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.
Rekhtman
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N
Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA. nrekhtm1@jhmi.edu
Hash
K S
KS
Moresi
J M
JM
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