Light and Electron Microscopic and Element Analysis of Pseudoxanthoma elasticum (Darier-Grönblad-Strandberg Syndrome)

Blümcke, S.; Langness, U; B, Liesegang; Fooke-Achterrath, M; Thiel, Hjalmar

doi:10.1016/s0005-8165(74)80118-6

Cited by 14 publications

(4 citation statements)

References 11 publications

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“…26,29 Generalized RPE degeneration, calcified BrM, and subretinal macrophage infiltration have been identified in Best disease and in angioid streaks. [30][31][32][33] Thus, within the RPE/BrM complex, RPE degeneration and BrM remodeling are common pathologic processes in both disorders.…”

Section: Discussionmentioning

confidence: 99%

Adult-Onset Best1-Vitelliform Dystrophy Associated With Angioid Streak-Like Changes in Two Siblings

Bracha

Alemán

et al. 2023

RETINAL Cases &Amp; Brief Reports

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Section: Discussionmentioning

confidence: 99%

Adult-Onset Best1-Vitelliform Dystrophy Associated With Angioid Streak-Like Changes in Two Siblings

Bracha

Alemán

et al. 2023

RETINAL Cases &Amp; Brief Reports

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“…In PXE, Ca is deposited in the elastic fibers of the dermis; this is also felt to be a secondary phenomenon (12) which may be preceded by undetected metal or trace element deposition. Only a few investigators (13)(14)(15)(16) have studied trace elements in the elastic fibers, and they have limited their investigations to Ca and P. We have specifically analysed both the degenerating elastic fibers and the sera of patients with PXE for trace elements, using a variety of instruments and techniques. Only Al was found to be present in any significant quantity in these patients; Fe, Ni, Co, Mn, Cr, Zn, Pb, Cd, S, K and CI were present in essentially normal amounts.…”

Section: Discussionmentioning

confidence: 99%

Trace Element Analyses in Pseudoxanthoma Elasticum

Kanzaki

Matsuno

Kamimura

et al. 1988

The Journal of Dermatology

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The dermis and sera of 11 patients with pseudoxanthoma elasticum (PXE) were analyzed by several techniques which test for the presence and quantity of trace elements (Fe, Ni, Co, Cu, Mn, Cr, Zn, Pb, Cd, AI, S, K, CI, Ca and P), The investigative methods included 1) electron microscopic point X-ray microanalysis, 2) inductively coupled plasma atomic emission spectrophotometric analysis (ICP-AES), 3) X-ray diffraction analysis and/or 4) atomic absorption spectro-photometric analysis, 1) Among 7 specimens of dermis showing histological degeneration of the elastic fibers, 2 showed prominent levels of AI. In early degeneration ofthe elastic fibers, Ca was present in only small amounts; however, in more fully developed lesions, large quantities of both Ca and P were found, while Fe, Co, Ma and Zn were detected in trace amounts. 2) ICP-AES analysis confirmed large deposits of AI, Ca, and P in diseased dermis (AI: 32J ppm wet tissue); other elements were found in normal quantities, AI was not detected in the control dermis, 3) X-ray diffraction analysis suggested the pattern of hydroxyapatite [Ca lO(P04 )6(O H )2]' which indicates that most of the Ca exists in a crystalline form. 4) Atomic absorption spectrophotometric analysis confirmed a large concentration of ug/dl, mean=17.2; normal <10) in all test patient's sera. The combined analysis revealed an increased amount of AI in patient's sera as well as in the elastic fibers of the dermis at an early stage of this disease (PXE). These findings suggest a possible role for AI in the pathogenesis of PXE.Abbreviations: PXE: pseudoxanthoma elasticum, ICP-AES: inductively coupled plasma atomic emission spectrometry

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“…However, both these abnormalities have not been confirmed by all the authors [29,34], Calcium, phosphate, and carbonate have been identified as constituents of the mineral deposits [1,4,6,18,41] and the hypothesis has been put for- ward that mineralization is a consequence of the deposition within the elastic fiber of polyanions that exhibit metachromatic properties and have high af finity for ruthenium red [28]. These polyanions have been recognized also in apparently unaffected fibers of the same subject [28], With regard to the amino acid composition of the elastin in PXE, the data available are rather scarce and seem to suggest an increased content in hydrophilic amino acids, such as glutamic and aspartic acids [3,32,38].…”

Section: Introductionmentioning

confidence: 92%

“…Pseudoxanthoma elasticum (PXE) is a genetically determined disease [7,34] in which the elastic fibers, especially in some areas, exhibit progres sing mineralization [1,4,6].…”

Section: Introductionmentioning

confidence: 99%

Pseudoxanthoma Elasticum

et al. 1981

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Ultrastructural and biochemical studies of the dermis from patients with pseudoxanthoma elasticum are described. Elastin fibers showed mineral deposits that were associated with an elastin material exhibiting altered affinity for the electron microscopical stains and altered ultrastructural organization. In the most affected patient the elastin was shown to be increased and to have an abnormal amino acid composition. In some patients the collagen fibers were laterally fused. The ground substance was increased as evidenced by electron microscopy and confirmed by a slight increase in uronic acids, hexosamines, and neutral sugars.

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Light and Electron Microscopic and Element Analysis of Pseudoxanthoma elasticum (Darier-Grönblad-Strandberg Syndrome)

Cited by 14 publications

References 11 publications

Adult-Onset Best1-Vitelliform Dystrophy Associated With Angioid Streak-Like Changes in Two Siblings

Adult-Onset Best1-Vitelliform Dystrophy Associated With Angioid Streak-Like Changes in Two Siblings

Trace Element Analyses in Pseudoxanthoma Elasticum

Pseudoxanthoma Elasticum

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