Thiemann Disease

Abstract

Thiemann disease is a rare genetic disorder that is considered to be a form of avascular necrosis of the proximal interphalangeal joints of the fingers and toes. The clinical symptoms usually appear in adolescence or puberty and may be confused with juvenile rheumatoid arthritis. The characteristic symmetrical, firm, relatively painless deformity and x-ray findings of the epiphysical irregularities should suggest the diagnosis. As rheumatologists become more familiar with the disease, it may be more frequently and promptly diagnosed.

In Brief

This familial fragmentation of digital epiphyses was initially mistaken for juvenile RA in the patients reported.