The disaccharides constituting chondroitinase-digestible glycosaminoglycan (GAG) in the skin lesions of patients with systemic sclerosis were determined using high-performance liquid chromatography (HPLC). In scleroderma there was an increase in the amount of delta Di-4S(DS), the main disaccharide unit of dermatan sulphate, and a decrease in delta Di-HA, the disaccharide unit of hyaluronic acid, as compared with normal skin from a similar site. The distribution pattern of the main disaccharides constituting chondroitin sulphate and dermatan sulphate in scleroderma differed from that in scars or scleredema.

POEMS syndrome is a multi-system disorder characterized by polyneuropathy, organomegaly, endocrinopathy, the presence of monoclonal protein and skin changes. We describe a case with scleroderma and angiomatous vascular proliferation as skin alterations which may be involved in this syndrome.

A new occupational disorder characterized by skin sclerosis is described. This disease developed acutely in workmen exposed to the vapor of epoxy resins. Based on animal experiments, an amine [bis(4-amino-3-methyl-cyclohexyl) methane] is suspected of being the causative agent of the skin sclerosis.

A glycosaminoglycan with scleroderma-inducing effect was isolated and partially purified from the urine of patients with systemic scleroderma. The glycosaminoglycan was an N-sulfated glycosaminoglycuronan and its high total sulfate and 2,5-anhydromannose contents suggest that the glycosaminoglycan is a degradation product of heparin or polysulfated heparan sulfate. Furthermore, the composition of the above glycosaminoglycan was similar to that of the N-sulfated glycosaminoglycan which we observed previously in uninvolved skin of scleroderma.

Adult patients having systemic scleroderma with generalized morphea-like skin lesions, whom we had examined during the past 7 years, were reexamined because their clinical features were somewhat similar to those of occupational scleroderma described hitherto. Out of 9 patients, 7 patients had been engaged in occupations exposing them to organic solvents for a long time before or after Raynaud’s phenomenon appeared. A skin sclerosis was also induced experimentally with some aliphatic hydrocarbons of organic solvents.