[Variability of the clinical picture and the classification of progressive systemic scleroderma]

Abstract

Fiftythree patients with progressive systemic sclerosis were studied. Four of them (3 males) had the diffuse form of the disease. The skin manifestation of this clinical picture is characterized by diffuse progression of the cutaneous sclerosis over almost the whole body surface, except for the hands where it eventually may appear late. The prognosis for these patients it especially poor. Fortyfive patients (44 females) had acrosclerosis in the widest sence. Twentyseven of these ("acrosclerosis stricto sensu") had cutaneous sclerosis of the hands, face, and often other parts of the body, but not on the abdomen, arms or thighs. The remaining 18 patients had sclerotic alterations on these surfaces also. In this syndrome (which the authors call "the intermediary syndrome"), i.e. where the abdomen, arms, and thighs also are affected, certain internal organs and the joints are more involved than in "acrosclerosis stricto sensu". With rare exceptions, a symptomatic tetrade (REST-syndrome) occurred in acrosclerosis and all the intermediary syndromes. This consisted of Raynaud's syndrome (R), esophagopathy (E), cutaneous sclerosis (S), and telangectasia (T). Fifty % of the patients in addition had calcinosis (C), either subcutaneous or para-articular. The tetrade "REST syndrome" becomes in these cases the pentade "CREST syndrome". The addition of calcinosis to the other four phenomena of the REST syndrome does not alter the frequency of internal organ involvement or the prognosis of the disease. The term "REST syndrome" and its variant "CREST syndrome" should replace the conservative term "acrosclerosis" because they add to the purely cutaneous phenomena other characteristic manifestations of the disease. Two patients could neither be classified under the REST syndrome nor the progressive diffuse syndromes. Two other patients had no cutaneous phenomena ("scleroderma sine scleroderma").