| • | AKA malignant atrophic papulosis |
| • | rare; multi-system thrombosis/ lymphocytic vasculitis – skin, GI tract, ocular, CNS |
| • | often fatal: intestinal perforations or CNS infarcts |
pathogenesis:
| • | unknown; a decrease in blood fibrinolytic activity seems to be present in most of the studied patients |
| • | some cases found to be associated with anti-phospholipid antibodies |
clinical:
| • | evolution of the lesion is characteristic: a papule evolving into a porcelain white atrophic lesion |
| • | usually multiple (up to 600 lesions in same patient) |
treatment:
| • | ASA or dipyridamole – in patients in whom increased platelet aggregation has been demonstrated |
| • | ethylestrenol – was used as an anabolic steroid (like danazol, it has fibrinolytic activity) |
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