What does pachydermoperiostosis mean?

What does pachydermoperiostosis mean?

Pachydermoperiostosis is a rare disorder characterized by clubbing of the fingers and toes; thickening of the skin of the face (pachyderma); excessive sweating (hyperhidrosis); and new bone formation associated with joint pain.

What causes pachydermoperiostosis?

Deficiency of the prostaglandin transporter (SLCO2A1) has been characterized as the main cause of primary hypertrophic osteoarthropathy. Mutations in the prostaglandin transporter gene, SLCO2A1, have been documented with pachydermoperiostosis.

What is primary hypertrophic osteoarthropathy?

The primary hypertrophic osteoarthropathy (pachydermoperiostosis) is a hereditary disease characterized by skin thickening (pachydermia), finger clubbing, and proliferation of periosteum (periostitis) with subperiosteal new bone formation.

Does clubbing hurt?

Clubbing may have a slow onset to the point where the individual is unaware of the change. It is usually painless but can rarely be accompanied by discomfort in the fingertips. Rare skin conditions including pachydermoperiostosis and palmoplantar keratoderma are unusual causes of nail clubbing.

What are the stages of finger clubbing?

Clubbing is present in one of five stages: No visible clubbing – Fluctuation (increased ballotability) and softening of the nail bed only. No visible changes of nails. Mild clubbing – Loss of the normal <165° angle (Lovibond angle) between the nailbed and the fold (cuticula).

What causes hypertrophic osteoarthropathy?

Hypertrophic osteoarthropathy (HOA) is mainly caused by mainly fibrovascular proliferation. It is characterized by a combination of clinical findings, including severe disabling arthralgia and arthritis, digital clubbing, and periostosis of tubular bones with or without synovial effusion.

What is hypertrophy of bone?

Increase in bone density. Bone hypertrophy occurs in response to physical activity. The bones in the throwing arm of a baseball pitcher and the racket arm of a tennis player are denser and thicker than the other arm.

Does COPD cause clubbing?

Chronic obstructive pulmonary disease (COPD) per se does not cause clubbing, but if clubbing is present in COPD, underlying lung cancer and bronchiectasis must be ruled out.

Is clubbing of fingers painful?

Clubbing tends to affect the thumb and forefinger first before progressing to the other fingers. Symptoms of clubbed fingers typically impact both hands. The condition is painless.

How is hypertrophic osteoarthropathy diagnosed?

The diagnostic criteria for hypertrophic osteoarthropathy (HOA) include clubbing and periostosis of the tubular bones. Three incomplete forms of hypertrophic osteoarthropathy are described: Clubbing alone. Periostosis without clubbing in the setting of an illness known to be associated with HOA.

Is hypertrophic osteoarthropathy Painful?

Patients with hypertrophic osteoarthropathy due to neoplastic or inflammatory pulmonary conditions typically report pain and swelling in joints and long bones. Joint symptoms can range from mild to severe arthralgias, and it can involve the small and large joints [1].

What are the two types of hypertrophy?

There are two types of muscular hypertrophy:

  • myofibrillar: growth of muscle contraction parts.
  • sarcoplasmic: increased muscle glycogen storage.