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[Systemic cutis laxa-like pseudoxanthoma elasticum]

H Schibli, S B Stutz, A Vogel

    Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete
    |February 1, 1982
    PubMed
    Summary
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    Pseudoxanthoma elasticum (PXE) type II presents with skin laxity and eye abnormalities, differing from classical PXE. This variant shows no signs of internal organ disease, offering a distinct clinical profile.

    Area of Science:

    • Dermatology
    • Ophthalmology
    • Genetics

    Background:

    • Pseudoxanthoma elasticum (PXE) is classified into four types based on clinical and genetic factors.
    • Recessive PXE type II is distinguished by skin laxity and wrinkling, without visceral manifestations.

    Observation:

    • Two patients presented with cutis laxa-like skin.
    • Both patients exhibited pathological ocular changes.
    • One patient also displayed peripheral vascular system alterations.

    Findings:

    • The presented cases align with the description of recessive PXE type II.
    • The findings highlight the ocular and vascular involvement in this PXE variant.
    • Absence of visceral disease symptoms was noted, consistent with PXE type II.

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    Implications:

    • This case report expands the understanding of PXE type II clinical spectrum.
    • It emphasizes the importance of ophthalmological and vascular evaluations in suspected PXE type II cases.
    • Further research into the genetic and clinical correlations of PXE subtypes is warranted.