ANNULAR ATROPHIC LICHEN-PLANUS AND SNEDDONS-SYNDROME

We report the case of a patient who had 2 rare diseases, annular atrop hic lichen planus (AALP) and Sneddon's syndrome (SNS). This patient ha d also digital nodules with histological abnormalities suggestive of S NS vasculopathy, which have not been reported so far. AALP is the most rare of all varieties of lichen planus since this case is the third r eported to date. The association of livedo racemosa and cerebrovascula r disease is the hallmark of SNS, the incidence of which is estimated to be 4 cases per year per million inhabitants. In both diseases, ail abnormal production of elastic-tissue-degrading enzymes or a constitut ional abnormality of the elastic tissue can be postulated, since SNS i s characterized by arteriolar changes with deterioration of the intern al elastic lamina and AALP by destruction of the dermal elastic tissue .