LE VARIETA CLINICHE DELLA SCLEROSI SISTEMICA PROGRESSIVA NEL SESSO MASCHILE

This is the report of 4 male cases of progressive systemic sclerosis (PSS) observed in a period of 13 months; the shortness of this period has exceptionaly made possible a comparative study which should be exempt from biases typically affecting retrospective comparisons. Generally women predominate among patients affected with PSS, but our series of male cases stands out because everyone of these patients represents a distinct type of the disease, too: according to Holzmann's classification, we can distinguish one type II, one type IV, and two types III, one of these latter is associated with lung silicosis. This seems to be an atypical form of Erasmus' syndrome, because this patient shows signs that could be related to an overlap syndrome. Besides, every patient out of these four presents a different evolutive pattern of the disease (2 acute progressing forms, and 2 insidiously developed chronic ones). Although we cannot affirm that male response to symptomatic treatments - and particularly to ketanserin and ketotifen - is different from that observed in female patients (in fact the sample is too small, and follow-up is yet too short), anyway PSS in male patients does not seem to be unlike the female disease, either clinically or biological, if we overlook a remarkable tendency of men to develop precociously profuse macular and/or annular teleangiectasies on face skin.