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Contributor

Jonathan Miller, BS

Diagnosis

pseudoxanthoma elasticum

Body Site

neck

Age

31 years

Pigmentation

Organization

Color

Morphology

Pattern

Comments

This 31-year-old man presented with painless, progressive loss of vision and was found to have angioid streaks and drusen of the optic nerve head on ophthalmalogic exam. The angioid streaks represent tears in Bruch's membrane. A skin biopsy showed changes typical of pseudoxanthoma elasticum. In PXE, calcification also occurs in the internal elastic lamina of vessles leading to atherosclerosis and eventually coronary disease, cerebrovascular disease, and renovascular hypertension.

Description

Higher power demonstrates short, wavy, irregularly shaped basophilic elastic fibers in the middle third of the dermis. A Von Kossa stain showed marked calcium deposition.

Categories

cutaneous sign of systemic disease genodermatosis/genetic disorder

Image Added

12/15/2004 21:39:40

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Other Resources

PubMed Medline Plus Medscape

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