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Image Number #275 (Pseudoxanthoma elasticum)

Diagnosis: Pseudoxanthoma elasticum

Description: Note the broken curled elastic fibres in the dermis with some deep calcification.

Morphology: Papules,yellow

Site: Groin adult

Sex: M

Age: 35

Type: Clinical & Histology

Submitted By: Ian McColl

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Differential Diagnosis
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History:

Pseudoxanthoma elasticum is a rare inherited disease with damage to elastic fibres in the skin causing weakness of the skin with loss of elaticity.As the elastic fibres in blood vessels are also involved patients are susceptible to strokes and heart attacks at an early age.

The patient shown above had already had a stroke at aged 35.His groin skin was very lax due to the loss of elasticity.His images show the classic plucked chicken appearance.This is described as the finding of multiple yellowish flat topped papules both discrete and confluent , in the skin creases of the sides of the neck and the flexures.

DermNetNZ   eMedicine   PubMed   Dermatology Online   Archives   JAAD for "Pseudoxanthoma elasticum"

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