Paraneoplastic pemphigus (PNP) is a rare and life-threatening autoimmune bullous disease that classically occurs in the setting of an underlying neoplasm. PNP is also known as paraneoplastic autoimmune multiorgan syndrome (PAMS) because of its varied mucocutaneous and systemic deleterious effects. Due to the variable disease characteristics and lack of formally accepted diagnostic criteria, PNP remains a diagnostic challenge for clinicians.
Paraneoplastic
Diagnostic Criteria of Paraneoplastic Neurological Disorder (PND) of the CNS
Definite PND
- Classic syndrome with cancer diagnosed within 5 years of neurological symptom development
- Nonclassic syndrome that resolves or significantly improves after cancer treatment
- Nonclassic syndrome with cancer diagnosed within 5 years of neurological symptom development and positive antineuronal antibodies
- Neurological syndrome (classic or not) without cancer and with well-characterized antineuronal antibodies