Skin lesions can be followed by systemic
manifestations, sometimes a few years later. Features can involve the eyes
(avascular patches), CNS (20% cerebrovascular or medullar accident), and
GI system (40-60%, particularly small bowel with abdominal pain,
bleeding, and diarrhea; intestinal perforations and peritonitis are the
leading causes of death). Pleural and pericardial effusion can be observed.