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Secondary anetoderma overlying pilomatrixomas.

Five pilomatrixomas with anetodermic cutaneous changes are presented. Four large tumors revealed soft, atrophic, pink translucent skin covering a firm subcutaneous mass, and their appearance was similar to that of a keloid or hypertrophic scar. One small tumor revealed soft, slightly atrophic, reddened or purplish skin covering a firm subcutaneous mass: its clinical appearance was similar to that of a hematoma or minor infection. Histopathological examination of the skin showed atrophic and edematous changes in the dermis associated with diminished fragmented collagen and absent elastic tissue. It is supposed that the dermal atrophic appearance bears a relation to the loss of elastic fibers and the dermal edema is related to the leakage of lymphatic fluid. Both phenomena may be caused by continuous pressure from the outside, as these anetodermic pilomatrixomas were located where they were apt to be exposed to mechanical irritation.

Adolescent↗

Elastic elements in the media and adventitia of human intracranial extracerebral arteries.

We find that the media and adventitia of adult human cerebral arteries contain elastic fibers forming a dense, coherent network, similar to that found in muscular arteries of the same size in other organs. The external elastic layer in the adult human is masked for the currently employed staining methods. By treatment with 90% formic acid before fixation, the original staining character of elastic tissue can be restored. The light microscopic and scanning electron microscopic features of this network of elastic fibers are presented.

Adolescent↗

Analysis of mammalian connective tissue: relationship between hierarchical structures and mechanical properties.

It is widely accepted that the mechanical properties of implants must match those of the surrounding connective tissue to prevent stress concentration and premature failure. The purpose of this paper is to review the structure-mechanical property relationships that exist for connective tissue. The mechanical properties of connective tissue depend on the content of collagen, elastic tissue, and proteoglycans, as well as the geometric arrangement of the fibrous components, age, and location of the specimen. To a first approximation the geometry and loading pattern of the collagen networks in these tissues dominate the mechanical response at high strains. The behavior of the elastic fiber networks dominate the low-strain mechanical response in tissues where energy and shape recovery are critical. Proteoglycans are involved in resisting tissue compressive forces. Since the stiffness of connective tissue increases with age, it is necessary to attempt to match this property by designing implants that have similar behaviors to insure that stress concentration does not occur at the interface between the implant and host.

Biomechanical Phenomena↗

Radiation therapy induces tenascin expression and angiogenesis in human skin.

In analysing radiation-induced connective tissue changes, we studied tenascin expression, elastic fibres, angiogenesis and physio-mechanical properties in irradiated and contralateral healthy skin of radiotherapy-treated breast cancer patients. Skin biopsies were obtained from a radiotherapy-treated skin area and a corresponding non-treated skin area. Haematoxylin-eosin and Verhoeff stainings as well as immunohistochemical stainings for tenascin and factor VIII were performed. Epidermal and total skin thickness, together with the amount of elastic tissue calculated by computerized digital image analysis, were measured. Suction blisters were induced on both skin areas. Transepidermal water loss was analysed. Skin elasticity was also measured. Tenascin expression was found to be increased in irradiated human skin. In haematoxylin-eosin and factor VIlI-stained sections, an increase in the number of blood vessels was detected. Although skin stiffness measured by an elastometer was increased in irradiated skin, no marked difference in the elastic fibres could be found between treated and non-treated skin. The increased tenascin expression could be due to activation of cytokines as a result of irradiation. An increase in angiogenesis could be caused by an activation of angiogenetic factors by irradiation or due to direct radiation damage on blood vessel walls. Our findings suggest that the effects of irradiation tend to accumulate in the dermal parts of skin. The higher skin stiffness values measured by elastometer in irradiated skin could be due to an accumulation of dermal connective tissue as a result of irradiation.

Adult↗

[Elastolysis of the eyelids. A rare cause of ptosis].

BACKGROUND: Mid-dermal elastolysis is a rare disorder of elastic tissue which is characterized clinically by wrinkling of the skin and histologically by the selective absence of elastic fibers in the mid dermis. Two patients with severe ptosis and a loss of the lateral tarsal suspension are reported. PATIENTS AND METHODS: A 36-year-old male patient presented with bilateral ptosis, skin atrophy of the eyelids and a loss of lateral tarsal suspension. With normal clinical and routine laboratory investigations, punch biopsy revealed clear signs of mid-dermal elastolysis. Plastic surgery of both eyelids (levator advancement) was performed with good success after a 1-year interval without progression of ptosis. Another 37 year old patient suffered from mild ptosis, skin atrophy of the eyelids and a loss of lateral tarsal suspension. As the patient chose not to have surgical treatment, he was treated with oral corticosteroids. To differentiate between an acute and a chronic type of elastolysis a punch biopsy is required. In addition, we want to highlight the surgical option in chronic and non-progressive cases. CONCLUSIONS: In contrast to formerly reported localized forms of acute acquired cutis laxa with complete loss of elastic fibres in the reticular and papillary dermis, we report a chronic type of elastolysis in these patients. In our experience in cases without any apparent progression of symptoms a surgical repair can provide long-term success.

Adult↗

Age-related changes in elastic fibers and elastin of lung.

The effect of age on the lung elastic tissue of inbred BALB/c mice were studied. Static compliance of excised lungs increased with age. Morphometrically determined total elastic fiber length increased with lung expansion in age- and sex-matched mice (r = 0.83, P smaller than 0.001), indicating an axial extension of elastic fibers. However, total elastic fiber length of aging lungs fixed at a distending pressure of 15 cm H2O showed no significant change despite an age-related increase in lung volume (male, r = 0.96, P smaller than 0.001; female, r = 0.95 P smaller than 0.001). The correlative finding of decreased elastin content (r = -0.87, P smaller than 0.001) indicates that there is a loss of elastic fibers in the aging lung. It is suggested that the absence of pseudoelastin fibers, as demonstrated by histochemical techniques, accounts for the observed differences in elastin content of aging human and mouse lungs.

Aging↗

[Formation of elastic fibers around basal cell carcinomas: light microscopical observations (author's transl)].

From 275 specimens of all sorts of basal cell epitheliomas the new formation of elastic fibers was histologically investigated by means of selective elastic tissue stains such as orcein and resorcinfuchsin. In nearly one third of the basal cell epitheliomas newly formed elastic fibers could be observed in the stroma. In places where a new formation of elastic fibers was demonstrated fibroblasts and mast cells (toluidine blue stain) were unusually numerous. Apparently the influence of the growing tumor masses of basal cell epitheliomas leads to a dermo-epidermal interaction in the form of 1. increase of mast cells 2. stimulation of fibroblasts 3. new formation of elastic fibers. The observation suggest that there exists a relationship between the above noted findings. Due to the dermo-epithelial interaction (even in senile skin) in the stroma of basal cell epithelioma fibroblasts can be stimulated to such a degree that they regain the ability to produce elastic fibers-a function which usually is greatly diminished with increasing age. The findings seem to be important from the view point of rejuvenation of aging human skin.

Adult↗

Controlled pressure-volume factors in the enlargement of intracranial aneurysms.

Pressure-volume relations were investigated on a model aneurysm wall made of elastic tissue and collagen. The model wall had a Young's Modulus of 2 x 10(7) dynes/cm2, approximating the elastance of fresh aneurysm walls obtained at autopsy. The model wall was fixed over the top of a glass T-tube, 6 mm in diameter. Pressure pulse waves of water or outdated human blood entered at the bottom of the T-tube and exited by way of a controlled resistance, while pressure was monitored by a strain gauge and recorded on an ink writer from the other arm. Incremental increases in systolic pressure produced a nonlinear N-shaped pressure-volume curve. There was an initial linear enlargement in volume with increased pressure until a pressure threshold was reached at a point of high nonlinear compliance. At this point there was an abrupt jump in volume (mean increase = 70 +/- 14%) to a new stable equilibrium volume. A further increase in pressure (24 +/- 7 mm Hg) could eventually cause aneurysm rupture. This always occurred in the thinner, more compliant part of the wall. With higher pulse rates, the jump in volume occurred at a smaller volume, whereas with thinner aneurysm walls of higher compliance the pressure threshold for a jump in volume was significantly lower. At a higher peripheral resistance there was a higher pressure threshold. The authors suggest that there is one possible mechanism that may occur in the neck of some developing human aneurysms and could explain early and rapid initial growth in size.

Collagen↗

[Study of elastic fiber changes of human skin in distinction between antemortem and postmortem wounds].

Elastic fiber changes of volunteers' antemortem and postmortem skin wounds of various time were observed in order to find possible differences between them. These sections of the wounds were stained by Hart's modification of Weigert's elastic tissue stain.. However, this study showed that there were no differences in the nature and distribution of the elastic fibers in the dermis of volunteers' antemortem and postmortem human skin wounds. Therefore, this suggests that the appearances of the elastic fibers in the dermis should not be as a means of differentiating antemortem skin wounds from postmortem wounds in forensic identification.

Adolescent↗

Fibrillin-1 and fibulin-2 interact and are colocalized in some tissues.

Microfibrils 10-12 nm in diameter are found in elastic and non-elastic tissues with fibrillin as a major component. Little is known about the supramolecular structure of these microfibrils and the protein interactions it is based on. To identify protein binding ligands of fibrillin-1, we tested binding of recombinant fibrillin-1 peptides to different extracellular matrix proteins in solid phase assays. Among the proteins tested, only fibulin-2 showed significant binding to rF11, the N-terminal half of fibrillin-1, in a calcium-dependent manner. Surface plasmon resonance demonstrated high affinity binding with a Kd = 56 nM. With overlapping recombinant fibrillin-1 peptides, the binding site for fibulin-2 was narrowed down to the N terminus of fibrillin-1 (amino acid positions 45-450). Immunofluorescence in tissues demonstrated colocalization of fibrillin and fibulin-2 in skin, perichondrium, elastic intima of blood vessels, and kidney glomerulus. Fibulin-2 was not present in ocular ciliary zonules, tendon, and the connective tissue around kidney tubules and lung alveoli, which all contain fibrillin. Immunogold labeling of fibulin-2 on microfibrils in skin was found preferentially at the interface between microfibrils and the amorphous elastin core, suggesting that in vivo the interaction between fibrillin-1 and fibulin-2 is regulated by cellular expression and deposition as well as by protein-protein interactions.

Amino Acid Sequence↗

[Matrix vesicles in various "elastic" organs].

Matrix vesicles (MV) have been found during normal development in the rabbit and rat aorta and pulmonary trunk as well as in the elastic cartilage of the rat external ear. In spite of the morphological variability of MV, two basic types can be ruled out: MV with dense osmiophilic content, and light or electron-translucent MV. MV of the first type are considered to be extracellular lysosomes. MV in the vessel wall are predominantly localized in the subendothelium, and the border-zones between media and endothelium or media and adventitia; in elastic cartilage they are localized near the perichondrium. Especially often they can be seen at the place of contact between elastic and other fibers of the intercellular space. They can even penetrate the elastic membranes and plates. The parts of the organs mentioned encounter higher functional demands and are more active during development and growth. The MV appear almost exclusively a short time before birth, and predominantly during the first 20-60 days of postnatal life. Their origin may be due to exocytosis and the detachment of parts (Mikroapokrinie) of endothelial cells, smooth muscle cells, fibrocytes and chondrocytes. The MV are considered to be a substrate for subsidiary extracellular mechanisms of adaptation during the normal ontogenesis of the elastic tissue of these organs.

Animals↗

Constitutive equations for fibrous connective tissues.

A general multiaxial theory for the constitutive relations in fibrous connective tissues is developed on the basis of microstructural and thermodynamic considerations. It is compatible with existing general material theories. In elastic tissues, the theory considers the strain-energy function to be the sum of strain-energies of the tissue's components. The stresses are derived from this strain-energy function. Viscoelastic constitutive relations are obtained in an analogous manner. Few examples are developed in detail. The results of the present strain-energy based theory are identical with those of the author's previous structural models (Lanir, 1979a, b) which are based on detailed equilibrium analysis. It turns out, however, that the analytical work involved in solving boundary value problems is considerably shorter if the present theory is used. The advantages of structural theories in avoiding ambiguity in material characterization and in offering an insight into the function, structure and mechanics of tissue components are discussed.

Animals↗

Detection of elastin in the human fetal membranes: proposed molecular basis for elasticity.

The human fetal membranes provide a sterile biomechanical container which adjust by growth to mid-pregnancy to the increase in fetal size, and by elasticity to the forceful movements of the fetus. The molecular basis for this elasticity is not known, yet reduced elasticity may lead to their premature rupture and preterm birth, a major problem in perinatal medicine. Classically, elastin confers the property of elastic recoil to elastic fibres which are assembled from a family of tropoelastin precursors. These are covalently cross-linked to form insoluble elastin by formation of desmosine and isodesmosine, catalysed by the enzyme lysyl oxidase. The amnion, chorion and decidua were shown by Northern analysis and RT-PCR to contain detectable levels of tropoelastin mRNA and the mRNA encoding lysyl oxidase. The proteins encoded by these mRNAs were also identified by Western blotting and immunolocalization. Further, insoluble elastin was extracted from the human fetal membranes and shown by comparison to elastin preparations from other elastic tissues to have a reasonable desmosine content. Finally, scanning electron microscopy confirmed the presence of multiple layers of an apparently very thin elastic system in this tissue. This biochemical and histopathologic study has demonstrated therefore that the human fetal membranes synthesize and deposit a novel elastic fibre. The presence of such an elastic system in these tissues provides, for the first time, a probable molecular basis for the elastic properties of this tissue.

Amino Acids↗

Improved iron-hematoxylin stain for elastic fibers.

A modification of Verhoeff's elastic tissue stain with connective tissue counterstaining is described. The modified procedure requires no differentiation of the elastic fibers, thus eliminating the problem of over- or understaining of elastic fibers. The procedure is easy to perform and yields consistently good results with sharply defined elastic fibers that are easily distinguishable from other connective tissue elements. It is recommended for routine use, particularly when photomicrography is desired.

Animals↗

[Gronblad-Strandberg syndrome. Report of four cases in one family].

The Grönblad-Strandberg syndrome is a rare congenital hereditary dysplasic disorder of the connective tissue, characterized by a progressive abnormal mineralization and dystrophic calcification of elastic tissue and collagen. This process affects tissues rich in elastic fibers and multiple systems of the organism, the cutaneous, ocular and vascular being the most common. These findings progress through the life of the affected person. We present 4 cases in the same family, with heterogeneous clinic pattern and evolution.

Adult↗

[Gronblad-Strandberg syndrome].

The Grönblad-Strandberg syndrome, also known as pseudoxanthoma elasticum, is a rare congenital dysplasic disorder of the connective tissue, characterized by a progressive abnormal mineralization and dystrophic calcification of elastic tissues and collagen (1). The mode of inheritance is uncertain, but autosomal recessive inheritance can be assumed. This process affects tissues rich in elastic fibres and multiple systems of the organism, being the most common the cutaneous (pseudoxanthoma elasticum), ocular (angioid streaks), vascular (occlusive vascular disease) and gastrointestinal manifestations. These findings progress through the life of the affected person, with variable pattern clinic and evolution.

Humans↗

Mitral valve prolapse in sickle cell disease: manifestation of a generalized connective tissue disorder.

Previous studies have shown an association of sickle cell disease with generalized connective tissue disorders such as pseudoxanthoma elasticum. We recently documented an unexpectedly high prevalence of mitral valve prolapse, a connective tissue disorder, in sickle cell disease. To investigate this association, skin biopsies were analyzed from 32 sickle cell disease patients, 11 of whom had mitral prolapse. Total and type III collagen, collagen solubility, and uronic acid were not different between the patients with or without mitral prolapse (p greater than 0.05). Computerized morphometric quantitation of the volume fraction of elastic fibers was greater in sickle cell disease patients than in 10 normals (3.1 +/- 0.1 mean +/- SEM vs 2.0 +/- 0.3%; p less than 0.01) but less than in three patients with pseudoxanthoma elasticum (9.7 +/- 0.6%; p less than 0.001). Desmosine radioimmunoassay (an index of elastic fibers) was greater in sickle cell disease patients with mitral prolapse than those without (239.3 +/- 9.3 vs 171.7 +/- 25.4 ng/mg wet weight; p less than 0.02). Histopathologic grading showed a similar trend (p = 0.07). The combined probabilities of these three independent tests of elastic fiber quantity showed an increased elastic fiber concentration in mitral prolapse patients compared to those without mitral prolapse (p less than 0.02). Thus, there is no evidence for a specific collagen defect; rather, sickle cell disease appears to be associated with a spectrum of elastic tissue disorders, a feature that could predispose to mitral valve prolapse.

Adolescent↗