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Mucocutaneous bullous amyloidosis with an unusual mixed protein composition of amyloid deposits

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Abstract:

Summary

We describe a case of fatal systemic amyloidosis presenting with mucocutaneous bullous lesions in a patient with IgA κ monoclonal gammopathy. The amyloid plaques were composed of an unusual mixture of immunoglobulin κ light chain and amyloid A proteins. Whereas oesophageal and oropharyngeal blisters are known to occur in several types of bullous dermatoses, to our knowledge this is the first report of oesophagopharyngeal blisters complicating bullous amyloidosis.

Keywords: amyloid A; bullous amyloidosis; immunoglobulin; oesophagus

Document Type: Research Article

DOI: http://dx.doi.org/10.1111/j.1365-2133.2005.07063.x

Affiliations: Division of Dermatopathology, Department of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, MD, 21287, U.S.A.

Publication date: April 1, 2006

bsc/bjd/2006/00000154/00000004/art00028
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