Structure of the Dermis in Type VIIC Ehlers—Danlos Syndrome

Pierard, Gérald; Hermanns‐Lê, Trinh; Arrese-Estrada, Jorge; Piérard-Franchimont, C; Lapière, Charles M.

doi:10.1097/00000372-199304000-00005

Cited by 15 publications

(10 citation statements)

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“…Some classes of monocytes and macrophages may also contain factor XIIIa [14,38]. The characteristics of factor XIIIa-positive cells have been studied most extensively in human skin, in which there is a population of dermal dendritic cells that bind antibodies against factor XIIIa [6,15,36,37,62,63]. They have been shown to display phagocytic functions [5,16], to act as antigen-presenting cells [15,37] and to be involved in regulatory mechanisms of deposition of extracellular macromolecules in the dermis [61].…”

Section: Introductionmentioning

confidence: 99%

Inverse modulation of intraepithelial Langerhans' cells and stromal macrophage/dendrocyte populations in human papillomavirus-associated squamous intraepithelial lesions of the cervix

Al-Saleh

Delvenne

Arrese

et al. 1995

Vichows Archiv A Pathol Anat

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Ninety-four cervical biopsies from normal tissue to high-grade squamous intraepithelial lesion (SILs) were examined for the presence of intraepithelial Langerhans' cells and subpopulations of stromal macrophages/dendrocytes by immunohistochemistry using anti-S100, -L1, -CD68 and -factor XIIIa antibodies. Human papillomavirus (HPV) detection was performed in all cases by using first a mixture of DNA probes for 14 HPV types commonly found in anogenital biopsies at low stringency conditions (Tm -40 degrees C) and by reanalyzing the tissues at high stringency (Tm -10 degrees C) with HPV 6/11, 16/18 and 31/33/35 biotinylated probe cocktails and individual digoxigenin-labelled probes. SILs and metaplastic tissues were significantly associated with a depletion of S100-positive intraepithelial Langerhans' cells when compared with normal epithelium. In contrast, there was a significant increase in L1-positive stromal macrophages in SIL biopsies compared with normal or metaplastic cervix. A significantly higher density of CD68-positive macrophages was also observed in high-grade SILs compared with normal or metaplastic biopsies and with low-grade SILs. The density of factor XIIIa-positive dendrocytes was found to be higher in SILs compared with metaplastic tissues and in high-grade SILs when compared with normal cervical biopsies. No specific relationship was found between the densities of these cells and the HPV type detected in SILs separated into low grade and high grade. The significance of this inverse modulation of intraepithelial Langerhans' cells and stromal macrophages/dendrocytes in normal and SIL biopsies is discussed in relation to HPV infection and malignant transformation.

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Section: Introductionmentioning

confidence: 99%

Inverse modulation of intraepithelial Langerhans' cells and stromal macrophage/dendrocyte populations in human papillomavirus-associated squamous intraepithelial lesions of the cervix

Al-Saleh

Delvenne

Arrese

et al. 1995

Vichows Archiv A Pathol Anat

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“…3 The proper synthesis, accumulation, and cross-linking of collagen molecules, and most importantly the orderly packing of collagen fibrils in bundles impart the tensile strength of the skin. The abnormal collagen composition, as well as the morphologic changes in the collagen bundles and fibrils, have been amply described in EDS, [4][5][6][7][8][9][10][11] but elastic fibers, hyaluronic acid, and other proteoglycans have rarely been studied.…”

mentioning

confidence: 99%

Ultrastructural Alterations of Elastic Fibers and Other Dermal Components in Ehlers-Danlos Syndrome of the Hypermobile Type

Hermanns‐Lê

Pierard

2007

The American Journal of Dermatopathology

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Ehlers-Danlos syndrome (EDS) is a heterogeneous group of connective tissue disorders with varied molecular abnormalities. Some morphologic alterations are described in collagen and elastic fibers. We report on previously undescribed ultrastructural changes in the elastic fibers found in the skin of 22 of 29 patients suffering from the hypermobile type of EDS. They existed in variable combinations of a fragmented appearance with frayed contours, internal microcavities, some elastotic-like changes, and discrete calcified foci. The collagen fibrils and their bundles were also altered with morphologic aspects reminiscent of those found in the classical type of EDS. Large globules of hyaluronic acid were also dispersed in the amorphous matrix.

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“…Therefore, other investigations should be performed to confirm the precise diagnosis of EDS. Histopathological dermal changes with reduction or absence of Factor XIII a+ dermal dendrocytes type1 (DD1) are observed in the EDS classic type [40] and in dermatosparaxis [41]. In EDS, there are no type-specific ultrastructural alterations in collagen and elastic fibers, except for the hieroglyphic-shaped doi:…”

Section: Diagnosismentioning

confidence: 99%

“…fibril cross-sections of the EDS dermatosparaxis type [41]. In spite of heterogeneity in both the structural and biochemical abnormalities, the overall architecture and ultrastructure of the dermal components changes are of diagnostic relevance and they occasionnaly suggest the EDS type [42][43][44][45], Furthermore, they contribute rapidly to the diagnosis before getting the information from genetic analysis.…”

Section: Diagnosismentioning

confidence: 99%

Gynecologic and obstetric impact of the ehlers-danlos syndrome : clues from scrutinizing dermal ultrastructural alterations

Hermanns‐Lê¹,

Pierard²,

Piérard-Franchimont³

et al. 2014

Gynecol

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Background: The Ehlers-Danlos syndrome (EDS) represents a heterogenous group of heritable connective tissue disorders chiefly characterized by joint laxity, increased skin distensibility and connective tissue fragility. Each EDS type corresponds to a multisystemic disorder with widespread implications. Physicians evoke the EDS diagnosis in presence of some typical cutaneous signs, such as atrophic scars and frequent hematomas. Methods: To revisit the transmission electron microscopy support from skin biopsies when screening EDS in gynecologyobstetrics. Results: EDS women commonly suffer from a series of gynecologic and obstetric disorders. Dermal ultrastructural changes are keynote features suggesting some EDS types. Conclusion: Gene mutations are mostly identified in the classic and vascular EDS types, but only in sporadic cases of the hypermobile EDS type which apparently represents the most frequent type. Dermal ultrastructural changes represent relevant diagnostic clues for the practitioner. Preconception counseling about possible complications is welcome. Management requires precise diagnosis of the EDS type.

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Structure of the Dermis in Type VIIC Ehlers—Danlos Syndrome

Cited by 15 publications

References 0 publications

Inverse modulation of intraepithelial Langerhans' cells and stromal macrophage/dendrocyte populations in human papillomavirus-associated squamous intraepithelial lesions of the cervix

Inverse modulation of intraepithelial Langerhans' cells and stromal macrophage/dendrocyte populations in human papillomavirus-associated squamous intraepithelial lesions of the cervix

Ultrastructural Alterations of Elastic Fibers and Other Dermal Components in Ehlers-Danlos Syndrome of the Hypermobile Type

Gynecologic and obstetric impact of the ehlers-danlos syndrome : clues from scrutinizing dermal ultrastructural alterations

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