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Perforating pseudoxanthoma elasticum. Its distinction from elastosis perforans serpiginosa
Archives of Pathology & Laboratory Medicine
|October 1, 1976
Summary
Pseudoxanthoma elasticum (PXE) can cause elastic fibers to perforate the epidermis, a phenomenon termed "perforating PXE." This condition is distinct from elastosis perforans serpiginosa (EPS), with most co-occurring cases likely being perforating PXE.
Area of Science:
- Dermatology
- Pathology
- Genetics
Background:
- Pseudoxanthoma elasticum (PXE) is a rare genetic disorder affecting elastic tissues.
- Clinical manifestations typically involve the skin, eyes, and cardiovascular system.
- Epidermal involvement is not widely recognized.
Observation:
- This report details a case of PXE with a unique feature: elastic fibers perforating the epidermis.
- The proposed term 'perforating PXE' describes this specific phenomenon.
- A clear distinction is drawn between perforating PXE and elastosis perforans serpiginosa (EPS).
Findings:
- The study suggests that most reported instances of coexisting PXE and EPS are, in fact, perforating PXE.
- Evidence for the true coexistence of EPS with PXE remains limited.
- Histopathological examination confirmed elastic fiber perforation through the epidermis.
Implications:
- Recognizing perforating PXE can refine diagnosis and management strategies for PXE patients.
- This finding may lead to re-evaluation of previously reported cases of PXE with EPS.
- Further research is warranted to fully understand the pathophysiology and prevalence of perforating PXE.