Epidermolysis Bullosa: Basic and Clinical Aspects

Front Cover
Springer Science & Business Media, May 21, 1992 - Medical - 302 pages
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.
 

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Contents

Current Perspectives and Differential Diagnosis in Epidermolysis Bullosa
3
The Basement Membrane Zone at the DermalEpidermal Junction of Human Skin
19
Pathology and Pathogenesis of Epidermolysis Bullosa
37
Collagenase and Connective Tissue Remodeling in Recessive Dystrophic Epidermolysis Bullosa
63
Linkage Studies in Epidermolysis Bullosa
70
Epidermolysis Bullosa Acquisita
75
Epidermolysis Bullosa Simplex A Clinical Overview
89
Junctional Epidermolysis Bullosa A Clinical Overview
118
Dental Aspects of Epidermolysis Bullosa
198
Otorhinolaryngologic Aspects of Epidermolysis Bullosa
210
Rheumatologic Aspects of Epidermolysis Bullosa
217
Reconstructive Surgery for Patients with Epidermolysis Bullosa
223
Anesthesia for the Epidermolysis Bullosa Patient
228
Prenatal Diagnosis and Genetic Screening for Epidermolysis Bullosa
235
Physical Rehabilitation of Epidermolysis Bullosa Patients
256
Nutritional Management of the Epidermolysis Bullosa Patient
265

Recessive Dystrophic Epidermolysis Bullosa A Clinical Overview
135
Dominant Dystrophic Epidermolysis Bullosa A Clinical Overview
152
Gastrointestinal Aspects of Epidermolysis Bullosa
169
Ophthalmological Aspects of Epidermolysis Bullosa
185
Hematologic Problems in Epidermolysis Bullosa
191
Medical and Surgical Treatment of the Skin in Epidermolysis Bullosa
271
Nursing Aspects of Epidermolysis Bullosa A Comprehensive Approach
285
Index
299
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