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

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.