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 kappa monoclonal gammopathy. The amyloid plaques were composed of an unusual mixture of immunoglobulin kappa 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.
Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA. nrekhtm1@jhmi.edu
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N
Rekhtman
K S
KS
Hash
J M
JM
Moresi
D000369:Aged, 80 and over; D000686:Amyloidosis / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D001768:Blister / Q000209:etiology; D004935:Esophageal Diseases / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D010265:Paraproteinemias / Q000150:complications; D010608:Pharyngeal Diseases / Q000209:etiology; D000685:Serum Amyloid A Protein / Q000032:analysis; D012872:Skin Diseases, Vesiculobullous / Q000150:complications / Q000378:metabolism* / Q000473:pathology
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D000685:Serum Amyloid A Protein