Disease Information for Paraneoplastic Pemphigus

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

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


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