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

• Published in: British journal of dermatology (1951) Vol. 154; no. 4; pp. 751 - 754
• Main Authors: Rekhtman, N., Hash, K.S., Moresi, J.M.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01-04-2006; Blackwell; Oxford University Press
• Subjects: Aged, 80 and over; amyloid A; Amyloidosis; Amyloidosis - complications; Amyloidosis - metabolism; Amyloidosis - pathology; Biological and medical sciences; Blister - etiology; bullous amyloidosis; Bullous diseases of the skin; Dermatology; Esophageal Diseases - etiology; Fatal Outcome; Female; Humans; immunoglobulin; Immunoglobulin kappa-Chains - analysis; Immunoglobulin Light Chains - analysis; Medical sciences; Metabolic diseases; oesophagus; Other metabolic disorders; Paraproteinemias - complications; Pharyngeal Diseases - etiology; Serum Amyloid A Protein - analysis; Skin Diseases, Vesiculobullous - complications; Skin Diseases, Vesiculobullous - metabolism; Skin Diseases, Vesiculobullous - pathology; Human; Bullous dermatosis; Immunohistochemistry; Skin disease; Immunoglobulins; immunoglobulin; Dermatology; Metabolic diseases; Enzymopathy; Esophagus; Case study; Anatomic pathology; amyloid A; oesophagus; bullous amyloidosis; Amyloid; Amyloidosis; Mucocutaneous; Elderly
• ISSN: 0007-0963; 1365-2133
• DOI: 10.1111/j.1365-2133.2005.07063.x

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.