Pseudoxanthoma elasticum: a report of 11 cases

INTRODUCTION: Pseudoxanthoma elasticum (PXE) is an inherited disorder of elastic tissue with many systemic manifestations PATIENTS AND METHODS: We performed a retrospective study from all the patients diagnosed with PXE at the department of dermatology of La Rabta hospital of Tunis, between 1986 and 2003. RESULTS: During the observation period, we identified 11 patients with PXE, 5 males and 6 females with a mean age of 28 years (10-47 years). Family history was found in 5 patients. Exhibit yellowish, pigskin, and popular lesions on the sides of the neck were observed in all cases. Systematic ophthalmologic examination revealed angio?d streaks in 4 patients. No abnormalities were found in cardiovascular and metabolic explorations. DISCUSSION: Diagnosis of PXE is based on clinical, histological and genetic criteria. Ocular and cardiovascular damage make all the gravity of the disease, from where interest of an ophthalmologic and cardiovascular examination systematic.