Isolated primary cardiac amyloidosis associated with porokeratosis of Mibelli.
In this report we briefly describe a 54-year-old woman who was referred to our institution for evaluation and management of newly diagnosed congestive heart failure associated with a skin rash. Detailed investigations revealed the presence of restrictive cardiomyopathy due to isolated primary cardiac amyloidosis as well as the presence of a skin disease named 'porokeratosis of Mibellli'. Interestingly, porokeratotic lesions rarely have been associated with localized cutaneous amyloidosis. Presumably, porokeratosis induces secondary dermal amyloid deposition by a yet unknown mechanism. This is the first case of primary amyloidosis associated with porokeratosis reported in the literature.
Protopsaltis
John
J
Katsantonis
John C
JC
Kokkoris
Stelios
S
Agapitos
Emmanuel
E
Lavranos
Giagkos
G
Korantzopoulos
Panagiotis
P
Giannoulis
Gregory
G
D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D005260:Female; D006333:Heart Failure / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D017499:Porokeratosis / Q000150:complications / Q000175:diagnosis* / Q000235:genetics