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GEORGE J.HRUZAMDLYNN A.CORNELIUSMDJON C.STARRMD
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.
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.
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.
Witt PD, Cohen DT, Mallory SB. Use of a Permanent Acellular Dermal Allograft in Recessive Dystrophic Epidermolysis Bullosa Involving the Hands. Arch Dermatol. 1999;135(5):503–506. doi:10.1001/archderm.135.5.503
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