Paraneoplastic Pemphigus Is a Life-Threatening Disease

Abstract

Paraneoplastic pemphigus is a multiorganic autoimmune disease, usually triggered by neoplasias, mainly of lymphoproliferative origin such as chronic lymphocytic leukemia, multiple myeloma, non-Hodgkin's lymphoma, Castleman disease, and thymoma. This disorder is characterized by the presence of autoantibodies that react against proteins, such as desmoplakins, desmocollins, and others existing in cell junctions. The prognosis is reserved, and the mortality rate of the disease is very high, thus proving to be an additional challenge in the therapeutic management of onco-hematological diseases. The objective of this chapter is to solve the main clinical aspects of paraneoplastic pemphigus in lymphoproliferative hematological diseases, anatomopathological and immunofluorescence characteristics, as well as associations with the main differential diagnoses and therapeutic management. We will also describe the main differential diagnoses of paraneoplastic pemphigus, such as various types of pemphigus including induced drug, bullous pemphigoid, drug eruption, lichen planus, graft versus host disease, erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. In addition, the prognosis and quality of life will be mentioned.

A case of pemphigus vulgaris in a patient with abdominal tumor as collaborative disease

“…This can occur due to damage or loss of intercellular adhesion due to IgG autoantibodies, sometimes IgA and IgM especially to desmoglein 3, but also can be desmoglein 1 as well, causing the release of epithelial cells known as acantholysis. 4, 5 The goal of treatment in PV is to stop the progression of the disease, and speed up the wound healing process. The first-line therapy in PV is corticosteroids and combination with other immunosuppressive agents may be considered to minimize the side effects of corticosteroids.…”