Fatal recurrent dermatoneuro syndrome associated with systemic AL amyloidosis

A male patient is presented with long-lasting paraproteinemia of monoclonal IgG λ, who suffered from recurrent, and until the last one, mostly reversible episodes of dermatoneuro syndrome, described exclusively in scleromyxedema. The skin biopsy revealed λ-light chain amyloid deposition instead of c...

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Bibliographic Details
Published in:	Clinical neuropathology Vol. 35; no. 2; p. 72
Main Authors:	Leonardis, Lea, Pihler, Dominika Novak, Zupan, Matija, Vizjak, Alenka, Belcijan, Neza Lebic, Jurcic, Vesna, Popovic, Mara
Format:	Journal Article
Language:	English
Published:	Germany 01-03-2016
Subjects:	Aged Amyloidosis - complications Amyloidosis - pathology Brain Diseases - etiology Brain Diseases - pathology Fatal Outcome Humans Immunoglobulin Light-chain Amyloidosis Male Middle Aged Skin - pathology Syndrome
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Summary:	A male patient is presented with long-lasting paraproteinemia of monoclonal IgG λ, who suffered from recurrent, and until the last one, mostly reversible episodes of dermatoneuro syndrome, described exclusively in scleromyxedema. The skin biopsy revealed λ-light chain amyloid deposition instead of changes typical for scleromyxedema. Systemic AL amyloidosis was diagnosed post mortem since the patient had no clinical signs of any other organ impairment except skin and brain. Neuropathology is described and possible etiopathogenesis of brain involvement is considered.
ISSN:	0722-5091
DOI:	10.5414/NP300924