Abstract
The pseudoxanthoma elasticum is a multisystemic heritable disease that primarily affects the connective tissue. It has been characterized by fragmentation and calcification of elastic fibers that can lead to complications of skin and cardiovascular system and changes in retina. Involvement of the oral mucosa has been described like white patches striated especially in the mucosa of both upper and lower lips. These oral signs are potentially useful to diagnose the disease, since it is an often undiagnosed disease due to the variability in phenotypic expressions. This study reports a case of pseudoxanthoma elasticum affecting a woman who developed lesions in the oral mucosa during the disease progression. Intraoral clinical assessment revealed the presence of changes mainly in lower labial mucosa and also slightly changes in the mouth floor and the upper labial mucosa. Therefore, the acknowledgment of oral pseudoxanthoma elasticum lesions helps dental practitioners to establish an early and appropriate diagnosis of this disease. This is very important because pseudoxanthoma elasticum is a multisystem disease with morbidity and mortality, and its early diagnosis and also the establishment of a follow-up protocol for these patients could prevent systemic and oral complications.