[Initial visceral involvement with diffuse systemic scleroderma].
Progressive systemic sclerosis (PSS) is characterized in most patients by initially unspecific symptoms like acrocyanosis. Raynaud's phenomenon, general malaise or acral paresthesia. Clinical manifestations appear with an edematous-inflammatory stage of disease leading to a chronic-progressive phase with increasing fibrosclerosis of the skin and internal organs. Dermal involvement and the demonstration of immunopathological abnormalities in the peripheral blood precede visceral fibrosis in most cases. We report on an unusual case of diffuse PSS with preceding involvement of internal organs. Early visceral fibrosis is demonstrated by histomorphological results and is correlated with laboratory findings. Serum antinuclear antibodies could not be obtained until 4 months ante finem and the liver was also involved in the fibrotic process.
Gruschwitz
M S
MS
Dermatologische Universitätsklinik, Erlangen.
Steffan
C
C
Albrecht
H P
HP
Hornstein
O P
OP
D000974:Antibodies, Antinuclear / Q000032:analysis; D003094:Collagen / Q000032:analysis; D017809:Fatal Outcome; D005355:Fibrosis; D006801:Humans; D007124:Immunoenzyme Techniques; D007668:Kidney / Q000473:pathology; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000473:pathology; D008168:Lung / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D011928:Raynaud Disease / Q000175:diagnosis / Q000473:pathology; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000473:pathology; D012598:Sclerosis; D012867:Skin / Q000473:pathology; D014781:Viscera / Q000473:pathology
D000974:Antibodies, Antinuclear; D003094:Collagen
Initialer viszeraler Befall bei diffuser systemischer Sklerodermie.