Disease Information for Paraneoplastic Pemphigus

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Clinical Manifestations
Signs & Symptoms
Blisters
Bulla/bullous rash
Disease Mechanism & Classification
Process
PROCESS/Neoplastic (category)
Definition

A summary of criteria for the diagnosis of paraneoplastic pemphigus includes the following: Painful mucosal erosions, sometimes with a skin eruption that eventually results in blisters and erosions, in the setting of confirmed or occult malignancy ; Histopathologic changes of acantholysis, keratinocyte necrosis, and interface dermatitis; DIF observation of immunoreactants, typically IgG and complement (C3) within the epidermal intercellular spaces as well as at the epidermal basement membrane;

IDIF observation of circulating antibodies specific for stratified squamous or transitional epithelia (transitional epithelium); Immunoprecipitation of a complex of proteins with typical molecular weights as described in Other Tests;

Painful, progressive stomatitis; Histopathologic changes of acantholysis or lichenoid/interface dermatitis; Demonstration of antiplakin antibodies;Demonstration of an underlying lymphoproliferative neoplasm

Paraneoplastic pemphigus is an autoimmune disorder initiated by an underlying lymphoproliferative disorder; Tumor antigens are hypothesized to evoke both a humoral and a cellular immune response that leads to blistering in mucosa and other epithelia. Affected organ systems include the integument, the respiratory tract, and the gastrointestinal tract; It has also been reported to occur in association with benign tumors.

Mortality/Morbidity: Paraneoplastic pemphigus is often fatal, especially when associated with malignancy. Mortality rates approach 90%; Causes of death include sepsis, with resultant multiorgan failure and respiratory failure due to the direct effects of the disease on the respiratory epithelium; The latter is being increasingly recognized, and pulmonary involvement has been found to occur in approximately 30-40% of patients; The susceptibility to infection caused by the loss of skin integrity is exacerbated by the potent immunosuppressive medications used to treat the condition

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External Links Related to Paraneoplastic Pemphigus
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NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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