Pseudoxanthoma Elasticum

Pseudoxanthoma elasticum (PXE) is an inherited disease of elastic tissue primarily affecting the skin, eyes, and arteries. We report a clinical case to emphasize the need for early diagnosis and follow-up, a multidisciplinary approach and the management of complications. A 62-year-old patient consulted for wrinkled layers of papules typically "shagreen". The skin lesions were disseminated throughout the body but predominated in the folds, abdomen and neck. This generalized form would be due to the delay in diagnosis. The extra cutaneous lesions make all the seriousness of this disease which requires lifelong monitoring, The ophthalmologic manifestations of PXE are potentially the most serious. In any case, they are the most dreaded by patients. There is a risk of reduced visual acuity and sometimes blindness. PXE patients frequently develop early peripheral atherosclerosis, from the fourth decade , manifested by decreased peripheral pulses, and sometimes intermittent claudication. Cardiovascular clinical signs in PXE patients may also include arterial hypertension, and exceptionally signs of angina pectoris. Other attacks are frequent (rheumatological, renal...). No specific treatment is available to date but A number of etiological treatments to prevent complications are currently being developed.