Spontaneous pneumoperitoneum in progressive systemic sclerosis (generalized scleroderma)

In a twenty-nine year old female patient with progressive systemic sclerosis (scleroderma) (PSS) and an impressing involvement of the intestine (disturbance of the oesophageal motility, pseudo-obstruction, malabsorption) a spontaneous, asymptomatic pneumoperitoneum is observed. Perforation or pneumatosis cystoides intestinalis (PCI) could not be proved. The possible pathogenesis, course, prognosis and therapy of this rare complication are discussed.