General Considerations

• Occurs in a rare primary and much more common secondary form
• Primary form is known as pachydermoperiostitis (HOA)
• Secondary form (AKA Marie-Bamberg Syndrome) occurs with a wide variety of pulmonary malignancies and chronic suppurative diseases
  • Since it most commonly occurs with pulmonary disease it is known as hypertrophic pulmonary osteoarthropathy (HPOA)
• Secondary form (HPOA) produces symmetrical periostitis
• About 3-5% of patients with HOA have primary HOA
  • Remaining 95-97% have secondary HOA, or hypertrophic pulmonary osteoarthropathy (HPOA)
• HPOA occurs in about 5% of patients with bronchogenic carcinoma and 50% of patients with pleural mesothelioma
• HPOA may precede the discovery of the lung cancer

 

 

 

Clinical Findings

• Clubbing of the fingers and toes
• Enlargement of the extremities
• Painful, swollen joints
• Warmth and burning of the fingertips, sometimes with skin thickening
• Hyperhidrosis
• Joint involvement, especially in the wrists, knees, ankles, and small joints of the hands may mimic inflammatory arthritis

Imaging Findings

• Conventional radiography is the study of choice
  • Nuclear medicine studies reveal earlier changes with a higher sensitivity
• Usually produces diaphyseal and metaphyseal periostitis
• Periosteal proliferation can be
  • Single and solid (usual)
  • Laminated and regular or irregular
    • Laminated periostitis may have an onionskin appearance
• Initially, periostitis is symmetric and involves the tibia, fibula, radius, ulna and, less commonly, the femur, humerus, metacarpals, metatarsal, and phalanges on both sides
• Periosteal proliferation extends into metaphysis
• Periosteal proliferation rarely extends to the epiphysis
• Periosteal reaction is separated from the underlying cortex by a narrow radiolucent band at first, then fuses with cortex
• Periarticular soft tissue swelling and clubbing may be present and the patient may have clinical and radiographic features of the underlying primary lesion
• Nuclear Medicine
  • Radionuclide bone imaging is very sensitive for detecting HPOA
  • Diffuse symmetric increased uptake in the diaphysis and metaphysis of tubular bones along their cortical margin is seen
  • Creates a distinctive "parallel track"

 

 

Differential Diagnosis

• Acromegaly
• Thyroid acropachy
  • Complication of thyrotoxicosis and associated with exophthalmos
  • Almost always limited to hands and feet
• Venous stasis
  • Periosteal reaction only in lower extremities
  • Phleboliths and skin ulcers
• Diffuse idiopathic skeletal hyperostosis (DISH)

Treatment

• Removal of primary tumor frequently leads to remission of symptoms
• Vagotomy or occlusion of the ipsilateral pulmonary artery can lead to regression of symptoms as can chemotherapy

Prognosis

• For HPO, same as those without HPO
• For HOA, usually spontaneously arrests within ten years of its onset

 

 

Hypertrophic pulmonary osteoarthropathy. Frontal (left) and lateral (right) radiographs of the distal tibia and fibula show a thick, irregular periosteal reaction (yellow and white arrows) that extends from the diaphysis to the metaphysis but spares the epiphyses. The patient had a bronchogenic carcinoma.
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