Pachydermoperiostosis

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 29069
ICD10 = ICD10|L|62|0|l|60, ICD10|M|89|4|m|86
ICD9 = ICD9|731.2, ICD9|757.39
ICDO =
OMIM = 167100
MedlinePlus =
eMedicineSubj = derm
eMedicineTopic = 815
MeshID = D010004

Pachydermoperiostosis (PDP) is a rare disorder characterized by clubbing of the fingers, thickening of the skin of the face (pachyderma) and periostosis. cite journal |author=Rendina D, De Filippo G, Viceconti R, "et al" |title=Interleukin (IL)-6 and receptor activator of nuclear factor (NF)-kappaB ligand (RANKL) are increased in the serum of a patient with primary pachydermoperiostosis |journal=Scand. J. Rheumatol. |volume=37 |issue=3 |pages=225–9 |year=2008 |pmid=18465459 |doi=10.1080/03009740701772457 |url=]

History

It was first described in 1868. [Friedreich, N. Hyperostose des gesammten Skelettes. Arch. Path. Anat. 43: 83-87, 1868.] However it wasn't until 1935, when Touraine-Solente-Golé recognised it as a syndrome. [Touraine, A.; Solente, G.; Gole, L.. Un syndrome osteodermopathique: la pachydermie plicaturee avec pachyperiostose des extremites. Presse Med. 43: 1820-1824, 1935.]

Forms

In fact, there are 3 forms:
* 1. a complete form (pachydermia and periostosis),
* 2. an incomplete form (without pachydermia), and
* 3. a forme fruste (pachydermia with minimal-to-absent skeletal changes).

References