Manifestaciones orales asociadas al pseudoxantoma elástico

Abstract

Elastic pseudoxanthoma is a rare hereditary dysplastic disorder of the connective tissue characterized by aberrant dystrophic calcification and progressive abnormal mineralization of the elastic tissues and collagen. The disease affects tissues containing abundant elastic fibers in different organ systems, producing characteristic and predominantly dermatological, ophthalmologic and vascular lesions. Elastic pseudoxanthoma progresses throughout the life of the patient and can occasionally also affect the oral cavity -typically the internal mucosa of the lower lip, in the form of yellowish plaques or papules. These alterations have been described in patients with severe skin involvement. The present study describes four patients with elastic pseudoxanthoma belonging to the same family, in different stages of the disease and hence with variable oral involvement. The clinical characteristics of these manifestations are described, along with their variable location and evolution.