[A case of systemic Pfeiffer-Weber-Christian Syndrome complicated by DIC (author's transl)].

The authors report on a case of fatal systemic P-W-Chr syndrome accompanied by DIC. The coagulation disturbance of the patient stopped after the administration of heparin, but this had no effect on the prognosis of the illness. The basic illness that had caused the syndrome could not be demonstrated either in vivo, or during the autopsy so that the above case is thought to be a sui generis form. According to the author's opinion the DIC is not an inducing factor but an accompanying phenomenon in the course of the basic disease.