[Cutaneous elastopathy and vascular anomalies associated with neurological disorders. Apropos of 2 cases].
The two cases described exhibited elastopathy associated with arterial stenosis (case 1) or aneurysms (case 2). The first was clearly a case of elastic pseudo-xanthoma with characteristic cutaneous lesions associated with retinal angioid streaks and severe arterial lesions, notably stenosis of the vertebral arteries (especially the left) causing disorders in the mechanism of balance. There were, in addition, mild diabetes, hyperlipidaemia and abnormal cutaneous pigmentation. The second patient, an African, had been hospitalised for an optochiasmatic syndrome which further investigation, notably by arteriography, indicated was caused by compression of the visual pathways due to  massive bilateral carotid aneurysms. The patient had molluscoid pseudo-tumours of the axillae and she reported that her father was in the same condition. Although in the first case, cutaneous biopsy enabled diagnosis of systematized elastorrhexis to be confirmed, histological investigation in the second case revealed mainly hyaline sclerosis of the deep dermal layer. The diagnosis of Ehlers-Danlos disease, suggested for this second patient, could not be confirmed. These two cases emphasize the possibility of various neurological disorders occurring in the course of dystrophy of the connectivo elastic tissue.
D000328:Adult; D000793:Angioid Streaks / Q000150:complications; D001157:Arterial Occlusive Diseases / Q000150:complications* / Q000000981:diagnostic imaging; D002340:Carotid Artery Diseases / Q000150:complications; D003937:Diagnosis, Differential; D004535:Ehlers-Danlos Syndrome / Q000175:diagnosis; D005260:Female; D006801:Humans; D002532:Intracranial Aneurysm / Q000150:complications* / Q000000981:diagnostic imaging; D008875:Middle Aged; D009461:Neurologic Manifestations; D011561:Pseudoxanthoma Elasticum / Q000150:complications* / Q000000981:diagnostic imaging / Q000235:genetics; D011859:Radiography; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000150:complications*; D013577:Syndrome; D014786:Vision Disorders / Q000209:etiology
Elastopathie cutanée et anomalies vasculaires associées avec troubles neurologiques A propos de deux cas