How is paraneoplastic pemphigus diagnosed?
No formally accepted diagnostic criteria exist for paraneoplastic pemphigus. [40] Diagnostic confirmation is typically by a combination of clinical presentation, histology, direct and indirect immunofluorescence microscopy, and immune serology.
What is paraneoplastic pemphigus?
Paraneoplastic pemphigus (PNP) is an often fatal paraneoplastic mucocutaneous blistering disease that is most commonly induced by lymphoproliferative disorders [1]. Paraneoplastic autoimmune multiorgan syndrome (PAMS) is an alternative term used to refer to PNP.
What cancers cause paraneoplastic pemphigus?
Hematologic malignancies are most commonly associated with paraneoplastic pemphigus, although it can also be associated with carcinomas, sarcomas, and benign neoplasms.
What drugs cause pemphigus?
Drugs that cause pemphigus include:
- Thiol drugs, including penicillamine, captopril.
- Antibiotics: penicillins, cephalosporins, vancomycin.
- Antihypertensive drugs: other angiotensin-converting enzyme inhibitors such as cilazapril, lisinopril, enalapril.
- Piroxicam.
How do you treat paraneoplastic pemphigus?
High-dose corticosteroids are first-line therapy for paraneoplastic pemphigus, followed by steroid-sparing agents such as azathioprine, cyclosporine, and mycophenolate mofetil. In general, the skin lesions of paraneoplastic pemphigus are more responsive to therapy than mucosal lesions.
How does pemphigus start?
Pemphigus vulgaris.
This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. The blisters typically are painful but don’t itch. Blisters in your mouth or throat may make it hard to swallow and eat.
What triggers pemphigus?
What causes pemphigus vulgaris? Pemphigus vulgaris is not fully understood. Experts believe that it’s triggered when a person who has a genetic tendency to get this condition comes into contact with an environmental trigger, such as a chemical or a drug.
What drugs can cause pemphigus?
How long can you live with pemphigus?
Today, the outcome looks good. For most people, the disease can be controlled with treatment. Many can eventually stop their treatment for a while. Before medicines like prednisone and azathioprine were used to treat pemphigus, a person lived about 5 years after getting pemphigus vulgaris, the most common type.
Where does pemphigus usually start?
Pemphigus vulgaris blisters often start in the mouth, but later on, they can develop on the skin. The skin may become so fragile that it peels off by rubbing a finger on it. Mucosal surfaces such as those of the nose, throat, eyes, and genitals may also be affected.
Who usually gets pemphigus?
Middle-aged or older people are most likely to get pemphigus, including the most common type, pemphigus vulgaris. This type usually begins between 50 and 60 years of age. Your risk of getting pemphigus vulgaris increases if you have: Jewish ancestry, especially Ashkenazi Jewish heritage.
What are the early signs of pemphigus?
Symptoms of Pemphigus
The main symptom of pemphigus is blistering of the skin and in some cases, the mucosal surfaces, such as the inside of the mouth, nose, throat, eyes, and genitals. The blisters are fragile and tend to burst, causing crusty sores.