Abstract
We report a woman with recessive dystrophic epidermolysis bullosa (RDEB) in whom there was prolonged sepsis and death at age 22 years. Autopsy revealed multiple epidermolytic skin lesions with chronic ulceration, mesangioproliferative glomerulonephritis and multifocal necrotizing leucoencephalopathy (MNL) of the pons. The latter two conditions may have been mediated by sepsis-associated cytokines. Although mesangioproliferative glomerulonephritis has previously been described in association with RDEB, to our knowledge this is the first report of MNL in a patient with RDEB.
Original language | English (US) |
---|---|
Pages (from-to) | 1266-1269 |
Number of pages | 4 |
Journal | British Journal of Dermatology |
Volume | 151 |
Issue number | 6 |
DOIs | |
State | Published - Dec 2004 |
Externally published | Yes |
Keywords
- Cytokines
- Mesangioproliferative glomerulonephritis
- Multifocal necrotizing leucoencephalopathy
- Recessive dystrophic epidermolysis bullosa
- Sepsis
ASJC Scopus subject areas
- Dermatology