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Dystrophic calcinosis cutis in pseudoxanthoma elasticum
Andrea Federico1, Sarah Weinel, Vilma Fabre
1New York University School of Medicine, New York, USA.
Journal of the American Academy of Dermatology
|March 18, 2008
Summary
Pseudoxanthoma elasticum (PXE) can lead to painful, widespread calcinosis cutis. This case highlights a severe presentation of PXE-associated dystrophic calcification, differing from previously reported milder instances.
Area of Science:
- Dermatology
- Medical Genetics
Background:
- Pseudoxanthoma elasticum (PXE) is a rare genetic disorder affecting elastic fibers, leading to calcification.
- Calcinosis cutis is characterized by calcium deposition in skin, with various forms including dystrophic.
Observation:
- A patient with PXE developed extensive and symptomatic dystrophic calcinosis cutis.
- This presentation was more severe and painful than the only other reported case of PXE with calcinosis cutis.
Findings:
- The patient experienced widespread, pruritic, and painful skin calcifications linked to PXE.
- Treatment with topical agents and aluminum hydroxide showed minimal improvement, compounded by patient noncompliance.
Implications:
- This case expands the understanding of PXE's cutaneous manifestations and the spectrum of calcinosis cutis.
- Further research into effective treatments for severe PXE-associated calcinosis cutis is warranted.
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