Paraneoplastic Pemphigus: A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?

Table 1

Paraneoplastic pemphigus: original diagnostic criteria of Anhalt et al. [7].

(1) Painful mucosal erosions and a polymorphous skin eruption in the context of an occult or known neoplasm generating a spectrum of histological features
(2) Intraepidermal acantholysis, dyskeratosis/keratinocyte necrosis, and vacuolar interface changes in histopathology
(3) Deposition of IgG and complement in intercellular epidermal and basement membrane zone seen on direct immunofluorescence
(4) Detection of serum autoantibodies to stratified squamous epithelia, columnar, and transitional epithelia by indirect immunofluorescence
(5) Serum immunoprecipitation of a characteristic complex of four proteins (250, 230, 210, and 190 kDa) from keratinocytes transitional epithelia by indirect immunofluorescence