Recessive dystrophic epidermolysis bullosa associated with mesangioproliferative glomerulonephritis and multifocal necrotizing leucoencephalopathy of the pons

T. D. Lehman, T. K. Morgan, Norman L. Lehman, H. Vogel, J. S. McGuire

Research output: Contribution to journalArticle

4 Scopus citations

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 languageEnglish (US)
Pages (from-to)1266-1269
Number of pages4
JournalBritish Journal of Dermatology
Volume151
Issue number6
DOIs
StatePublished - Dec 1 2004

Keywords

  • Cytokines
  • Mesangioproliferative glomerulonephritis
  • Multifocal necrotizing leucoencephalopathy
  • Recessive dystrophic epidermolysis bullosa
  • Sepsis

ASJC Scopus subject areas

  • Dermatology

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