Thiemann's disease. A brief reminder.
Two brothers with Thiemann's disease are described and the accumulated world literature is briefly reviewed. Thiemann's disease is considered to be a form of idiopathic avascular necrosis of the PIP joints of the hands, with occasional involvement of other joints. The characteristic deformity and x-ray findings are reviewed. Thiemann's disease is a genetic disorder and, in families in which detail is available, is inherited as an autosomal dominant with virtual complete penetrance.
D001168:Arthritis / Q000000981:diagnostic imaging; D003937:Diagnosis, Differential; D005384:Finger Joint; D005787:Gene Frequency; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D010007:Osteochondritis / Q000000981:diagnostic imaging / Q000235:genetics*; D010641:Phenotype; D011859:Radiography; D013577:Syndrome