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Touraine-Solente-Golé syndrome


Also known as:
Audry’s syndrome I
Brugsch's syndrome (same condition plus acromicria)
Friedreich-Erb-Arnold syndrome
Roy's syndrome
Roy-Jutras syndrome
Uehlinger's syndrome

Associated persons:
Julius Arnold
Charles Audry
Theodor Brugsch
Wilhelm Heinrich Erb
Nikolaus Friedreich
Laurent Golé
Albert Jutras
J. N. Roy
Gabriel Solente
Albert Touraine
Erwin Uehlinger

Description:
This osteodermopathic syndrome is characterized by cutis verticis gyrata (corrugated overgrowth of the scalp, or so-called "bull-dog scalp" lesions) also involving the forehead, face, and extremities; clubbing of the digits due to soft tissue hyperplasia; elephantiasis of long bones, enormously large hands and feet; periosteal lesions, fatigability, blepharitis, arthralgia, periosteal overgrowth, and occasionally pulmonary osteoarthropathy.

Occurs almost exclusively in males. More severe in females. Onset at the time of puberty, up to third decade. General health and mental status not affected. Inheritance is autosomal dominant with variability of expression. This condition differs from the Brugsch syndrome by the presence of acromegaly.

Bibliography:
  • N. Friedreich:
    Hyperostose des gesamten Skeletts.
    [Virchow’s] Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1868, 43: 83-87.

  • W. Erb:
    Über Akromegalie (krankhaften Riesenwuchs).
    Deutsches Archiv für klinische Medicin, Leipzig, 1887-1888, 42: 295-338.

  • J. Arnold:
    Acromgalie, Pachyacrie oder Ostitis? Ein anatomischer Bericht über den Fall Hagner.
    Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1891, 10: 1.

  • C. Audry:
    Pachydermia occipitale vorticalée (cutis verticis gyrata).
    Annales de dermatologie et syphiligraphie, Paris, 1909, 10: 257-258.

  • A. Touraine, G. Solente, L. Golé:
    Un syndrome ostéodermopathique. La pachyderme plicaturée avec pachypériostose des extrémités.
    La presse médicale, Paris, 1935, 42: 1820-1824.

  • J. N. Roy:
    Hypertrophie des tarses palpébraux, des téguments de la face et des extrémités des membres, associée à une osteo-periostose presque généralisée; un syndrome nouveau.
    Union médicale du Canada, Montreal, 1936, 65: 517-539.

  • E. Uehlinger:
    Hyperostosis generalisata mit Pachydermie. (Idiopathische familiäre generalisierte Osteophytose Friedreich-Erb-Arnold).
    [Virchow’s] Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1942: 308-396.

  • T. Brugsch:
    Akromikrie oder Dystrophia osteogenitalis.
    Medizinische Klinik, Berlin, 1927, 23: 81-82.

  • H. G. Brugsch:
    Acropachyderma with pachyperiostitis. Report of case.
    Archives of Internal Medicine, Chicago, 1941, 68: 687-700.



 
 

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