Visceral manifestations of scleroderma; patho-morphologic and clinical study of three cases, verified by autopsy
Gluck, E.; Humerfelt, S.
Annales d'Anatomie Pathologique 2(4): 529-547
1957
ISSN/ISBN: 0003-3871 PMID: 13498407 Document Number: 10381
The authors give an account of three cases of sclerodermia localised in the viscera. In two of these cases, the cutaneous affection was of the sclerodactylia type with a Raynand syndrome in one case together with subcutaneous and renal calcinosis. In the case of the third patient, the cutaneous signs did not suggest clinically the diagnosis of sclerodermia and it was only at the autopsy that the affection was recognised due tot the characteristic visceral lesions. The visceral manifestations in one case consisted of an atrio-ventricular blockage, due to a cardiac fibrosis. The two other patients died from terminal uremia and in their kidneys were found vascular lesions which are generally considered typical of renal sderodermic disease. The oesophagus was affected in the three cases. They go on to give a critical evaluation of the clinical picture and of the anatomopathological facts, and they discuss the pathogenesis of the visceral localisations of this disease.
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