Diseases and Epidermolysis Bullosa....Pages 501-505
Cyclophosphamide in Autoimmune Blistering Diseases: Safety, Efficacy and Evidence Base....Pages 507-513
Using Intravenous Immunoglobulins in Autoimmune Bullous Diseases....Pages 515-521
Rituximab and Its Use in Autoimmune Bullous Disorders....Pages 523-529
Front Matter....Pages 531-531
The Treatment of Pemphigus Vulgaris and Pemphigus Foliaceus....Pages 533-541
Management of Bullous Pemphigoid....Pages 543-550
Current Management of Pemphigoid Gestationis....Pages 551-555
Treatment of Chronic Bullous Disease of Childhood....Pages 557-560
Management of Linear IgA Disease in Adults....Pages 561-563
Mucous Membrane Pemphigoid....Pages 565-571
Treatment of Dermatitis Herpetiformis....Pages 573-578
Front Matter....Pages 531-531
Epidermolysis Bullosa Acquisita....Pages 579-587
Management of Bullous Systemic Lupus Erythematosus....Pages 589-592
Treatment and Prevention of Glucocorticoid-Induced Osteoporosis....Pages 593-603
Prevention and Therapy of Steroid-Induced Diabetes....Pages 605-610
Prevention and Treatment of Infections in Patients with Autoimmune Blistering Disease....Pages 611-615
Management of Erythema Multiforme, Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis....Pages 617-622
Quality of Life in Autoimmune Blistering Diseases....Pages 623-631
Extent Measures in Autoimmune Bullous Disease....Pages 633-642
The Pathogenesis, Assessment and Management of Pruritus in Autoimmune Bullous Diseases....Pages 643-649
Front Matter....Pages 651-651
Supportive Care: Bathing, Wound Care, Nutrition, Pain and Itch Management, Psychosocial Support, Palliation....Pages 653-666
Monitoring to Prevent Complications: Anemia, Infections, Osteopenia, Failure to Thrive, Renal Disease, Squamous Cell Carcinoma, Cardiomyopathy....Pages 667-685
Treatment of EB Complications: Esophageal Strictures, Pseudosyndactyly, Urethral and Meatal Stenosis, and Laryngeal Stenosis....Pages 687-694
Special Issues in Epidermolysis Bullosa: Eyes, Hair, Nails, Teeth and Pregnancy....Pages 695-709
Living with Epidermolysis Bullosa: Reviewing the Impact on Individuals’ Quality of Life....Pages 711-717
The Pathogenesis and Management of Pruritus in Epidermolysis Bullosa....Pages 719-727
Future Therapies for Epidermolysis Bullosa....Pages 729-736
Back Matter....Pages 737-752