AI Article Synopsis

  • Pseudoxanthoma elasticum (PXE) is a rare genetic disorder that leads to abnormal calcium deposits in elastic tissue, causing skin changes and other health issues.
  • The study focused on a 13-year-old patient with flare-ups in PXE lesions, leading to a closer examination of the inflammation involved through skin biopsies and various analyses.
  • Findings showed inflammation with T-cell infiltrates in the skin, particularly with a Th1 response, suggesting an inflammatory role in PXE disease, but the exact impact on the condition needs more research.

Article Abstract

Pseudoxanthoma elasticum (PXE) is a genetic disorder characterized by ectopic calcification of tissues rich in elastic fibres (OMIM 264800). To date, PXE is considered a metabolic disease linked to an imbalance between pro- and anti-calcifying factors. The occurrence of sporadic erythematous flareups of PXE skin lesions is a complaint that we heard about on several occasions at the French PXE reference centre. However, this rare clinical aspect had never been extensively studied. We have had the opportunity to investigate a 13-year-old patient experiencing an erythematous flareup of his PXE lesions. We conducted this work to identify what type of inflammation was implicated in his lesions. An incisional skin biopsy on a recent erythematous inguinal PXE lesion was performed and sent for histological and transcriptomic analyses. The findings were compared to a non-erythematous PXE-affected skin biopsy obtained from another young PXE patient. Histological examination revealed perivascular T-cell infiltrates with Th1 polarity and elevated expression of cytotoxicity markers in RNAseq and RT-qPCR analyses. There was no increase in Th17 or Th2 markers. Our findings support the previous evidence of a possible inflammatory component in the development of PXE. Whether Th1-dependent inflammation contributes to the pathology as an active process or is an aggravating factor requires further investigations.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11608869PMC
http://dx.doi.org/10.1002/ski2.430DOI Listing

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