Cutaneous amyloidoses and systemic amyloidoses with cutaneous involvement.
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 heterogeneous 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 histoanatomical 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.
Schreml
Stephan
S
Department of Dermatology, University Hospital Regensburg, Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany. stephan.schreml@klinik.uni-regensburg.de
Szeimies
Rolf-Markus
RM
Vogt
Thomas
T
Landthaler
Michael
M
Schroeder
Josef
J
Babilas
Philipp
P
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000682:Amyloid / Q000032:analysis; D000686:Amyloidosis / Q000145:classification / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D003879:Dermatologic Agents / Q000627:therapeutic use; D006801:Humans; D010348:Patient Care Team; D044469:Racial Groups; D012867:Skin / Q000473:pathology; D012873:Skin Diseases, Genetic / Q000145:classification / Q000175:diagnosis* / Q000628:therapy; D012875:Skin Diseases, Metabolic / Q000145:classification / Q000175:diagnosis* / Q000628:therapy
D000305:Adrenal Cortex Hormones; D000682:Amyloid; D003879:Dermatologic Agents