Epidermolysis Bullosa

1. Front Matter

   Pages i-xiv

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2. Current Perspectives

   1. Front Matter

      Pages 1-1

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   2. Current Perspectives and Differential Diagnosis in Epidermolysis Bullosa

      • Robin A. J. Eady

      Pages 3-15

3. Basic Science Aspects

   1. Front Matter

      Pages 17-17

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   2. The Basement Membrane Zone at the Dermal — Epidermal Junction of Human Skin

      • David T. Woodley, Scott McNutt

      Pages 19-36

   3. Pathology and Pathogenesis of Epidermolysis Bullosa

      • Jo-David Fine

      Pages 37-62

   4. Collagenase and Connective Tissue Remodeling in Recessive Dystrophic Epidermolysis Bullosa

      • Elke Voges, Annemarie Kronberger, Rosalind A. Grymes, Eugene A. Bauer

      Pages 63-69

   5. Linkage Studies in Epidermolysis Bullosa

      • Ervin H. Epstein Jr.

      Pages 70-74

   6. Epidermolysis Bullosa Acquisita

      • David T. Woodley, W. Ray Gammon, Robert A. Briggaman

      Pages 75-86

4. Clinical Overview

   1. Front Matter

      Pages 87-87

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   2. Epidermolysis Bullosa Simplex: A Clinical Overview

      • Andrew N. Lin, D. Martin Carter

      Pages 89-117

   3. Junctional Epidermolysis Bullosa: A Clinical Overview

      • Andrew N. Lin, D. Martin Carter

      Pages 118-134

   4. Recessive Dystrophic Epidermolysis Bullosa: A Clinical Overview

      • Robert A. Briggaman

      Pages 135-151

   5. Dominant Dystrophic Epidermolysis Bullosa: A Clinical Overview

      • Andrew N. Lin, D. Martin Carter

      Pages 152-165

5. Extracutaneous Manifestations and Their Management

   1. Front Matter

      Pages 167-167

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   2. Gastrointestinal Aspects of Epidermolysis Bullosa

      • Gulchin Ergun, Robert A. Schaefer

      Pages 169-184

   3. Ophthalmological Aspects of Epidermolysis Bullosa

      • Scott E. Brodie

      Pages 185-190

   4. Hematologic Problems in Epidermolysis Bullosa

      • Patricia J. Giardina, Andrew N. Lin

      Pages 191-197

   5. Dental Aspects of Epidermolysis Bullosa

      • John J. Putnam, George. W. Sferra Jr.

      Pages 198-209

   6. Otorhinolaryngologic Aspects of Epidermolysis Bullosa

      • Andrew N. Lin, Shelley R. Berson, Robert F. Ward

      Pages 210-216

Because skin blisters are the initial manifestation of epidermolysis bullosa (EB), patients invariably present to the dermatologist for diagnosis and treatment. However, EB is a systemic disease whose management requires input from clinicians in virtually all fields of medicine, including pediatri­ cians, surgeons, dentists, gastroenterologists, hematologists, otorhinolaryn­ gologists, dietitians, and physical therapists, to name a few. Because EB is a rare disease, few clinicians are familiar with it, and many recoil at the pros­ pect of caring for individuals covered with blisters caused by a disease they know little about. For patients, insult is thus added to injury and they feel abandoned, neglected, and frustrated. One way to remedy this deplorable situation is to provide clinicians with a compact source of information de­ tailing the principles of EB diagnosis and treatment. This text seeks to fulfill this role. From 1986-1991, The Rockefeller University Hospital has been the co­ ordinating center of the National EB Registry. Supported by The National Institutes of Health, this Registry consists of four university centers* commit­ ted to collecting clinical data concerning diagnosis, treatment, and epidemio­ logy on all American EB patients. As of April 1992, nearly 1,799 EB patients have enrolled nationwide. The Registry is now in its second five-year phase of operation.

• anesthesia
• differential diagnosis
• management
• nursing
• nutrition
• pathology
• prenatal diagnosis
• reconstructive surgery
• rehabilitation
• rheumatism
• screening
• skin
• surgery
• tissue
• treatment

• Laboratory for Investigative Dermatology, The Rockefeller University, New York, USA

  Andrew N. Lin, D. Martin Carter

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