Chronic intestinal pseudo-obstruction in two patients. Overlap of features of systemic sclerosis and visceral myopathy

Two patients with chronic intestinal pseudo-obstruction are reported, one of whom had definite systemic sclerosis while the other had certain manifestations of the disease. Biopsies of the small intestine revealed increased collagen and elastic fibres in the muscularis propria and subserosa in each case, while ganglion cells were normal. In addition, one patient had severe atrophy of the inner muscle layer and the other obvious vacuolation of the muscularis propria. Ultrastructural examination confirmed degeneration of the smooth muscle fibres. These latter features have been considered the hallmark of visceral myopathy and their finding in this patient therefore suggests that the different causes of pseudo-obstruction may not be completely demarcated and cases in which the features overlap may occur.