ATAXIA-TELANGIECTASIA AND NECROBIOSIS LIPOIDICA - AN EXPLAINABLE ASSOCIATION

In the last decade, several cases of ataxia-telangiectasia with necrob iosis lipoidica have been described in the literature but the reason f or this association remained obscure. Recently great strides have been made in cytogenetics. The development of Louis-Bar Disease has been a ttributed to a defective regulation of the Immunoglobulin Gene Superfa mily. According to this, we are now able to explain the pathogenesis o f the main cutaneous manifestations associated with ataxial telangiect asia. Necrobiosis lipoidica particularly, does not seem to occur by ch ance as formerly suggested. It would result from both the immune chara cteristics and the abnormal fibroblasts of these patients. Future stud ies should specify the exact chronology and the relative importance of each etiopathogenic mechanism