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Original

Paraneoplastic autoimmune multiorgan syndrome: Review of the literature and support for a cytotoxic role in pathogenesis

, &
Pages 617-630 | Published online: 07 Jul 2009
 

Abstract

Paraneoplastic autoimmune multiorgan syndrome (PAMS), first described as paraneoplastic pemphigus in 1990, is an autoimmune blistering disease associated with neoplasia. Patients with this rare disorder have severe blistering and painful erosions of the oral cavity and various other cutaneous findings ranging from classic pemphigus vulgaris-like erosions to targetoid lesions resembling erythema multiforme and papular to more confluent lichenoid eruptions. This syndrome involves multiple organ systems, and its high rate of mortality often stems from constrictive bronchiolitis obliterans. The histologic findings are as diverse as the clinical presentation, often making diagnosis difficult initially. Immunodermatologic and serologic laboratory findings typically establish the diagnosis. These results can be confirmed with immunoprecipitation profiling of specific molecular weight protein markers. The proposed pathogenesis of PAMS continues to evolve, and recent reports implicate the involvement of cell-mediated, cytotoxic immunity, in addition to humoral autoantibodies. This review characterizes and summarizes the clinical, pathologic, and immunohistologic features of PAMS and outlines the possible role of cytotoxic T lymphocytes in the pathogenesis of this syndrome.

Abbreviations
CTL=

cytotoxic T lymphocyte

DIF=

direct immunofluorescence

DSG=

desmoglein

GVHD=

graft-versus-host disease

Ig=

immunoglobulin

IIF=

indirect immunofluorescence

INF-γ=

interferon γ

iNOS=

inducible nitric oxide synthase

IVIg=

intravenous immunoglobulin

MHC=

major histocompatibility complex

NK cells=

natural killer cells

PAMS=

paraneoplastic autoimmune multiorgan syndrome

PNP=

paraneoplastic pemphigus

Abbreviations
CTL=

cytotoxic T lymphocyte

DIF=

direct immunofluorescence

DSG=

desmoglein

GVHD=

graft-versus-host disease

Ig=

immunoglobulin

IIF=

indirect immunofluorescence

INF-γ=

interferon γ

iNOS=

inducible nitric oxide synthase

IVIg=

intravenous immunoglobulin

MHC=

major histocompatibility complex

NK cells=

natural killer cells

PAMS=

paraneoplastic autoimmune multiorgan syndrome

PNP=

paraneoplastic pemphigus

Acknowledgement

Editing, proofreading, and reference verification were provided by the Section of Scientific Publications, Mayo Clinic.

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