Scleredema adultorum: Quail-quantitative analysis of collagen fibers

Dermatology Unit, S. Andrea Hospital, II School of Medicine, University of Rome "Sapienza", Italy.
Journal of endocrinological investigation (Impact Factor: 1.45). 09/2011; 34(8):650-1. DOI: 10.1007/BF03345400
Source: PubMed
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    ABSTRACT: Scleredema adultorum of Buschke is a rare disorder of unknown aetiology. It is characterized by diffuse, non-pitting swelling and induration of the skin. Skin biopsies reveal marked thickening of the dermis due to collagenous replacement of the subcutis and deposition of hyaluronic acid between the collagen fibers. The disease classically only affects the skin. In 24 cases an associated monoclonal gammopathy has been detected. A 75-year-old patient had a 19-year history of scleredema adultorum. In addition to a monoclonal gammopathy the patient suffered from involvement of the tongue, pharynx and upper esophagus. Furthermore a polyneuropathy, ocular involvement with restricted eye movements and a sicca syndrome were present. The simultaneous occurrence of cutaneous scleredema with any one of the above mentioned symptoms has been reported before. The wide variety of extracutaneous manifestations of scleredema as found in our patient is amazing and has not been previously described.
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    ABSTRACT: A review of thirty-three cases of scleredema, with particular reference to clinical and histologic findings, revealed that the disease was often of insidious onset and the course was usually prolonged. A preceding respiratory tract infection was uncommon and usually did not indicate a short course. The patients were divided into two groups according to the presence or absence of diabetes. Diabetes, when present, was typically the late-onset, insulin-dependent type and difficult to control. In many of these patients, the onset of scleredema was so subtle that it went unnoticed by the patient until pointed out by an examining physician.
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    ABSTRACT: We describe a patient with insulin-dependent diabetes without vascular complications in whom scleredema was confined to the thighs. Electron microscopy demonstrated heterogeneity in both the size and density of the collagen fibrils and the presence of filamentous material between them.
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