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Use of a Permanent Acellular Dermal Allograft in Recessive Dystrophic Epidermolysis Bullosa Involving the Hands
Peter D. Witt, MD;
Daniel T. Cohen, BA;
Susan B. Mallory, MD
from the Departments of Surgery, Plastic and Reconstructive (Drs Witt and Mr Cohen), Internal Medicine (Dermatology) (Dr Mallory), and Pediatrics (Dr Mallory), Washington University School of Medicine, St Louis Children's Hospital, and the Department of Plastic and Reconstructive Surgery, Shriners Hospital for Children (Dr Witt), St Louis, Mo
Arch Dermatol. 1999;135:503-506.
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INTRODUCTION
RECESSIVE DYSTROPHIC epidermolysis bullosa (RDEB) is characterized by blistering and scarring of the skin and mucous membranes. The hands are particularly vulnerable because of the contact and shearing forces of normal daily activity.
REPORT OF A CASE
An 11-year-old white boy presented with RDEB and extensive epidermal scarring around the hand that caused restrictive adduction of the thumbs and pseudosyndactyly. Repeated cycles of blistering and scarring resulted in gradual encasement of the hand in an epidermal "cocoon"(Figure 1). Functional impairments included loss of fine motor manipulation of objects commensurate with the loss of digital prehension.
Figure appears in full text version.
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Figure 1. Characteristic thumb adduction contracture along with epidermal "cocoon" and pseudosyndactyly.
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THERAPEUTIC CHALLENGE
Our goal was to relieve the scar contractures associated with the epidermal encasement of the hand in a patient with RDEB, while minimizing donor site morbidity and graft hypertrophy.
SOLUTION
We used a permanent . . . [Full Text of this Article] Preparation of Acellular Dermal Allograft Surgical Procedure
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