TY - CHAP M1 - Book, Section TI - Amyloidosis A1 - Gorevic, Peter D. A1 - Phelps, Robert G. A2 - Kang, Sewon A2 - Amagai, Masayuki A2 - Bruckner, Anna L. A2 - Enk, Alexander H. A2 - Margolis, David J. A2 - McMichael, Amy J. A2 - Orringer, Jeffrey S. Y1 - 2019 N1 - T2 - Fitzpatrick's Dermatology, 9e AB - AT-A-GLANCEThere are 36 types of amyloid disease, defined by the subunit proteins that constitute the fibrils in these disorders, 11 of which involve the skin.Cutaneous amyloidosis may reflect a systemic form of amyloid, or be localized to the skin and/or mucous membranes.Precursors of fibril subunit proteins may circulate in blood and/or be synthesized locally; they may be wildtype or mutant molecules that can be typed by DNA sequencing.Amyloid in skin may be sampled by lesional biopsy or abdominal fat pad aspiration or biopsy; it is characterized by metachromasia and apple-green birefringence after staining with Congo red, or yellow-green fluorescence after staining with thioflavin T.Deposits may localize to the papillary dermis (macular and lichen amyloidosis) or be characterized by congophilic angiopathy and involvement of adnexal structures (nodular amyloidosis).Amyloid can be typed by immunohistochemistry, immunoelectron microscopy, and/or laser capture mass spectroscopy followed by protein sequencing.Treatment of amyloid diseases is determined by the fibril subunit protein, associated clinical manifestations, and whether disease is systemic or localized.Treatment strategies include suppression of precursor protein, disruption of oligomers and/or fibrils, and/or enhanced clearance of deposits.Localized cutaneous amyloidoses present specific challenges and opportunities for diagnosis and therapy because of the accessibility of skin lesions, association in some cases with defined genetic abnormalities, and multifactorial etiologies for itch in pathogenesis. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - dermatology.mhmedical.com/content.aspx?aid=1161338505 ER -