Pierre-marie-bamberger syndrome M89.49

Author: Dr. med. S. Leah Schröder-Bergmann

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Last updated on: 29.10.2020

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Synonym(s)

Marie Bamberger Disease; Marie Bamberger Syndrome; Pierre-Marie-Bamberger disease; Secondary hypertrophic osteoarthropathy

History
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Initial description

The syndrome was first described by Bamberger in 1889. In 1890 Pierre Marie described the symptom picture of hypertrophic osteoarthropathy.

Definition
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The Pierre-Marie-Bamberger syndrome is a syndromal disease in which swelling occurs in the area of the diaphyses, preferably on long tubular bones and changes in the finger and toe end joints. The disease is a precancerous condition.

Occurrence
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The syndrome occurs in approximately 5% of patients withnon-small cell lung cancer.

In 70 - 90 % a malignant disease is found to be the cause of Pierre-Marie-Bamberger syndrome.

It also occurs in the following diseases:

  • severe lung diseases
  • congenital or acquired heart defects
  • Pulmonary metastases
  • Cirrhosis of the liver
  • Leukaemia
  • M. Hodgkin
  • inflammatory bowel disease
  • Occupational (pneumatic hammer)
  • for otherwise healthy persons

Etiology
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Chronic hypoxia leads to erythrocytosis with formation of drumstick fingers and toes and watch glass nails and also to swelling in the area of the diaphyses, preferably on the long tubular bones. There are also indications of a venous circulatory disorder.

Clinical picture
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The patients show the following symptoms:

  • Drum flail fingers or toes
  • Watch glass nails
  • Joint pain (can affect all joints, preferably medium-sized joints)
  • Swelling of the joints
  • Soft tissue thickening in the acra area
  • chronic proliferative subperiosteal osteitis
  • occasional redness or livid discoloration of the nail bed

Imaging
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The diagnosis is made on the basis of an X-ray of the affected parts of the skeleton. Here a periosteal proliferation in the area of the diaphyses of the tubular bones is shown.

Differential diagnosis
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Polyarthritis

Therapy
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Treatment of the underlying disease. The symptoms can almost completely disappear after the end of therapy.

Note(s)
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Drum flail fingers and watch glass nails precede a carcinoma by weeks, therefore the above-mentioned symptoms should always be considered as a malignoma. The same applies if glucocorticoids, non-steroidal anti-inflammatory drugs and immunosuppressive drugs are not responded to in cases of arthritis.

Literature
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  1. Arasteh K et al (2012) Internal Medicine 432
  2. Bosnjakovic-Büscher S (1983) X-ray diagnostics of skeletal diseases Part 5 Osteopathies 609-633
  3. Hatir I et al (2015) Journal of Rheumatology 74: 728-730
  4. Herold G et al (2018) Internal Medicine 151
  5. Schmalzing M (2018) Journal of Rheumatology 77: 309-321

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Last updated on: 29.10.2020