- Differential Diagnosis
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- Disease Information
- Disease Comparison
Disease Processes ▼
- Auto Immune
- Infected Organ-Abcess
- Infectious agent
- Poison Agent
- Poisoned Organ
- Structural-Anatomic-Foreign body
- Surgical Procedure-Complication
Major Organs-Systems ▼
- Nervous & Sensory System (Neurology)
- Cardiovascular System
- Respiratory (Pulmonary) System
- Gastro-Intestinal (Digestive) System
- Urinary System
- Dermatologic System
- Endocrine System
- Immune System
- Musculoskeletal System
- Genital Reproductive System
- Hematopoietic System (Hematology)
- Lymphatic System
Disease Information for Paraneoplastic Pemphigus
Available only to registered users.
- 3 possible findings found
- 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)