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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 61 (1983), S. 1199-1207 
    ISSN: 1432-1440
    Keywords: Hemochromatosis arthropathy ; Nosology ; Tissue typing ; Assessment of relatives
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In 22 of 35 patients (63%) with idiopathic hemochromatosis arthropathy could be demonstrated. In 20 patients the metacarpophalangeal joints (mainly II and III) with preference of the right hand were affected. Chondrocalcinosis of the wrist and knee was found both in two patients with metacarpophalangeal joint disease and in two patients without metacarpophalangeal disease. Further joints affected were the wrists (14), the other finger joints (11), and the knees (6). The dominant clinical complaint was pain in motion. Swelling and redness were rare findings only in case of acute exacerbations. The radiologic changes in the metacarpophalangeal joints were narrowing of the joint spaces, subchondral cysts, sclerosis of subchondral bone of metacarpal heads, and marginal osteophytic appositions at the joints. In one third of the patients arthropathy was evident before the diagnosis of idiopathic hemochromatosis was made. Histocompatibility testing confirmed that HLA-A3 is significantly more frequent in patients with idiopathic hemochromatosis than in normal persons. A statistically significant difference concerning HLA-phenotypes between patients with arthropathy and patients without arthropathy could not be detected. There was no case of arthropathy when 98 relatives of the patients were examined. However, idiopathic hemochromatosis was first detected in ten persons of this group.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 60 (1982), S. 1319-1328 
    ISSN: 1432-1440
    Keywords: Eosinophilic Fasciitis ; Shulman Syndrome ; Eosinophile Fasziitis ; Shulman-Syndrom
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Sklerodermieähnliche Hautindurationen ohne Organmanifestationen, Gelenkskontrakturen, Bluteosinophilie und ein entzündliches Infiltrat der Faszie zwischen Subkutis und Muskulatur gelten als die Charakteristika der eosinophilen Fasziitis (EF). Wir berichten über einen 30jährigen Diabetiker mit dem von Shulman 1974 definierten Syndrom. Eine Kortikosteroidtherapie führte zur Remission der Krankheitssymptome, jedoch zeigte eine Rebiopsie, daß die Therapie keinen Einfluß auf den pathomorphologischen Befund hatte. Anhand einer Literaturübersicht unter Berücksichtigung von 118 Fällen werden die Gemeinsamkeiten und Unterschiede von EF und der systemischen Sklerodermie diskutiert.
    Notes: Summary Scleroderma like skin indurations without internal organ involvement, joint contractures, eosinophilia, and inflammatory infiltration of the fascia between subcutis and muscle are considered as characteristic features of eosinophilic fasciitis (EF). We report a further case of a 30 years old diabetic confirming to the syndrome defined by Shulman 1974. Corticosteroid therapy resulted in remission of disease symptoms, however a rebiopsy revealed no effect of therapy on histopathologic changes. A critical review of 118 cases in the literature is presented to explore the relationship of EF to scleroderma.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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