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 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.

Which cancer is associated with hypertrophic osteoarthropathy?

Hypertrophic pulmonary osteoarthropathy (HPO) is a rare paraneoplastic syndrome that is frequently associated with lung cancer; however, the incidence of clinically apparent HPO is not well known.

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 does clubbing mean in medical terms?

a. Clubbing is defined as a bulbous enlargement of the connective tissue in the distal phalanges of the fingers and toes.

How is hypertrophic osteoarthropathy treated?

Primary hypertrophic osteoarthropathy Medical care is palliative and includes nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, tamoxifen, retinoids, and risedronate to alleviate the painful polyarthritis/osteoarthropathy. Colchicine may be helpful for the pain due to subperiosteal new bone formation.

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.

What is the most common cause of secondary hypertrophic osteoarthropathy?

Although secondary HOA is most commonly associated with intrathoracic disease, it is important to be aware of its link to nonpulmonary diseases, and avoid the pitfall of labeling all cases of secondary HOA as hypertrophic pulmonary osteoarthropathy. The most common cause of HOA is non–small cell lung carcinoma (NSCLC).

Is clubbing reversible?

Clubbing is usually acquired and is associated with certain cardiopulmonary and gastrointestinal disorders, but may occur in congenital or familial forms. Acropachy is an alternative term for clubbing. Acquired clubbing is often reversible when the associated condition is treated successfully.

What are the stages of clubbing?

Stages

• No visible clubbing – Fluctuation (increased ballotability) and softening of the nail bed only.
• Mild clubbing – Loss of the normal <165° angle (Lovibond angle) between the nailbed and the fold (cuticula).
• Moderate clubbing – Increased convexity of the nail fold.