Progressive systemic sclerosis (PSS) is characterized in most patients
by initially unspecific symptoms like acrocyanosis, Raynaud's phenome
non, general malaise or acral paresthesia. Clinical manifestations app
ear with an edematous-inflammatory stage of disease leading to a chron
ic-progressive phase with increasing fibrosclerosis of the skin and in
ternal organs. Dermal involvement and the demonstration of immunopatho
logical abnormalities in the peripheral blood precede visceral fibrosi
s in most cases. We report on an unusual case of diffuse PSS with prec
eding involvement of internal organs. Early visceral fibrosis is demon
strated by histomorphological results and is correlated with laborator
y findings. Serum antinuclear antibodies could not be obtained until 4
months ante finem and the liver was also involved in the fibrotic pro
cess.