IJD
Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
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CASE REPORT
Year : 2012  |  Volume : 57  |  Issue : 3  |  Page : 210-212

Pseudoxanthoma elasticum with periumbilical perforation in a Nullipara


Department of Dermatology, Stanley Medical College, Chennai, India

Correspondence Address:
Parimalam Kumar
Department of Dermatology, Stanley Medical College. Chennai-600 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.96195

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Pseudoxanthoma elasticum (PXE) is an inherited multisystem disorder that primarily affects the skin and is characterized by progressive calcification and degeneration of the elastic fibers. PXE has recently been found to be caused by mutations in the ATP-binding cassette transporter C6 (ABCC6) or the multidrug resistance-associated protein 6 (MRP6) genes. Perforating PXE is a rare presentation that is usually seen in the periumbilical area in obese multiparous black women; it has distinct clinical and histopathological features and there may or may not be systemic manifestations. We report an unusual case of PXE in a nulliparous woman, with perforation in the periumbilical area and without any systemic involvement.


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