Familial Acral Localized Late-Onset Focal Dermal Elastosis

Camacho, Diana; Machan, Salma; Pielasinski, Úrsula; Revelles, Juan M.; Fariña, María del Carmen; Santonja, Carlos; Requena, Luís

doi:10.1097/dad.0b013e31823adf2e

Cited by 11 publications

(13 citation statements)

References 11 publications

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“…All three entities share an increased number of elastic fibers in the upper dermis. Elastic fibers can be both normal‐looking or thickened . Increased number of fibroblasts with prominent endoplasmic reticulum have been found in elastoderma confirming the excess synthesis of elastic fibers .…”

Section: Evg Staining In Primary Elastic Tissue Disordersmentioning

confidence: 86%

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The utility of elastic Verhoeff‐Van Gieson staining in dermatopathology

et al. 2012

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Elastic fibers are important components of the skin and are responsible for skin elasticity. Genetic defects are well-known in numerous hereditary elastic tissue disorders and skin biopsies are often the first step in the evaluation of those disorders. Verhoeff-Van Gieson elastic staining is a simple method that is used for visualizing elastic fibers. With the development of modern immunohistochemical methods, the value of routine histochemical staining is sometimes underestimated. Histochemical stains are less expensive, easy to perform and help to resolve numerous diagnostic quandaries in dermatopathology. This article focuses on the value of elastic tissue staining in dermatopathology, with a focus on primary elastic tissue disorders, alopecia, inflammatory skin disorders and neoplastic proliferations. Connective tissue comprises of three types of fibers: collagen fibers, reticular fibers and elastic fibers. These fibers are made of proteins and composed of long peptide chains. While the main function of collagen and reticular fibers is to provide tensile strength and support respectively, the elastic fibers are mainly there for providing stretchability and flexibility. Elastic fibers can stretch up to 1.5 times their length, and snap back to their original length when relaxed. The long and branching arrangement of fibers enables them to stretch and recoil thus providing flexibility to tissues. Elastic fibers are composed of two structural components: elastin and microfibrils, and include elastin, elaunin and oxytalan.

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Section: Evg Staining In Primary Elastic Tissue Disordersmentioning

confidence: 86%

“…Prolonged sun exposure has also been suggested as a possible etiology . Familial cases of late‐onset focal dermal elastosis and linear focal elastosis have been reported …”

Section: Evg Staining In Primary Elastic Tissue Disordersmentioning

confidence: 99%

The utility of elastic Verhoeff‐Van Gieson staining in dermatopathology

et al. 2012

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“…Late-onset focal dermal elastosis (LOFDE) is a rare disorder of elastic tissue, characterized by a PXE-like yellowish papular eruption, with local accumulation of elastic fibers in the mid- and deep reticular dermis [66, 67]. The disease occurs mainly in elderly people and typically affects the sides of the neck and flexural areas, closely mimicking PXE from the clinical point of view [66].…”

Section: Dermatological Diseases With (Near-) Identical Skin Lesionsmentioning

confidence: 99%

“…The disease occurs mainly in elderly people and typically affects the sides of the neck and flexural areas, closely mimicking PXE from the clinical point of view [66]. The elderly onset of this disease suggests that LOFDE may be a disorder of aging.…”

Section: Dermatological Diseases With (Near-) Identical Skin Lesionsmentioning

confidence: 99%

“…The most prominent histopathologic feature is a local accumulation of normal-appearing elastic tissue or thick, interlacing elastic fibers in the mid- and deep reticular dermis without fragmentation, calcification, or phagocytosis of elastic fibers [69–71]. A hydroxyproline assay of lesional skin may show a modest increase in collagen content and a more substantial increase in elastin [72].…”

Section: Dermatological Diseases With (Near-) Identical Skin Lesionsmentioning

confidence: 99%

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Histopathology of Pseudoxanthoma Elasticum and Related Disorders: Histological Hallmarks and Diagnostic Clues

2012

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Among ectopic mineralization disorders, pseudoxanthoma elasticum (PXE)—a rare genodermatosis associated with ocular and cardiovascular manifestations—is considered a paradigm disease. The symptoms of PXE are the result of mineralization and fragmentation of elastic fibers, the exact pathophysiology of which is incompletely understood. Though molecular analysis of the causal gene, ABCC6, has a high mutation uptake, a skin biopsy has until now been considered the golden standard to confirm the clinical diagnosis. Although the histological hallmarks of PXE are rather specific, several other diseases—particularly those affecting the skin—can present with clinical and/or histological characteristics identical to or highly resemblant of PXE. In this paper, we will summarize the histopathological features of PXE together with those of disorders that are most frequently considered in the differential diagnosis of PXE.

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Acquired Disorders of Dermal Connective Tissue

Fahy,

Lovell

2024

Rook's Textbook of Dermatology

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This chapter reviews acquired changes in dermal connective tissue during normal ageing and in disease processes, including photodamage. Corticosteroids are a potent cause of dermal atrophy, which may also be congenital or induced by inflammatory disorders related to morphoea. Enzymatic degradation of elastic tissue results in localised or generalised skin laxity, which resembles genetic disorders such as pseudoxanthoma elasticum and cutis laxa. In contrast, some disorders are characterised by excessive deposition of collagen or elastic tissue. Fibrosis is associated with contracture, which causes deformity and restricted movement as in palmoplantar fibromatosis. Several chemical environmental triggers induce diffuse fibrosis resembling systemic sclerosis. Keloids represent an abnormal scarring response to trauma. Finally, dermal connective tissue components may be extruded via the epidermis in perforating disorders such as elastosis perforans serpiginosa.

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Familial Acral Localized Late-Onset Focal Dermal Elastosis

Cited by 11 publications

References 11 publications

The utility of elastic Verhoeff‐Van Gieson staining in dermatopathology

The utility of elastic Verhoeff‐Van Gieson staining in dermatopathology

Histopathology of Pseudoxanthoma Elasticum and Related Disorders: Histological Hallmarks and Diagnostic Clues

Acquired Disorders of Dermal Connective Tissue

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