Pachydermoperiostosis

Pachydermoperiostosis
Other namesPrimary hypertrophic osteoarthropathy
SpecialtyRheumatology Edit this on Wikidata

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]

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]

  1. ^ a b c d e Castori M, et al. (2005). "Pachydermoperiostosis: an update". Clin. Genet. 68 (6): 477–486. doi:10.1111/j.1399-0004.2005.00533.x. PMID 16283874. S2CID 21973812.
  2. ^ Yazici Y, Schur PH, Romain PL (2011). "Malignancy and rheumatic disorders". MA.
  3. ^ Martínez-Ferrer A, et al. (2009). "Prostaglandin E2 and bone turnover markers in the evaluation of primary hypertrophic osteoarthropathy (pachydermoperiostosis): a case report". Rheumatol. Clin. 28 (10): 1229–1233. doi:10.1007/s10067-009-1197-9. PMID 19455364. S2CID 24964091.
  4. ^ Gómez Rodríguez N, Ibáñez Ruán J, González Pérez M (2009). "Primary hypertrophic osteoarthropathy (pachydermoperiostosis). Report of 2 familial cases and literature review". Rheumatol Clin. 5 (6): 259–263. doi:10.1016/s2173-5743(09)70134-0.
  5. ^ a b Ghosn S, et al. (2010). "Treatment of pachydermoperiostosis pachydermia with botulinum toxin type A.". J. Am. Acad. Dermatol. 63 (6): 1036–1041. doi:10.1016/j.jaad.2009.08.067. PMID 20933299.
  6. ^ Leni George; et al. (2008). "Frontal rhytidectomy as surgical treatment for pachydermoperiostosis". J Dermatol Treat. 19 (1): 61–63. doi:10.1080/09546630701389955. PMID 18273728. S2CID 27962868.