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Engel-von Recklinghausen syndrome

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Now mostly historical term for a generalized rarefying bone disorder with skeletal deformation, seen in advanced hyperparathyroidism. It is characterized by cysts or brown tumours in the form of deeply pigmented foci consisting of fibrous scarring of the bone with pseudocysts, haemorrhages, and collection of osteoclasts. There is arthralgia, fractures, bowing of long bones and often gross deformities of the spine and chest.

Bibliography

  • G. Engel:
    Über einen Fall von cystoider Entartung des ganzen Skelettes.
    Giessen, F. C. Pietsch, 1864.
  • F. D. von Recklinghausen:
    Demonstration von Knochen mit Tumor bildender Ostitis deformans.
    Tageblatt der Naturforschenden Versammlung 1889. Heidelberg, 1890, p 321. Die fibröse und deformirende Ostitis, die Osteomalacie und die osteoplastische Carcinose in ihren gegenseitigen Beziehungen.
    Festschrift für Rudolf Virchow. Georg Reimer Verlag; Berlin, 1891. Untersuchungen über Rachitis und Osteomalacie.
    Jena, G. Fischer, 1910.

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