Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
8715527 | Journal of the American Academy of Dermatology | 2017 | 14 Pages |
Abstract
Cutaneous manifestations secondary to paraprotein deposits in the skin include a group of different disorders that although rare, may be the first clinical manifestation of the underlying hematologic dyscrasia. In this article we review the clinical manifestations and histopathologic findings of the processes that result from specific deposition of the paraprotein in different structures of the skin. Paraneoplastic processes frequently associated with hematologic malignancies will not be covered in this review. Some of the disorders included here result from deposition of the intact paraprotein in the skin, whereas in other cases the lesions are due to deposition of modified paraproteins in the form of amyloid substance, cryoglobulins, or crystalglobulins. Cutaneous amyloidoma refers to nodular dermal deposits of amyloid derived from immunoglobulin light chains produced by local plasma cells in the absence of systemic amyloidosis. Dermatologists and dermatopathologists should be aware of the clinical and histopathologic features of these rare disorders because sometimes the cutaneous lesions are the first sign of an underlying silent hematologic malignancy with paraproteinemia.
Keywords
RAECSHLCDDHeavy chain deposition diseaseCryoglobulinemiaReactive angioendotheliomatosisAngioendotheliomatosisAmyloidosisperiodic acid–Schiffimmunoglobulin Light chain deposition diseaseAmyloid light chainLight chainsWaldenström macroglobulinemiaMultiple myelomaPASCrystal-storing histiocytosisParaproteinCryoglobulins
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Authors
Victoria MD, Lorenzo MD, Heinz MD, Luis MD,