Digital clubbing and hypertrophic osteoarthropathy


Key Points

  • Hypertrophic osteoarthropathy is a syndrome characterized by abnormal proliferation of the skin and osseous tissues at the distal parts of the extremities.

  • Three features are typically present: a peculiar bulbous deformity of the tips of the digits conventionally described as clubbing , periostosis of the tubular bones, and synovial effusions.

History

Digital clubbing is one of the oldest clinical signs in medicine. Its original recognition has been attributed to Hippocrates (circa 450 bc ). Marie, in 1890, and Bamberger, in 1891, described the fully developed syndrome. Marie distinguished it from acromegaly and suggested the term pulmonary hypertrophic osteoarthropathy . Because the site of primary disease may be in areas other than the lungs, this designation fell into disuse. The term used today is hypertrophic osteoarthropathy (HOA). Paleopathologic studies have demonstrated changes consistent with HOA in human skeletal remains from different ancient civilizations.

Epidemiology

There are no systematic studies of the prevalence of digital clubbing in either the general population or hospital inpatients. The deformity is associated with a variety of internal illnesses, so most clinicians, regardless of their specialty, have frequent encounters with patients who display this abnormality.

The veterinary literature contains reports of this illness in different species of mammals, in which the syndrome appears in response to the same illnesses as those reported for humans. HOA has been artificially produced in dogs by surgically caused right-to-left shunts of blood or by chemically induced lung cancer. Nevertheless, a stable animal model of the syndrome, accessible for systematic studies, has not been developed.

Classification

The classification of HOA is outlined in Fig. 214.1 . There is now evidence to support the contention that clubbing and HOA represent different stages of the same disease process. In the overwhelming majority of cases, the finger deformity is the first manifestation, and as the syndrome progresses, periostosis becomes evident. The degree of association of clubbing with the diverse illnesses varies, with clubbing ranging from being a constant finding, as in cases of cyanotic heart diseases, to being a rare manifestation, as in patients with cancer of the lung, liver cirrhosis, or Graves disease.

Fig. 214.1, Various forms of hypertrophic osteoarthropathy (HOA). AV , Arteriovenous; POEMS , polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes.

Synonyms for the deformity besides clubbing include drumstick, pendulum , and Hippocratic fingers . Primary HOA is also known as pachydermoperiostosis . The term acropachy is etymologically the most appropriate and has been used to describe either clubbing or the fully developed syndrome.

Clinical Features

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