Degos disease is a rare disorder, characterized by a vasculopathy of unknown origin that leads to typical skin lesions and involves other organ systems. It is frequently a lethal condition; death occurs as a consequence of intestinal perforation. In about 20% of cases, the central nervous system is involved and the neurological symptoms can be prominent. The incidence of the disease in children is very uncommon. We report the case of teenage girl who had Degos disease with prominent neurological involvement. (J Child Neurol 1987;2:42-46).
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