Isolated primary cardiac amyloidosis associated with porokeratosis of Mibelli.

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.

• Authors: John Protopsaltis, John C Katsantonis, Stelios Kokkoris, Emmanuel Agapitos, Giagkos Lavranos, Panagiotis Korantzopoulos, Gregory Giannoulis
• Journal: International journal of cardiology (Int J Cardiol) Vol. 126 Issue 2 Pg. e22-4 (May 23 2008) ISSN: 1874-1754 [Electronic] Netherlands
• PMID: 17397946 (Publication Type: Case Reports, Letter)

Topics

• Amyloidosis (complications, diagnosis, genetics)
• Female
• Heart Failure (complications, diagnosis, genetics)
• Humans
• Middle Aged
• Myocardium (pathology)
• Porokeratosis (complications, diagnosis, genetics)