Fibroelastolytic papulosis of the neck (FEPN) encompasses a spectrum of two disorders that were previously reported as pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN). The clinical presentation of FEPN is asymptomatic to mildly pruritic whitish-yellow papules that may coalesce into cobblestone patterned plaques that resemble pseudoxanthoma elasticum (PXE). The histology is characterized by a decrease or loss of elastic fibers in the papillary dermis and this is sometimes accompanied by a minimal or nodular increase of dermal collagen fibers. We report here on a 28-year-old Korean man with asymptomatic, multiple, skin-colored to slightly yellowish, match-head sized, cobblestone-patterned papules on the neck, and these were histologically consistent with FEPN and the papules showed slightly increased dermal collagen associated with decreased and fragmented elastic fibers, elastin and tropoelastin. The pathogenesis of FEPN in this case might have been related with mild dermal inflammation, followed by fragmentation, elastolysis and increased dermal collagen.
Adult ; Collagen ; Dermis ; Elastic Tissue ; Elastin ; Humans ; Inflammation ; Neck ; Pseudoxanthoma Elasticum ; Tropoelastin

OBJECTIVES: To evaluate the relation between single nucleotide polymorphisms (SNPs) of tropoelastin gene and aortic dissection (AD) via identifying SNPs in the tropoelastin gene, and to detect the level of tropoelastin mRNA, elastin and elastic fibers. METHODS: The specimens of the AD group ( RESULTS: Seven SNP loci of the tropoelastin gene were detected in these samples. Among them, 5 SNP loci were polymorphic. The frequency of 3 SNP loci[rs2071307 (G/A), rs34945509 (C/T) and rs17855988 (G/C)] was significantly different between the AD group and the control group (all CONCLUSIONS The polymorphisms of rs2071307 (G/A), rs34945509 (C/T), and rs17855988(G/C) in the tropoelastin gene may eventually affect the synthesis of elastic fibers and they may play an important role in the occurrence of AD.
Aneurysm, Dissecting/genetics* ; Elastic Tissue ; Elastin/genetics* ; Humans ; Polymorphism, Single Nucleotide ; Tropoelastin/genetics*

BACKGROUNDThe pathological characteristics of abdominal aortic aneurysm (AAA) involved the regression of extracellular matrix (ECM) in aortic walls, especially elastic structure in medial layer. As the major structural protein of aorta, elastin contributes to the extensibility and elastic recoil of the vessels. We hypothesized that overexpression of elastin in vessel walls might regenerate the elastic structure of ECM, restore the elastic structure of the aneurysmal wall, and eventually lead to a reduction of aortic diameters (ADs) in an experimental model of AAA.

METHODSTropoelastin (TE) of Sprague Dawley (SD) rat was synthesized by reverse transcription polymerase chain reaction and used to construct adneviral vectors containing elastin precursor protein (AdTE-GFP). Cultured vascular smooth muscle cells (VSMCs) from aortas of male SD rats were transfected with AdTE-GFP, AdGFP, adenoviral vector (AdNull), and phosphate buffered saline (PBS). Immunofluorescence staining was performed to determine the expression of elastin in transfected cells. The expression of elastic fibers in ECM of VSMCs transfected with AdTE-GFP were detected by fluorescence microscopy and transmission electron microscopy (TEM) at 1, 3, and 5 days following gene transfer. The AAA vessel walls were infused with AdTE-GFP or an empty AdNull, or PBS directly into the aneurysmal lumen. ADs of the aneurysms were compared in infused aortas. Formation of new elastic fibers in vivo was assessed by hematoxylin and eosin, and elastic von-Giesson staining. Recombinant elastin-GFP in vivo was identified by immunohistochemical staining.

RESULTSElastic fibers were increased both in ECM of VSMC and in vessel walls after gene transfer. Histological studies revealed that the AdTE-GFP-transduced aortas had elastic fiber regeneration in the aneurysmal walls. The AdTE-GFP-transduced aortas showed a decreased AD (23.04% ± 14.49%, P < 0.01) in AAA vessel walls.

CONCLUSIONSElastic fibers have been successfully overexpressed both in vitro and in a rat model of AAA by a technique of gene transfer. The overexpression of elastic fibers within the aneurysmal tissue appeared to reverse the aneurysm dilatation in this model.

Animals ; Aortic Aneurysm, Abdominal ; metabolism ; therapy ; Elastic Tissue ; metabolism ; Elastin ; genetics ; metabolism ; Fluorescent Antibody Technique ; Male ; Muscle, Smooth, Vascular ; metabolism ; Rats ; Rats, Sprague-Dawley ; Tropoelastin ; genetics ; metabolism

A 63 year-old woman visited our hospital with a palpable chest wall mass in the infrascapular region. We performed excision of the mass. The mass was histolocally diagnosed as elastofibroma. Elastofibroma is characterized by the proliferation of fibrous tissue with elastin. It is a relatively slow growing benign soft tissue tumor and it is most often found in the infrascapular region. We have experienced a cases of this rare disease and we report on it together with a review of the relevant literature.
Elastin ; Female ; Humans ; Rare Diseases ; Thoracic Wall ; Thorax

A 71-year-old woman visited our hospital with the chief complain of a mass in her infrascapular region. We performed tumor excision and we diagnosed it as elastofibroma dorsi. Elastofibromas are benign soft tissue tumors that mostly arise in the infrascapular lesion; it is a slowly growing lesion that's characterized by the proliferation of fibrous tissue with elastin. Its incidence is very low and its pathogenesis remains unclear. We report here on this case, and we include a review of the relevant literature.
Aged ; Elastin ; Female ; Fibroma ; Humans ; Incidence ; Thoracic Wall ; Thorax

Many factors influence the elastin-like polypeptides (ELPs) self-assembled into micron-sized particles. However, few efforts were made to investigate these factors. Using the ELPs [KV8F]n as the target, we studied systematically the factors with the dynamic light scattering. Our results show that the particle size increased and the uniform of particles decreased with the increase of the molecular weight. The analysis of size variation in self-assembled ELPs in response to changes in salt concentration indicated that the size increased with increasing the salt concentration, and the opposite response was observed when the concentration was above 0.4 mol/L. Under these conditions, the particles are micron-sized and larger than 1.1 μm. However, when the fusions containing the same ELPs and xylanase or 1,3-propanediol oxidoreductase, the size of the self-assembled ELPs particles decreased dramatically, which was only about 1/10 of that of the free ELPs. We proposed that the solvent accessible charged area of the enzymes could interact with the ELPs, the sterical hindrance of the enzymes prevent the aggregation of the ELPs. This might be the most important parameter in altering the particle size sharply.
Elastin ; chemistry ; Molecular Weight ; Particle Size ; Peptides ; chemistry ; Salts ; chemistry

Elastofibroma dorsi, a rare, noncapsulated benign entity is characterized by the proliferation of fibrous tissue with elastin and occurs most often in the infrascapular area of elderly women. It is a relatively slowly growing lesion and no reports of malignant transformation exist. Which is overlooked easily because it rarely causes symptoms such as tenderness, pain, or restriction of movement. The diagnosis of elastofibroma is established by typical histopathologic findings. Radiographic evaluation may lead to a presumptive diagnosis. We experienced a case of elastofibroma dorsi in a 48-year-old woman and report this case with a review of the literature.
Aged ; Diagnosis ; Elastin ; Female ; Fibroma ; Humans ; Middle Aged

Elastin is a natural biomedical material of great potential. Being endowed with the special crosslinking and hydrophobic structure, elastin retains many good properties such as good elasticity, ductibility, biocompatibility, biodegradability and so on. Nowadays, elastin as a material, which is gradually attracting people' s attention in the biomedical materials field, has been used as tissue engineering scaffolds, derma substitutes and other biomedical materials. In this context, a systematic review on the characteristics of elastin as a biomedical material and on the actuality of its application is presented. Future developments of elastin in the field of biomedical applications are also discussed.
Biocompatible Materials ; Elastin ; chemistry ; physiology ; Humans ; Skin, Artificial ; Tissue Scaffolds

Congenital auricular deformities such as lop ear, cup ear, prominent ear, and Stahl's ear can be corrected nonsurgically in the early neonatal period(first week after birth) obtaining satisfactory results. Auricular cartilage consists of chondrocytes and intercellular materials that are mainly assembled from collagen, elastin, and a proteoglycan aggergate. Cartilageelasticity is dependent upon the concentration of the proteoglycan aggregate. Hyaluronic acid, which is a constituent of proteoglycan aggregate and is increased by estrogens, has an important play on disconnecting the proteoglycan aggregate. So the increased estrogen results in the lack of elasticity. The circulating levels of free estradiol are higher during the first 72 hours after birth and decreases thereafter, so the ear is soft and easily malleable in this early neonatal period. In this study, we have applied aluminium splint (90 degree-angulated, 180 degree-angulated, and rolled) on rabbit ear. All of the rabbit was divided into 3 groups containing 3 types of deformities. In group 1, estrogen was applied to the deformed ear with regional injection to the perichondrium of auricular cartilage. In group 2. estrogen was applied topically with ointment. In group 3, as control group, saline was injected to the perichondrium. Four weeks later, in group 2, the shape of the deformed rabbit ear was maintained, and did not return to its original shape even after splint removal. From these results, we concluded that estrogen ointment could be tried as adjunctive therapeutic modalities to the nonsurgical treatment of the congenital auricular deformities.
Chondrocytes ; Collagen ; Congenital Abnormalities ; Ear Cartilage ; Ear* ; Elasticity ; Elastin ; Estradiol ; Estrogens* ; Hyaluronic Acid ; Parturition ; Proteoglycans ; Splints

Penicillamine dermatopathy refers to the characteristic hemorrhagic skin lesions found in persons receiving long-term penicillamine therapy for either Wilson's disease or cystinuria. These lesions are thought to develop as a result of faulty collagen and elastin synthesis. We described a 31-year-old woman with Wilson's disease who developed mild pruritic grouped matchhead-sized cream-colored papules on the dark reddish plaques on both knees and elbows.
Adult ; Collagen ; Cystinuria ; Elastin ; Elbow ; Female ; Hepatolenticular Degeneration ; Humans ; Knee ; Penicillamine* ; Skin