Cutaneous amyloidoses and systemic amyloidoses with cutaneous involvement

Schreml, Stephan and Szeimies, Rolf-Markus and Vogt, Thomas and Landthaler, Michael and Schroeder, Josef and Babilas, Philipp (2010) Cutaneous amyloidoses and systemic amyloidoses with cutaneous involvement. EUROPEAN JOURNAL OF DERMATOLOGY, 20 (2). pp. 152-160. ISSN 1167-1122, 1952-4013

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Abstract

Extracellular deposition of altered autologous protein (amyloid protein) within the dermis is the hallmark of cutaneous amyloidoses and systemic amyloidoses with cutaneous involvement. Amyloidoses may be acquired or hereditary in nature and subclassification differentiates between primary amyloidosis (no obvious predisposing disease) and secondary amyloidosis (specific underlying disease). More than 26 different proteins and peptides have been identified as amyloid precursors and these proteins are used to subclassify this heterogenous group of diseases. The amyloid proteins show an anti-parallel beta-sheet conformation and form non-branching linear filaments of variable lengths and diameters of approximately 7.5 to 10 nm. However, the exact etiopathogenesis of amyloid formation still remains unclear. Depending on histo-anatomical distribution and amount, amyloid may cause progressive and life-threatening organ dysfunction. Clinical presentation, histology, electron microscopy, and biochemical-immunological differentiation represent decisive tools for an accurate diagnosis.

Item Type: Article
Uncontrolled Keywords: NODULAR AMYLOIDOSIS; LICHEN AMYLOIDOSIS; BULLOUS AMYLOIDOSIS; TRANSTHYRETIN AMYLOIDOSIS; FLUORESCENCE MICROSCOPY; SECONDARY AMYLOIDOSIS; FAMILIAL AMYLOIDOSIS; TERM HEMODIALYSIS; FAT TISSUE; CONGO RED; amyloidosis; hereditary; non-hereditary
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Dermatologie und Venerologie
Medicine > Lehrstuhl für Pathologie
Depositing User: Petra Gürster
Date Deposited: 14 Apr 2020 09:06
Last Modified: 14 Apr 2020 09:06
URI: https://pred.uni-regensburg.de/id/eprint/25060

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