Lethal epidermolysis bullosa in black South African neonates.

BACKGROUND

While there have been a number of reports of epidermolysis bullosa (EB) occurring in South African black neonates, the disorder occurs rarely and the electron-microscopic findings in affected South African patients have not been documented previously.

METHODS

All patients with neonatal EB who were biopsied for electron microscopy at the South African Institute for Medical Research between 1986 and 1992 were included in the study. The eight boys and three girls had extensive blistering and all died within the first few months of life.

RESULTS

Nine of the patients showed the features of junctional EB, whereas the remaining two were classified as dystrophic EB. There were no differences in the clinical manifestations between the two categories.

CONCLUSIONS

The lethal form of EB in South African black neonates is associated with at least two types of basement membrane defect.