Paraneoplastic pemphigus
Paraneoplastic Pemphigus (pronounced: para-neo-plastic pem-phi-gus) is a rare, multiorgan autoimmune blistering disorder associated with malignancy.
Etymology
The term "Paraneoplastic" is derived from the Greek words "para" meaning "beside" and "neoplasia" meaning "new growth", referring to the condition's association with cancer. "Pemphigus" is derived from the Greek word "pemphix" meaning "bubble or blister", describing the skin condition's primary symptom.
Definition
Paraneoplastic Pemphigus is a severe form of pemphigus, characterized by painful blisters and erosions on the skin and mucous membranes. It is often associated with underlying neoplasms, most commonly non-Hodgkin's lymphoma and Castleman's disease.
Symptoms
The symptoms of Paraneoplastic Pemphigus include painful sores in the mouth, lips, and on the skin; polymorphous skin eruptions; and conjunctivitis. In some cases, the condition may also affect the lungs, leading to bronchiolitis obliterans, a severe lung disease.
Diagnosis
Diagnosis of Paraneoplastic Pemphigus is based on clinical features, histopathology, immunofluorescence studies, and the presence of autoantibodies against various components of the plakin family.
Treatment
Treatment of Paraneoplastic Pemphigus involves managing the underlying malignancy and controlling the blistering and erosive skin disease. This often includes systemic corticosteroids, immunosuppressive agents, and in some cases, plasmapheresis or intravenous immunoglobulin (IVIG).
Prognosis
The prognosis of Paraneoplastic Pemphigus is generally poor due to the severity of the condition and its association with malignancies. The mortality rate is high, often due to sepsis, malignancy, or respiratory failure.
See Also
External links
- Medical encyclopedia article on Paraneoplastic pemphigus
- Wikipedia's article - Paraneoplastic pemphigus
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