Pachydermoperiostosis

Orpha code: 2796OMIM code: 614441

Definition

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
Classification

Malformation syndrome

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

No additional description.

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