Joint contractures and waxy skin in diabetes.
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Biomedical subjects
Publications and source records attributed to R M Fine.
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Four patients with metastases to the brain were treated by high-dose fractionated radiation therapy. In all four cases, a complete response and prolonged disease-free survival could be documented. Unlike the standard therapy for such patients (i.e., craniotomy and postoperative irradiation), high-dose fractionated radiation therapy carries no operative risk and can encompass multiple brain metastases and metastases in deep or critical intracranial sites.
Desquamative gingivitis, a sub-epithelial vesiculo-bullous disease involving the gingiva, has been shown to have positive direct immunofluorescent findings similar to those of cicatricial pemphigoid. However, the lack of severe scarring, limitation of lesions to the anterior portion of the mouth and the tendency toward limitation of lesions to the oral mucosa over long periods of time in desquamative gingivitis, suggest that desquamative gingivitis and cicatricial pemphigoid are distinct and separate entities.
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A patient with tricuspid regurgitation by clinical evaluation, cardiac catheterization, and angiography was found at autopsy to have a cardiomyopathy involving both ventricles but with predominant involvement of the right ventricle. He also had primary testicular failure and a distinctive type of collagenoma. The patient's two brothers were examined and found to have similar collagenomas and testicular failure. Evidence for a mild to moderate degree of cardiomyopathy was also apparent by findings on physical examination, chest roentgenogram, electrocardiogram, and echocardiogram. The father was known to have had a similar skin lesion and congestive heart failure. The aspects of the clinical presentation of this patient and the findings in the two brothers are discussed. A common genetic link in this familial syndrome is not elucidated.
EDCl is a novel glycoprotein, mol wt 27,000, isolated in 1976 from leukemic urine. It inhibits the serine proteases trypsin and chymotrypsin and is antigenically related to interalpha trypsin inhibitor (IATI), mol wt 170,000, a normal plasma antiprotease. Since psoriasis is a non neoplastic hyperproliferative state, we have now measured EDCl by a specific radioimmunoassay (RIA) in plasma and urine of 24 untreated psoriatic patients. EDCl was not detectable in normal urine (less than 1 mg/gm creatinine) or plasma (less than 1 mg/L). 55% of psoriatic urine specimens were positive by RIA, containing 8 to 110 mg/gm creatinine. 75% of plasmas were positive, containing 12 to 32 mg/L. Plasma and urine contents of EDCl were significantly (P less than .05) correlated with severity of clinical disease (% skin involved) but not with age, sex, distribution or type of lesion, family history or arthritis.
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