Document Detail

Recessive dystrophic epidermolysis bullosa associated with mesangioproliferative glomerulonephritis and multifocal necrotizing leucoencephalopathy of the pons.
MedLine Citation:
PMID:  15606525     Owner:  NLM     Status:  MEDLINE    
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.
T D Lehman; T K Morgan; N L Lehman; H Vogel; J S McGuire
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The British journal of dermatology     Volume:  151     ISSN:  0007-0963     ISO Abbreviation:  Br. J. Dermatol.     Publication Date:  2004 Dec 
Date Detail:
Created Date:  2004-12-20     Completed Date:  2005-04-13     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0004041     Medline TA:  Br J Dermatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  1266-9     Citation Subset:  IM    
Department of Dermatology, Stanford University Medical Center, Stanford, CA 94305, U.S.A.
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MeSH Terms
Epidermolysis Bullosa Dystrophica / complications*,  pathology
Fatal Outcome
Glomerulonephritis, Membranoproliferative / etiology*,  pathology
Leukoencephalopathy, Progressive Multifocal / etiology*,  pathology
Pons / pathology

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