Innovative performance evaluation methodologies and tools: Selected papers from ValueTools 2006
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Exegesis. – We report a 36 years old black man presenting over many years polyarthralgias, broadning of fingers and clubbing of the fingers and toes extremities.

This was mentionned on other family members. The physical examination was otherwise unremarkable. There were no skin thickening, no psoriasis-like and cardio-pulmonary disease features.

These following exams were normal; Hemogram, fibrinogen, C reactive protein, rheumatoïd factor, serum calcium and phosphorus, thyroid hormones, growth hormone, chest X-ray, gastroduodenoscopy, electrocardiogram. The skeletal X-ray documented a widespread bone formation, a sacro-iliac osteosclerosis and interosseous ossifications beetwen tibias and fibulas.

Conclusion. – Pachydermoperiostosis diagnosis was set up on 3 out of the 4 Borochowitz criteria. The absence of pachyderma defines this incomplete form. The osteoarticular manifestations lead mainly to differential diagnosis with the secondary hypertrophic osteoarthropathy and chronic inflammatory rheumatisms.

The underlying pathogenic mechanism of this disease remains still unclear.


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Pachydermoperiostose. A propos d'un cas
La Revue de Medecine Interne

Pachydermopériostose. À propos d'un cas
La Revue de Médecine InterneVolume 27, Issue 9September 2006, Pages 710-712
F. Kabi, O. Mkinsi, S. Janani, N. Raissouni

Abstract

Résumé

Introduction

La pachydermopériostose (PDP) ou ostéoarthropathie hypertrophiante (OAH) primitive est une maladie héréditaire rare.

Fait clinique

Nous rapportons une observation de PDP chez un jeune homme de 22 ans, issu d'un mariage consanguin. Le tableau associait une arthropathie, un hippocratisme digital, une périostose diffuse et une pachydermie des extrémités et du front. Tout le bilan réalisé à la recherche d'une étiologie est resté négatif. Ainsi le diagnostic de PDP était retenu et le malade était traité par colchicine.

Discussion

Les problèmes diagnostiques que peut poser la PDP, surtout avec les OAH secondaires et les rhumatismes inflammatoires chroniques sont discutés.

Introduction

The pachydermoperiostosis (PDP) or primitive hypertrophic osteoarthropathy (HOA) is a rare hereditary disease.

Case record

We report a 22-year-old man born to consanguineous marriage who presented presented with PDP. This patient disclosed an arthropathy, a clubbing, a diffuse periostosis, and a pachyderma of the hands, the feet and the forehead. All the examinations that were performed to look for an etiology remained negative. Diagnosis of PDP was considered and the patient treated with colchicine.

Discussion

We discuss the diagnostic issues raised by PDP, especially with the secondary HOA and chronic inflammatory rheumatisms.


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doi:10.1016/j.revmed.2007.10.418
Copyright © 2007 Elsevier Masson SAS All rights reserved.

Cas clinique

Ostéoarthropathie hypertrophiante primitive chez un adolescent

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