Pachydermoperiostosis

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.

Coarse facial features

Absence of fine and sharp appearance of brows, nose, lips, mouth, and chin, usually because of rounded and heavy features or thickened skin with or without thickening of subcutaneous and bony tissues.


Total: 2

                      


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PMID (PMCID)
15168249
MALE Adult
Pachydermoperiostosis associated with juvenile polyps of the stomach and gastric adenocarcinoma.
Ikeda F, Okada H, Mizuno M, Kawamoto H, Okano N, Okazaki H, Hamazaki S, Shiratori Y.
J Gastroenterol. 2004;39(4):370-4.
Pachydermoperiostosis (PDP) is a rare syndrome, and the presence of digital clubbing, radiographic periostosis, and coarse facial features are the main diagnostic criteria.
6825994
MIXED_SAMPLE Adult
Pachydermoperiostosis, hypertrophic gastropathy, and peptic ulcer.
Lam SK, Hui WK, Ho J, Wong KP, Rotter JI, Samloff IM.
Gastroenterology. 1983;84(4):834-9.
Two brothers with pachydermoperiostosis, an autosomal dominant syndrome characterized by digital clubbing, periosteal new bone formation, coarse facial features with thick, furrowed, and oily skin, presented in their twenties with severe complicated duodenal ulcer disease requiring multiple operations.