Apligraf in the Treatment of Severe Mitten Deformity Associated with Recessive Dystrophic Epidermolysis Bullosa

Author(s): Fivenson DP, Scherschun L, Cohen LV

Source: Plastic & Reconstructive Surgery 112: 584-588, 2003.

Summary:

Dystrophic epidermolysis bullosa (EB) is a genetically determined disease of the skin manifested by severe deforming bullae and scarring resulting from friction and contact.  It is the most severe form of a family of diseases associated with specific genetic mutations.  Extensive scarring, particularly of the hands and the extensor surfaces of the upper and lower extremities, produces severely crippling mutilation.  The epidermal surface of the esophagus is often affected, leading to nutritional compromise and further inhibiting wound healing.  Infection, squamous cell carcinoma (scar carcinoma), and esophageal stricture can lead to fatal complications.  The mitten hand deformity is characterized by scar syndactylization of the fingers, with loss of digital motor abilities.  Surgical treatment has typically consisted of release of contractures, with or without grafting.  Skin grafting has been controversial, with poor results in the fingers and frequent donor site complications related to involvement of the donor site from the disease process.  The authors report what is claimed to be the first use of graftskin (Apligraf), a bilayer, allogenic, human skin substitute, in the treatment of severe hand contractures in EB patients.  Five subjects, ages 3-61, underwent complete epidermal denudation of the hands and release of webspace contractures (syndactyly).  Flexion contractures were also released with transverse incisions and Z-plasties.  Graftskin was then applied.  No suturing was performed.  Bulky compression dressings were maintained in position of function for 1 week, then reapplied for a second week.  All patients experienced substantial improvements in range of motion at 3 months, although no statistical analysis was undertaken.  All patients had recurrent erosions, and two experienced refusion of three webspaces following trauma.

The authors report that their treatment results in rapid wound healing and a dramatic reduction in the frequency of repeated blistering.  They hypothesize that this may result from improved wound control from the grafts, provision of healing elements such as cytokines to the wound bed, or replacement of basement-membrane-defective diseased tissues.  Their results are encouraging, but early, and they propose longer-term studies and DNA analysis to determine the effectiveness and mechanism of improvement.  Indeed, comparison studies between graftskin and simple excision/release with secondary healing would be very instructive in determining the true efficacy of this technology in dystrophic EB.
 

 


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