Pachydermoperiostosis
Rare genetic disorder that affects bones and skin / From Wikipedia, the free encyclopedia
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Pachydermoperiostosis (PDP) is a rare genetic disorder that affects both bones and skin.[1] Other names are primary hypertrophic osteoarthropathy or Touraine-Solente-Golé syndrome.[2] It is mainly characterized by pachyderma (thickening of the skin), periostosis (excessive bone formation) and finger clubbing (swelling of tissue with loss of normal angle between nail and nail bed).[1][3]
Pachydermoperiostosis | |
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Other names | Primary hypertrophic osteoarthropathy |
Specialty | Rheumatology |
This disease affects more men than women.[1][4] After onset, the disease stabilizes after about 5–20 years. Life of PDP patients can be severely impaired.[1][5] Currently, symptomatic treatments are NSAIDs and steroids or surgical procedures.[5][6]
In 1868, PDP was first described by Friedreich as 'excessive growth of bone of the entire skeleton'. Touraine, Solente and Golé described PDP as the primary form of bone disease hypertrophic osteoarthropathy in 1935 and distinguished its three known forms.[1]