Surgical treatment of pseudosyndactyly of the hand in epidermolysis bullosa: histological analysis of an acellular allograft dermal matrix.
Recessive dystrophic epidermolysis bullosa is an inherited mechanobullous disorder of skin and mucous membranes. The most striking clinical characteristic of the disease is the formation of blisters following trivial trauma. Repeated cycles of blistering and scarring result in gradual encasement of the hand in an epidermal "cocoon." The authors treated an 11-year-old boy with recessive dystrophic epidermolysis bullosa who presented with hand contractures and interdigital pseudosyndactyly. Treatment included release of contractures and application of a biosynthetic dermal analog. This report is a histological analysis of the dermal matrix 1 year after initial placement of the allograft. Fibroblasts repopulating the dermal allograft had a normal synthetic phenotype and lacked the myofibroblastic features seen in the ungrafted control biopsy. Collagen and elastin in the repopulated dermal allograft had normal dermal orientation and maturity in contrast to the sparse, immature collagen and lack of elastin compared with the dermis of an ungrafted control region. Results of this histological study indicate that treatment of recessive dystrophic epidermolysis bullosa with an acellular human dermal allograft may restore some features of normal dermal architecture. Although the initial results are encouraging, longer follow-up is required before definitive conclusions can be made.
Witt
P D
PD
Department of Surgery, Plastic and Reconstructive, Washington University School of Medicine, St. Louis Children's Hospital, MO, USA.
Cheng
C J
CJ
Mallory
S B
SB
Lind
A C
AC
D002648:Child; D003286:Contracture / Q000209:etiology / Q000601:surgery*; D000078404:Epidermal Cells; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications / Q000378:metabolism / Q000601:surgery*; D006225:Hand / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D016038:Skin Transplantation / Q000379:methods* / Q000473:pathology* / Q000502:physiology; D014184:Transplantation, Homologous