Periumbilical Perforating Pseudoxanthoma Elasticum

ABSTRACT

Periumbilical perforating pseudoxanthoma elasticum (PPPXE) is a localized acquired disorder found most frequently in obese, multiparous, middle-aged women. It is characterized clinically by yellowish, lax, well-circumscribed, reticulated or cobblestoned patches or plaques in the periumbilical region. Multiparity, obesity, massive ascites, and abdominal surgery are thought to the initiating factors. There is controversy about the etiology of PPPXE. Some authors have classified it as a separate entity from hereditary pseudoxanthoma elasticum (PXE), while others speculate that this condition merely represents a variable expression of PXE in which systemic associations are likely. We report a case of periumbilical perforating pseudoxanthoma elasticum associated with a clinical PXE lesion on the anterior neck.