Pachydermoperiostosis

Orpha code: 2796OMIM code: 614441

Definicja

Pachydermoperiostosis (PDP) is a form of primary hypertrophic osteoarthropathy (see this term), a rare hereditary disorder, and is characterized by digital clubbing, pachydermia and subperiosteal new bone formation associated with pain, polyarthritis, <i>cutis verticis gyrata</i>, seborrhea and hyperhidrosis. Three forms have been described: a complete form with pachydermia and periostitis, an incomplete form with evidence of bone abnormalities but lacking pachydermia, and a <i>forme frusta</i> with prominent pachydermia and minimal-to-absent skeletal changes.

Disease data
Klasyfikacja

Malformation syndrome

Synonimy
PDP
PDP
Zespół Touraine'a, Solente i Gole'a
Touraine-Solente-Gole syndrome
Kod ORPHA
2796
Kod OMIM
614441
Kod ICD10
M89.4
Kod ICD11
FB86.10

No additional description.

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