Title: Periumbilical Perforating Pseudoxanthoma Elasticum / PPPXE – A Rare Case Report
Authors: Dr Prajul Mehta, Dr Ashwani Rana
DOI: https://dx.doi.org/10.18535/jmscr/v9i11.02
Abstract
Pseudoxanthoma elasticum (PXE) is a rare inherited disorder involving degeneration and pathological mineralization of the elastic fibers. It predominantly involves the skin, eyes and cardiovascular system. Periumbilical perforating pseudoxanthoma elasticum (PPPXE) is a rare localized variant of PXE frequently seen in obese, multiparous, middle-aged women. We hereby report a rare case of PPPXE in a multiparous woman, presented with a large yellowish plaque around the umbilicus without any systemic involvement. The other sites usually affected were not involved.
Keywords: PXE, PPPXE, elastic fibres.
