Anther's study on 32 cases of progressive systemic sclerosis and reviewing 437 cases of this disease reported in literature in Japan during past 15 years presented the following results.
1) Clinical manifestations: The incidence of induration, Raynaud's phenomenon and hyperpigmentation of the skin were high. In particular, Raynaud's phenomenon was the most important sign in the initial stage. Arthralgia, exhaustion, emaciation and pyrexia were also commom, related with the growth of the width of induration.
2) Laboratory studies: Hypergammaglobulinemia and increase of sedimentation rate were noted. The date of the tests mentioned above were correlated with the width of induration. Both tests may reflect the prognosis of patients who suffer from progressive systemic sclerosis.
3) Histopathologic findings: Atrophy of the epidermis and flattening of the rete ridges were prominent through the stage of induration and atrophy. Hyperpigmentation of the basal layer was also seen associated with the progress of lesions. In the edematous stage, inflammatory infiltration, teleangiectasia and edema in the dermis were observed. In addition, hypertrophy of collagen fibers, thickening of the walls of the vessels and atrophy of sweat glands were conspicuous in the all stage.
