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  • Title: Isolated primary cardiac amyloidosis associated with porokeratosis of Mibelli.
    Author: Protopsaltis J, Katsantonis JC, Kokkoris S, Agapitos E, Lavranos G, Korantzopoulos P, Giannoulis G.
    Journal: Int J Cardiol; 2008 May 23; 126(2):e22-4. PubMed ID: 17397946.
    Abstract:
    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.
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