CONGENITAL LIVEDO-RETICULARIS AND RECURRENT STROKE-LIKE EPISODES

Three children with pronounced livedo reticularis present since birth (cutis marmorata-telangiectasia congenita) have been followed to the a ges of eight, 17 and 21 years. During childhood they developed frequen t recurrent transient stroke-like hemipareses, affecting either side o f the body, associated with ipsilateral pain, headache, visual symptom s, dysphasia, fits and confusion. Intellectual failure and, in one, pr ogressive spasticity have followed. Attacks were more frequent in wint er. Other problems have included abnormal peripheral vascular response s to temperature change, gastro-intestinal bleeding, glaucoma, local t issue hypertrophy and, in the two older patients, renal involvement wi th hypertension. Their condition represents a form of congenital vascu lopathy. Anticonvulsants, anti-migraine agents, anti-platelet drugs an d flunarizine have been ineffective. Nifedipine prevented further atta cks in one patient and reduced attacks in another, but has not helped the third child. Adequate clothing and warmth may also be important.