Bart syndrome, report of a case
1 – 1,000,000 CASE
HOSPITAL GENERAL DE LA ZONA NORTE DE PUEBLA
«Bicentenario de la Independencia»
PLASTIC SURGERY DEPARTMENT
Dystrophic Epidermolysis Bullosa + Aplasia Cutis.
Femalw 51 days age.
Consisted of any one or a combination of the following three characteristics: congenital absence of skin, blistering and associated nail abnormalities.
Caused by STRUCTURAL abnormalities in the anchoring fibrils. Genetic linkage of the inheritance of the disease points to the región of chromosome 3 near the collagen, type VII, alpha 1 gene (COL7A1)
FRECUENCY. 1-1,000,000 NB
The absense of skin was covered for 5 days with EPIFAST (live cultivated epidermal Keratinocytes), also the blisterings. The allografts of human epidermis cultured in vitro are an active biological dessing which contain human keratinocytes cultured in vitro.
Professor Department of Health Sciences, UDLAP
Hospital General Zona Norte Puebla, México