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IN 1900, Buschke1 described the case of a forty-six-year-old carriage painter who suffered from persistent brawny induration of the skin of the face, chest and upper extremities. Following influenz...

A special form of scleredema, confined to adults with severe and complicated diabetes mellitus, has come to be recognized. It lasts for an indefinite period of time, without tendency to spontaneous remission. The name diabetic scleredema is proposed for this form. Two such patients are presented and the literature on the subject is reviewed. We believe that diabetic scleredema should be added to the list of conditions complicating or in any way associated with diabetes mellitus.

A case of the uncommon association of scleredema and diabetes mellitus is presented. Such patients usually are obese, and the scleredema quite extensive and longlasting. The difference between this entity and scleredema adultorum is discussed. Attempts at treatment during a two-week period were unsuccessful.

Scleredema is a rare connective tissue disease "characterized by firm nonpitting edema, usually affecting the face, neck, scalp, conjunctivae, and thorax, occasionally the arms and more rarely the legs, sparing the hands and feet."1 The etiology is unknown but often follows a streptococcal infection. Systemic manifestations of the disease have been described,2 and cardiac involvement has been present in some of these patients (Table 1).3-6 The purpose of this report is to describe a child with scleredema and carditis and to review this association. CASE REPORT A 14-year-old retarded white boy was in good health until six weeks prior to admission when he developed pharyngitis and an upper respiratory tract infection.

The clinical course, laboratory findings and histopathology of scleredema adultorum in a four-year-old boy were typical. Temporary elevation of urinary chondroitin sulfuric acid was observed. The urinary findings and histopathology suggest the possibility that scleredema may be an "acquired" and transitory mucopolysaccharidosis.

Two patients with scleroderma manifested immune hematologic disorders. The first patient had severe thrombocytopenia which responded strikingly to corticosteroid therapy. Nine years later severe hemolytic anemia developed which subsided after corticosteroid therapy. The second patient presented with spontaneous bleeding associated with a platelet count of 15.000/ cu mm. Platelet agglutinins were present. Corticosteroid and immunosuppressive therapy reduced the titer of platelet agglutinins, with cessation of spontaneous mucosal bleeding and reduction of petechiae formation. The presence of associated diseases in these patients emphasizes the need to look for concomitant autoimmune hematologic disorders in patients with scleroderma.

This is a report of eight cases of scleredema associated with diabetes mellitus. There were six men and two women, with ages ranging from 41 to 80 years. The predominant clinical features were obesity, long-standing diabetes mellitus, and scleredema; there was a high incidence of heart disease. Five patients had diabetic retinopathy. Six were maturity-onset, overt diabetics while two were chemical or latent diabetics. Most patients were resistant to antidiabetic therapy. On skin biopsies the dermis was about three times thicker than normal. Histologically there was a marked, benign hyperplasia of collagen fibers involving the dermis and subcutaneous tissue. Chemical analysis of the skin revealed an increase in collagen and glycosaminoglycans, proportional to the increase in skin thickness. Analysis of the glycosaminoglycans revealed both hyaluronic acid and dermatan sulfate.

Skin from 6 patients with scleroderma, 3 with scleredema and 5 normal controls were subjected to qualitative and quantitative analysis of glycosaminoglycans. Glycosaminoglycans were identified by cetylpyridinium chloride-salt fractionation and by cellulose acetate electrophoresis. Our data suggest that in scleredema there may be an increase in hyaluronic acid while the concentration of dermatan sulfate is normal. All scleroderma specimens revealed a marked increase in dermatan sulfate.