La maladie de Thiemann. À propos d’un cas

Abstract

Thiemann disease is a rare form of a vascular necrosis which involved the epiphyses of phalanges in PIP and DIP joints leading to deformity of fingers and toes. The clinical symptoms usually appear in adolescence. Thiemann disease is usually familial with an autosomal dominant transmission. Sporadic cases also reported and tend to occur in males. T.L., a 15-year old, presented with a six months history of pain and swelling of the interphalangeal joint of the thumb and the proximal interphalangeal joint of both little finger. He has no history of specific injury, no family history of joint disorder. He has no episode of arthritis. No particular hyper laxity. No metabolic or endocrine disorders. Blood tests was strictly normal. Markers of acute inflammation including C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were within normal limits. Serology for rheumatoid factor, antinuclear antibodies and HLA antigen B27 were also negative. Radiography demonstrated irregularity of the epiphyses of the interphalangeal joint of the thumb. The bones appear broadened, sclerotic. MRI demonstrated osteonecrosis in the head of the proximal phalanx of the thumb and the little finger. This disorder can be distinguished from osteoarthritis, juvenile rheumatoid arthritis, and gout by its early age of onset, equal sex incidence, characteristic joint distribution, and lack of abnormal laboratory findings. The etiology is unknown. Most authors classified Thiemann's disease as a juvenile osteochondritis comparing it with diseases like Calve Perthes, Kienbock, Scheuermann. The role of trauma is undertermined. Although Thiemann's disease was iniatlly described in 1909, few reports of the disease have been published, probably of its relative rarity or of failure of diagnosis. Trauma may worsen the prognosis by deformation of susceptible epiphysis. Treatment is usually symptomatic.