AB0930 The Pachydermoperiostosis: Clinical and Radiologic Characteristics (About 6 Cases)

Abstract

BackgroundThe pachydermoperiostosis is a rare genetic disorder. This is the primitive and idiopathic form of the hypertrophic osteoarthropathy (HOA). The disease is characterised clinically by digital clubbing and hypertrophic skin changes, seborrhoea with hyperhidrosis, and arthralgia or arthritis. This disease can be a differential diagnosis with secondary HOA and chronic inflammatory rheumatism.ObjectivesThe objective of our study was to describe the clinical and radiological aspects of pachydermoperiostosis.MethodsThis is a retrospective study of 6 cases of pachydermoperiostosis collected in the Department of Rheumatology over a period of 14 years [2000-2014].ResultsOur population consists of 6 men. The mean age was 27.5 years. Five patients complained of inflammatory arthralgias and one patient of polyarthritis. Skin thickening (pachyderma) was observed in all cases. The skin of the hands and feet showed excessive sweating in 4 cases. Finger clubbing was noted in 5 cases. Excessive sebaceous secretions was observed in 2 cases. Inflammation tests were disturbed in 4 cases. Hypergammaglobulinemia was present in one case. The skeletal X-ray documented marked periosteal thickening of the long bones and sacro-iliac osteosclerosis (periostosis) in all cases. Bone scintigraphy showed intense hyperfixation of the long bones and sacro-iliac joints. After excluding secondary causes, primary HO diagnosis was established. The treatment consisted of analgesics in all cases, NSAIDs in 2 cases and tamoxifen in 2 cases.ConclusionsPachydermoperiostosis is a rare hereditary disorder, which affects both bones and skin. It is characterized by a combination of dermatologic changes (pachydermia or thickening of the skin) and rheumatologic manifestations (periostosis and finger clubbing).Disclosure of InterestNone declared