Ms. Lin et al., Desmoglein-1-specific T lymphocytes from patients with endemic pemphigus foliaceus (fogo selvagem), J CLIN INV, 105(2), 2000, pp. 207-213
Pogo selvagem (FS), the endemic form of pemphigus foliaceus, is a cutaneous
autoimmune disease characterized by subcorneal blistering of the epidermis
and the production of autoantibodies against the desmosomal antigen desmog
lein-1 (Dsg1). Previously, we showed that mice injected with autoantibodies
from FS patients develop a skin disease that reproduces the clinical, hist
ological, and immunological features of FS, indicating that autoantibodies
play an essential role in the development of this disease. The purpose of t
his study was to characterize the autoimmune T-cell response associated wit
h FS. We provide here the first evidence, to our knowledge, that the great
majority of FS patients have circulating T lymphocytes that specifically pr
oliferate in response to the extracellular domain of Dsg1. Long-term T cell
s developed from these patients also responded to Ds,ol, and this antigen-s
pecific response was shown to be restricted to HLA-DR molecules. These Dsg1
-reactive FS T cells exhibited a CD4-positive memory T-cell phenotype and p
roduced a T helper 2-like cytokine profile. These findings represent the in
itial steps in defining the role of T cells in FS autoimmunity.