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Dominant Dystrophic Epidermolysis Bullosa: A Clinical Overview

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Book cover Epidermolysis Bullosa

Abstract

Dominant dystrophic epidermolysis bullosa (DDEB) is characterized by formation of blisters below the lamina densa and by autosomal dominant inheritance. Patients often present with trauma-induced blisters at birth or shortly thereafter. In contrast to epidermolysis bullosa simplex (EBS), blisters heal with scarring (Fig. 10.1), but the severe mittenlike scars of the hands and feet so characteristic of recessive dystrophic EB do not occur in DDEB. Nails are often dystrophic (Fig. 10.2). Extracutaneous involvement is usually mild and is often limited to the mouth.

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Lin, A.N., Carter, D.M. (1992). Dominant Dystrophic Epidermolysis Bullosa: A Clinical Overview. In: Lin, A.N., Carter, D.M. (eds) Epidermolysis Bullosa. Springer, New York, NY. https://doi.org/10.1007/978-1-4612-2914-8_10

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  • DOI: https://doi.org/10.1007/978-1-4612-2914-8_10

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4612-7717-0

  • Online ISBN: 978-1-4612-2914-8

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