Unusual remodeling of the hyalinization band in vulval lichen sclerosus by type V collagen and ECM 1 protein

Godoy, Charles Antônio Pires de; Teodoro, Walcy Rosolia; Velosa, Ana Paula Pereira; Garippo, A. L.; Eher, Esmeralda Miristeni; Parra, Edwin R.; Sotto, Mírian Nacagami; Capelozzi, Vera Luíza

doi:10.6061/clinics/2015(05)09

Cited by 21 publications

(11 citation statements)

References 36 publications

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“…In addition to hyalinized and homogenized, sclerotic collagen fibrils, Godoy et al 82 observed reduced elastic fibers in the upper dermis of LS lesions, which was found to be associated with aberrantly increased collagen V deposition (in addition to type I & III collagen) and low ECM1 blood vessel expression within the hyalinized LS zone ( Table 2 ). Given that type V collagen is known to induce scleroderma in rabbits 83 and lung transplant rejection 84 it is possible that overexpression of collagen V may explain the observed collagen remodeling observed in LS 82.…”

Section: Autoimmune and Genetic Targets Of Lichen Sclerosusmentioning

confidence: 99%

Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

Tran¹,

Tan²,

Macri³

et al. 2019

Int. J. Biol. Sci.

107

108

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Lichen sclerosus (LS) is an inflammatory dermatosis with a predilection for anogenital skin. Developing lesions lead to vulvar pain and sexual dysfunction, with a significant loss of structural anatomical architecture, sclerosis, and increased risk of malignancy. Onset may occur at any age in both sexes, but typically affects more females than males, presenting in a bimodal fashion among pre-pubertal children and middle-aged adults. A definitive cure remains elusive as the exact pathogenesis of LS remains unknown. A general review of LS, histologic challenges, along with amounting support for LS as an autoimmune disease with preference for a T h 1 immune response against a genetic background is summarized. In addition to the classically referenced ECM1 (extracellular matrix protein 1), a following discussion of other immune and genetic targets more recently implicated as causative or accelerant agents of disease, particularly miR-155, downstream targets of ECM1, galectin-7, p53, and epigenetic modifications to CDKN2A, are addressed from the viewpoint of their involvement in three different, but interconnected aspects of LS pathology. Collectively, these emerging targets serve not only as inherently potential therapeutic targets for treatment, but may also provide further insight into this debilitating and cryptic disease.

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Section: Autoimmune and Genetic Targets Of Lichen Sclerosusmentioning

confidence: 99%

Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

Tran¹,

Tan²,

Macri³

et al. 2019

Int. J. Biol. Sci.

107

108

View full text Add to dashboard Cite

show abstract

“…Dysfunction of ECM1 in lichen sclerosus occurs due to an autoimmune reaction and leads to hyperkeratosis and/or atrophy of the epithelium, homogenization, sclerosis, and hyalinosis of collagen fibers in the dermis, and damage to the endothelium leading to increased permeability of the vascular wall [4]. In lichen sclerosus, a decrease in ECM1 expression in blood vessels occurs against the background of increased synthesis of type V collagen, which causes hyalinisation of the subepithelial zone [5]. It is important to note that the hyalinization of blood capillaries and collagen fibers of the papillary dermis is a classic sign of lichen sclerosus; back in 1986, this symptom was proposed as a marker of severity of this disease [6].…”

Section: Introductionmentioning

confidence: 99%

Multimodal Optical Coherence Tomography: Imaging of Blood and Lymphatic Vessels of the Vulva

Sirotkina¹,

Potapov²,

Vagapova³

et al. 2019

Sovrem Tehnol Med

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The aim of the study was to test the method of optical coherence tomography (OCT) for its ability to obtain images of blood and lymphatic vessels of the vulva.Materials and Methods. The study was performed using a multimodal optical coherence tomography device developed in the Institute of Applied Physics of the Russian Academy of Sciences (Nizhny Novgorod). In this setup, 3D images of 3.4×3.4×1.25 mm are created within 26 s. OCT angiography and OCT lymphangiography are based on the speckle structure analysis. Visualization of blood and lymphatic vessels does not require any additional contrast agents.A histological study of vulvar biopsy samples from two locations was performed in 3 patients without vulvar pathology and in 5 patients with vulvar lichen sclerosus; the results were then compared with 3D OCT images obtained at the same locations.Results. Using the multimodal OCT, we found that normal mucous membrane of the vulva had a well-developed network of blood and lymphatic vessels; their diameters were increasing with depth. In the subepithelial zone, only blood capillary loops could be seen, whereas lymphatic capillaries became detectable starting from a depth of 170 μm (submucosa layer).In the case of vulvar lichen sclerosus, the density of the blood vessel network decreases dramatically and the capillary loops in the subepithelial zone disappear. Lymphatic vessels become detectable at a depth of 300 μm, and only few thin vessels can be detected at depths of 300 to 600 μm. The results suggest that the presentation of blood and lymphatic vessels depends on the state of the vulvar connective tissue. Specifically, the number of blood and lymphatic vessels is reduced in areas of hyalinosis and sclerosis of collagen fibers.Conclusion. For the first time, using multimodal OCT, an in vivo study of blood and lymphatic vessels of the vulvar mucosa was performed on normal and lichen sclerosus-affected vulva; the obtained OCT images were then compared with histological images of the same tissues. The OCT method allows one to diagnose a zone of hyalinosis and sclerosis of collagen fibers characterized by an abnormally low number of blood and lymphatic vessels.

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“…Further potential actors of these histopathological changes are collagen V (COLV) and galectin-7 which both appear increased in VLS [84,85]. Increased COLV expression, and contextual ECM1 decrease, may contribute to both the disappearance of elastic fibres in the upper homogenized dermis and the abnormal collagen metabolism.…”

Section: Abnormal Collagen Metabolismmentioning

confidence: 99%

Vulvar Lichen Sclerosus from Pathophysiology to Therapeutic Approaches: Evidence and Prospects

et al. 2021

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Vulvar lichen sclerosus (VLS) is a chronic, distressing, inflammatory disease with an enormous impact on quality of life. Treatment goals are relieving symptoms, reversing signs and preventing anatomical changes. Despite the availability of numerous therapeutic options, treatment outcome may not be entirely satisfactory and a definitive cure does not exist. This may be due to the fact that the exact VLS etiopathogenesis remains unknown. The objectives of this paper were to review the most up-to-date knowledge on VLS etiopathogenesis and to consider the available therapies through the lens of a plausible pathogenetic model. An electronic search on both VLS etiopathogenesis and its treatment was performed using the National Library of Medicine PubMed database. Based on current knowledge, it is conceivable that various, heterogeneous environmental factors acting on a genetic background trigger an autoimmune, Th-1 response, which leads to a chronic inflammatory state. This, in turn, can determine both tissue and micro-vascular injury and activation of signaling pathways involved in fibroblast and collagen metabolism. This pathogenetic sequence may explain the effectiveness of anti-inflammatory treatments, mostly topical corticosteroids, in improving VLS clinical-pathological changes. Further deepening of the disease pathways will presumably allow key mediators to become new therapeutic targets and optimize the available treatments.

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Unusual remodeling of the hyalinization band in vulval lichen sclerosus by type V collagen and ECM 1 protein

Cited by 21 publications

References 36 publications

Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

Multimodal Optical Coherence Tomography: Imaging of Blood and Lymphatic Vessels of the Vulva

Vulvar Lichen Sclerosus from Pathophysiology to Therapeutic Approaches: Evidence and Prospects

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