Overview
Paraneoplastic pemphigus (PNP), also sometimes called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare and serious blistering disease that happens when the immune system attacks the skin and mucous membranes in response to an underlying tumor or cancer. The word 'paraneoplastic' means the condition is triggered by a cancer elsewhere in the body, not by a direct infection or injury to the skin itself. The immune system makes abnormal proteins called autoantibodies that mistakenly attack the tissues that hold skin cells together, causing painful blisters, sores, and peeling. The disease most commonly affects the mouth, lips, throat, and eyes, making it very hard to eat, drink, or swallow. Blisters can also appear on the skin of the body. In many cases, the lung lining becomes inflamed, which can cause serious breathing problems. The condition is strongly linked to blood cancers such as non-Hodgkin lymphoma, chronic lymphocytic leukemia, and Castleman disease, though other tumors can also trigger it. Treatment focuses on two main goals: treating the underlying cancer and calming the overactive immune system. Doctors use corticosteroids, immunosuppressive drugs, and sometimes rituximab to reduce the immune attack. Even with treatment, paraneoplastic pemphigus is a very serious condition with a high risk of life-threatening complications, especially lung disease. Early diagnosis and prompt treatment of the associated tumor offer the best chance of improvement.
Key symptoms:
Painful sores and blisters in the mouth and on the lipsBlisters or raw areas on the skin of the bodyDifficulty swallowing due to sores in the throatRed, inflamed, or crusted eyelids and eye irritationShortness of breath or difficulty breathingPeeling or sloughing of the skinSores inside the nose or on the genitalsFatigue and general feeling of being unwellWeight loss linked to difficulty eatingHoarse voice or throat pain
Clinical phenotype terms (7)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Paraneoplastic pemphigus.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Paraneoplastic pemphigus.
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Caregiver Resources
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Questions for your doctor
Bring these to your next appointment
- Q1.What type of tumor or cancer is causing my paraneoplastic pemphigus, and what are my treatment options for it?,Which autoantibodies were found in my blood tests, and what do they tell us about my prognosis?,What is my risk of developing lung disease (bronchiolitis obliterans), and how will you monitor for it?,Which immunosuppressive medications do you recommend for me, and what are the main side effects I should watch for?,If the cancer is treated successfully, is there a chance my skin and mouth symptoms will improve?,Are there any clinical trials for paraneoplastic pemphigus that I might be eligible for?,What warning signs should prompt me to go to the emergency room immediately?
Common questions about Paraneoplastic pemphigus
What is Paraneoplastic pemphigus?
Paraneoplastic pemphigus (PNP), also sometimes called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare and serious blistering disease that happens when the immune system attacks the skin and mucous membranes in response to an underlying tumor or cancer. The word 'paraneoplastic' means the condition is triggered by a cancer elsewhere in the body, not by a direct infection or injury to the skin itself. The immune system makes abnormal proteins called autoantibodies that mistakenly attack the tissues that hold skin cells together, causing painful blisters, sores, and peeling. The
How is Paraneoplastic pemphigus inherited?
Paraneoplastic pemphigus follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Paraneoplastic pemphigus typically begin?
Typical onset of Paraneoplastic pemphigus is adult. Age of onset can vary across affected individuals.
Which specialists treat Paraneoplastic pemphigus?
1 specialists and care centers treating Paraneoplastic pemphigus are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.