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Understanding and Treating Paraneoplastic Diseases
Monday, March 24
1:00 PM - 3:00 PM
Sergei A. Grando, MD, PhD, FAAD - Handout
Following this course, the attendee should be able to:
- Identify clinical variants of the paraneoplastic autoimmune multiorgan syndrome (PAMS)
- Recognize the pathophysiology of PAMS
- Manage patients with PAMS.
The objective is to characterize diverse clinical and immunopathological features of paraneoplastic autoimmune multiorgan syndrome (PAMS) known as paraneoplastic pemphigus (PNP). In fact, PNP represents only a single manifestation of a heterogeneous autoimmune syndrome, or PAMS, in which patients, in addition to small airway occlusion and deposition of autoantibodies in different organs, may display a spectrum of at least five different clinical and immunopathological mucocutaneous variants (i.e., pemphigus-like, pemphigoid-like, erythema multiforme-like, graft-vs-host disease-like, and lichen planus-like). The pathophysiological mechanisms of PAMS involve both humoral and cellular autoimmunity responses. Treatment is difficult and prognosis is doomed.