TY - JOUR
T1 - Paraneoplastic autoimmune multiorgan syndrome (PAMS)
AU - Didona, Dario
AU - Di Zenzo, Giovanni
AU - Joly, Pascal
PY - 2020/10/16
Y1 - 2020/10/16
N2 - Originally described by Anhalt as paraneoplastic pemphigus in 1990, paraneoplastic autoimmune multiorgan syndrome (PAMS) is a potentially lethal blistering disease, characterized by polymorphous clinical features, including mucocutaneous erosions, blisters, lichenoid papules, and erythema. Several autoantibodies have been detected in serum of PAMS patients, including anti-plakins, anti-alpha-2-macroglobulin like 1, and anti-desmogleins autoantibodies. The mortality rate of PAMS is up to 50%. This is due on the one hand to the poor response to treatments and on the other hand to the delay in the diagnosis and to the prognosis of the underlying neoplasia.
AB - Originally described by Anhalt as paraneoplastic pemphigus in 1990, paraneoplastic autoimmune multiorgan syndrome (PAMS) is a potentially lethal blistering disease, characterized by polymorphous clinical features, including mucocutaneous erosions, blisters, lichenoid papules, and erythema. Several autoantibodies have been detected in serum of PAMS patients, including anti-plakins, anti-alpha-2-macroglobulin like 1, and anti-desmogleins autoantibodies. The mortality rate of PAMS is up to 50%. This is due on the one hand to the poor response to treatments and on the other hand to the delay in the diagnosis and to the prognosis of the underlying neoplasia.
U2 - 10.23736/S0392-0488.20.06675-4
DO - 10.23736/S0392-0488.20.06675-4
M3 - Article
C2 - 33070576
JO - G Ital Dermatol Venereol
JF - G Ital Dermatol Venereol
SN - 1827-1820
ER -