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The histological features of muscle sarcoïdosis

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Summary

The histological pattern of muscle sarcoïdosis may be an interstitial diffuse infiltration containing giant cells, scattered epitheloïd cells and numerous lymphocytes, with progressive replacement of muscle tissue by a pallisade like and reticular framework of collagen fibres ending in a dense fibrous connective tissue. This evolution was seen in case I. In other instances (case 2) in addition to the cellular infiltration, there is a widespread degeneration of muscle fibres which may lead to floccular necrosis with macrophagia, but may present more specific changes, namely, the formation of numerous muscle multinucleated giant cells, and the development of epitheloïd tubercles within degenerated muscle fibres.

This histological pattern when associated with a clinical picture of late life muscular dystrophy has been named granulomatous myopathy.

Zusammenfassung

Das histologische Substrat der Muskel-Sarkoidose kann in einer diffusen interstitiellen Infiltration von zahlreichen Lymphocyten, verstreuten Epitheloidzellen und Riesenzellen mit progredientem Ersatz von Muskelgewebe durch ein palisadenartiges und retikuläres Netzwerk kollagener Fasern mit schließlichem Übergang in ein dichtes fibröses Bindegewebe bestehen. Diese Entwicklung bestand in Fall 1. In anderen Fällen (Fall 2) besteht neben der zelligen Infiltration eine ausgedehnte Muskelfaserdegeneration, die zu flokkulärer Nekrose mit Makrophagenbildung führen oder spezifischere Veränderungen mit Bildung zahlreicher vielkerniger myogener Riesenzellen sowie Entwicklung epitheloider Tuberkel innerhalb der degenerierten Muskelfasern aufweisen kann. Dieses histologische Substrat wurde in der Verbinding mit dem klinischen Bild der Muskel-Spätdystrophie als “granulomatöse Myopathie” bezeichnet.

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Authors and Affiliations

  1. Department of Neurology, Medical Clinic. Hopital Brugmann, Brussels University, 4 Place Van Gehuchten, Brussels, Berlgien

    C. Coërs

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  1. C. Coërs
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This work was supported by grants from the Muscular Dystrophy Associations of America Inc, and the Fonds de la Recherche Scientifique médicale.

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Coërs, C. The histological features of muscle sarcoïdosis. Acta Neuropathol 7, 242–252 (1967). https://doi.org/10.1007/BF00686375

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  • DOI: https://doi.org/10.1007/BF00686375

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