PERFORATING PSEUDOXANTHOMA ELASTICUM
By Gary M. White, MD
Perforating pseudoxanthoma elasticum (P-PXE) is a very rare skin condition caused by abnormal elastic fibers.
- A hyperpigmented, keratotic periumbilical plaque, usually in an obese, multiparous, black or Mediterranean woman is characteristic.
- This disease has also been called perforating calcific elastosis.
- Classic pseudoxanthoma elasticum does not usually perforate.
- Histopathology shows mid-dermal elastic fibers which have degenerated, calcified and are subsequently extruded.
A hyperpigmented, keratotic periumbilical plaque, usually in an obese, multiparous, black or Mediterranean woman is characteristic although Caucasian women may also be affected. Some patients present with a background of yellow, cobblestone papules and nodules with a few hyperkeratotic (perforating) lesions.
Classic PXE should be excluded with eye exam (Angioid streaks), review of systems to include cardiac problems, hypertension, and GI bleeding. Although there is no specific treatment, patients may be reassured.
Advances in Medicine.
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