Acroqueratoelastoidosis de Costa / Acrokeratoelastoidosis of Costa

La acroqueratoelastoidosis de Costa es una genodermatosisde herencia autosónimica dominante con penetrancia incompleta. Es un trastorno de las fibras elásticas exclusivamente cutáneo y a nivel acral. Se caracteriza por la presencia de pápulas eritematosas, amarillentas o del color de la piel normal en la cara marginal de los dedos de las manos o de los pies, que se agrupan, forman placas con aspecto de empedrado y se extienden de forma simétrica al dorso, las palmas y las plantas. El hallazgo histológico patognomónico es la elastorrexis. Se presenta el caso de un niño de 7 años con una acroqueratoelastoidosis que comprometía las manos y los pies, asociada a prurito.

Acrokeratoelastoidosis of Costa is a genodermatosis of autosomal dominant inheritance with incomplete penetrance.It is a cutaneous disorder of the elastic fibers at the acral level.The clinical feature is the presence of erythematous, yellowish or normal skin-colored papules on the marginal aspect of the fingers and/or toes, which agminate to form cobblestone-like plaques and extend symmetrically to the dorsum and palms and soles. The pathognomonic histologic finding is elastorrhexis. We present a 7-year-old boy with acrokeratoelastoidosis involving hands and feet, associated with pruritus.

Transmission and scanning electron microscopy of cutis rhomboidalis

Abstract Cutis rhomboidalis nuchae was assessed in a 65-year-old patient. Optical microscopy showed basophilic agglomerations in the reticular dermis with decreased elastic fibers. Trans- mission electron microscopy showed elongated, curved and fragmented structures, and in their interior the presence of electron-dense lumps was reduced and irregular, similar to modified elastic fibers, whereas the collagen fibers had a normal aspect. Scanning electron microscopy showed deposits between the bundles of collagen, resembling pebbles or stones. These findings demonstrate that, at one stage of the disease, the collagen remains normal and the alterations are seen in the elastic tissue.

Histomorphometric comparison of the corpus cavernosum of rats submitted to euthanasia with ketamine and xylazine or isoflurane

ABSTRACT Purpose: To compare the penile histoarchitecture of rats euthanized with isoflurane or with ketamine and xylazine. Methods: Fourteen male rats were divided into two groups: ISO, with animals euthanized with isoflurane; and K+X, with animals euthanized with ketamine (150 mg/kg) associated with xylazine (15 mg/kg). Immediately after the death, the penises were dissected, fixed in a 4% buffered formalin solution, and processed for histomorphometric analysis. The surface densities (Sv) of the corpus cavernosum structures (connective tissue, smooth muscle, sinusoidal space, and elastic fibers) were evaluated using Image J software. The distribution of collagen types I and III was qualitatively assessed. Statistical analyses were performed using the Student's t test for data comparison, considering it statistically significant when p < 0.05. Results: Regarding the Sv of connective tissue, smooth muscle and sinusoidal space, there were no differences between animals in both groups. On the other hand, the animals euthanized with the association of ketamine and xylazine showed the Sv of elastic fibers 24.8% higher in relation to animals euthanized with isoflurane. Conclusions: The euthanasia method affected one of the morphological parameters of the rat penises. The choice of euthanasia method must be standardized to reduce bias and to obtain reliable and reproducible results.

Ultrastructural and morphometric description of the ear skin and cartilage of two South American wild histricognate rodents (Dasyprocta leporina and Galea spixii) / Descrição ultraestrutural e morfométrica da pele e cartilagem auricular de dois roedores histricognatos silvestres da América do Sul (Dasyprocta leporina e Galea spixii)

Skin and cartilage have been the main source for the recovery of somatic cells to be used in conservation strategies in wild mammals. In this sense, an important step for the cryopreservation of these samples is to recognize the properties of the skin and cartilage. Thus, knowing that the skin may differ among species and aiming to contribute to the establishment of cryobanks, the study examined the differences in the ear skin and cartilage of wild rodents from South America, agouti (Dasyprocta leporina) and spix's yellow-toothed cavy (Galea spixii). Ultrastructural and quantitative methods were used to measure skin and cartilage thickness, density of collagen and elastic fibers, cell type number and distribution, and proliferative activity. Although ultrastructural analysis revealed a similar pattern between species, morphometric analysis of the skin and cartilage showed differences between agoutis and cavies regarding thickness of epidermis layers (corneum: 5.3±2.5μm vs. 3.9±0.6μm; intermediate: 16.4±6.2μm vs. 23.4±8.1μm; basal: 9.9±2.1μm vs. 4.8±0.5μm), dermis (183.1±44.0μm vs. 258.2±22.9μm), total skin (211.8±46.0μm vs. 290.3±23.7μm) and perichondrium (27.6±6.1μm vs. 10.5±1.8μm). A greater number of epidermal cells (61.7±15.2 vs. 24.8±7.6) and chondrocytes (32.7±9.0 vs. 27.5±4.7) were observed in agouti, while the cavy presented a greater number of melanocytes (12.6±4.7 vs. 29.9±6.2), keratinocytes (14.7±4.2 vs. 29.8±7.6), and fibroblasts (103.6±24.7 vs. 112.2±11.3). Moreover, a higher percentage of collagen fibers and proliferative activity was observed in the skin of cavies, when compared to the skin of agoutis. Therefore, there are differences between agouti and cavy for ear skin and cartilage, requiring the establishment of species-specific cryopreservation protocols.(AU)

A pele e cartilagem têm sido uma importante fonte de recuperação de células somáticas a serem utilizadas em estratégias de conservação em mamíferos silvestres. Nesse contexto, uma importante etapa para criopreservação é conhecer, inicialmente, as propriedades que compõem a pele e cartilagem. Sabendo, então, que a pele pode diferir-se entre espécies e com o objetivo de contribuir para o estabelecimento de criobancos, o estudo evidenciou as diferenças da pele e da cartilagem do pavilhão auricular apical de cutias (Dasyprocta leporina) e preás (Galea spixii) que são roedores silvestres presentes na América do Sul. Para tanto, métodos ultraestruturais e quantitativos foram utilizados para mensurar a espessura da pele e da cartilagem, densidade de fibras colágenas e elásticas, número e distribuição dos tipos celulares e atividade proliferativa. Embora as propriedades ultraestruturais em cutias e preás tenham se mostrado semelhantes, avaliações acerca da morfometria da pele e da cartilagem demonstrou diferenças, especialmente nas camadas epidérmicas (córnea: 5,3±2,5μm vs. 3,9±0,6μm; espinhosa: 16,4±6,2μm vs. 23,4±8,1μm; basal: 9,9±2,1μm vs. 4,8±0,5μm), derme (183,1±44,0μm vs. 258,2±22,9μm), pele total (211,8±46,0μm vs. 290,3±23,7μm) e pericôndrio (27,6±6,1μm vs. 10,5±1,8μm). Além disso, um número maior de células epidérmicas (61,7±15,2 vs. 24,8±7,6) e condrócitos (32,7±9,0 vs. 27,5±4,7) foram observados em cutias, enquanto em preás um maior número de melanócitos (12,6±4,7 vs. 29,9±6,2), queratinócitos (14,7±4,2 vs. 29,8±7,6) e fibroblastos (103,6±24,7 vs. 112,2±11,3) foram evidenciados. Ainda, em preás, uma maior porcentagem de fibras colágenas e da atividade proliferativa foram observadas quando comparadas a pele de cutias. Portanto, existem diferenças entre cutias e preás para pele e cartilagem do pavilhão auricular, exigindo desta forma um estabelecimento de protocolos de criopreservação específica para cada uma destas espécies.(AU)

Association between single nucleotide polymorphisms of tropoelastin gene and aortic dissection / 中南大学学报(医学版)

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.

Elastosis Perforans Serpiginosa: a D-penicillamine induced dermatoses in a patient with Wilson's disease

Long term use of D-penicillamine for Wilson's disease can be associated with many adverse reactions and systemic side effects. We report the case of a 28-year-old male patient diagnosed with Wilson's disease presenting with a serpiginous raised violaceous skin lesion in the anterior aspect of the neck over the last six months and two small papules with central umbilication during the last month. Histopathological examination of skin lesions demonstrated transepidermal perforating channel, and the Verhoeff's-van Gieson stain showed marked increase number of irregular serrated elastic fibers suggesting the diagnosis of D- penicillamine induced elastosis perforans serpiginosa.

Pseudoxanthoma elasticum-like papillary dermal elastolysis in non-exposed skin

Abstract Pseudoxanthoma elasticum-like papillary dermal elastolysis is an acquired elastic tissue disorder clinically similar to pseudoxanthoma elasticum in the absence of systemic involvement. Histopathologically, special staining of elastic fibers demonstrates a total or partial band-like loss of elastic fibers in the papillary dermis. Although ultraviolet radiation seems to be one of the main etiological factors in this entity, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck of a woman who wore hijab.

Multiple whitish papules on the posterior neck of an elderly woman

Abstract White fibrous papulosis of the neck is a rare entity, with fewer than 50 cases described. It is a benign pathology whose main interest lies in its broad differential diagnosis, especially with pseudoxanthoma elasticum. The authors report the case of a 77-year-old woman with multiple yellow-white monomorphic papules on the posterior cervical region, with years of evolution. Cutaneous biopsy revealed a nodular area in the superficial and middle reticular dermis, with slight thickening of the collagen fibers and focally enlarged elastic fibers, aspects highlighted in the Verhoeff staining that additionally showed absence of elastic fibers in the papillary dermis.

Age-dependent histomorphometric evolution of the corpus spongiosum

Abstract Purpose: To quantify the age-dependent changes in the relative volume of elastic fibers, collagen fibers and the proportion of collagen types I/III in the corpus spongiosum of rats. Methods: Forty-eight rats, raised under similar conditions, were divided into four groups (G1 to G4) and underwent penectomy at the ages of 6, 9, 12 and 24 months, respectively. Histological sections from the middle segment of the penis were stained with Weigert's resorcin-fuchsin, Masson's trichrome and Picrosirius red, the volumetric density of elastic fibers, collagen fibers and the proportion of collagen types I and III in the corpus spongiosum were determined by stereological analysis. Results: A reduction in the proportion of collagen I/III between the groups G3 and G4 (p < 0.048) was observed. In the volumetric analysis of elastic fiber, we observed a significant rise between the groups G2 and G3 (p < 0.03) and a reduction of the volume between the groups G3 and G4 (p < 0.01). However, there was no difference in the quantity of total collagen between the groups (p > 0.54). Conclusions: Aging in rats did not change the quantity of total collagen but reduced the proportion of collagen types I/III and the volume of elastic fibers.

Expression of stromal elements of prostatic adenocarcinoma in different gleason scores

Abstract Purpose: To quantify and compare the expression of stromal elements in prostate adenocarcinoma of different Gleason scores with non-tumor area (control). Methods: We obtained 132 specimens from samples of prostate peripheral and transition zone. We analyzed the following elements of the extracellular matrix: collagen fibers, elastic system, smooth muscle fibers and blood vessels. The tumor area and non-tumor area (control) of the TMA (tissue microarray) were photographed and analyzed using the ImageJ software. Results: The comparison between the tumor area and the non-tumor area showed significant differences between stromal prostate elements. There was an increase of collagen fibers in the tumor area, mainly in Gleason 7. Elastic system fibers showed similar result, also from the Gleason 7. Blood vessels showed a significant increase occurred in all analyzed groups. The muscle fibers exhibited a different behavior, with a decrease in relation to the tumor area. Conclusions: There is a significant difference between the extracellular matrix in prostate cancer compared to the non-tumor area (control) especially in Gleason 7. Important modifications of the prostatic stromal elements strongly correlate with different Gleason scores and can contribute to predict the pathological staging of prostate cancer.

Stereological analysis of elastic fibers of the corpus cavernosum of rats during the aging process

Abstract Purpose To evaluate changes in the quantity of elastic fibers in the corpora cavernosa of rats during the natural aging process, and to assess the degree of this change by determining volumetric density (Vv) at different ages via stereological analysis. Methods Forty-eight rats, raised under similar conditions, were subjected to the natural aging process and divided into four groups (G1 to G4), according to age at the time of penectomy (6, 9, 12, and 24 months, respectively). Histological sections of the middle segment of the penis were stained with Weigert's resorcin-fuchsin, and the volumetric density (Vv) of elastic fibers of the corpora cavernosa were determined via stereological analysis. Results There were no statistically significant differences in Vv among groups G1, G2, and G3. These three groups were therefore considered as a single group. The mean Vv of this group showed a statistically significant reduction compared to that of G4 (0.16 vs. 0.11, p<0.05). Conclusion Natural aging in rats was responsible for a reduction in volumetric density of elastic fibers of the corpora cavernosa (approximately 30% decrease in Vv) during senescence.

Effects of tadalafil to prevent injury on corpus cavernosum after vascular or nervous peri-prostatic bundle injury. Experimental model in rats

Abstract Purpose: To evaluate the effects of tadalafil (TD) in preventing histological alterations of the corpus cavernosum caused by isolated lesions of cavernous nerve (ILCN) and artery (ILCA) in rats. Methods: Fifty male Wistar rats were randomly assigned in five groups: G1: control; G2: bilateral ILCN; G3: bilateral ILCA; G4: ILCN+TD; G5: ILCA+TD. The cavernous bodies were submitted to histomorphometry, immunohistochemistry and biochemical analysis. Results: Nerve density was significantly higher in G2 and G4 compared to control (22.62±2.84 and 19.53±3.47 vs. 15.72±1.82; respectively, p<0.05). Smooth muscle density was significantly lower in G2 and G3 in comparison to G1 (12.87±1.90 and 18.93±1.51 vs. 21.78±1.81, respectively; p<0.05). A significant decrease in the sinusoidal lumen area was observed in G2 compared to controls (5.01±1.62 vs. 9.88±3.66, respectively; p<0.05) and the blood vessel density was increased in G2 and G3 (29.32±4.13 e 20.80±2.47 vs. 10.13±2.71, p<0.05). Collagen density was higher in G3 compared to G1 (93.76±15.81 vs. 64.59±19.25; p<0.05). Conclusions: Histomorphometric alterations caused by ILCN were more intense than those produced by vascular injury, but the collagen analyses showed more fibrosis in animals with ILCA. TD was effective in preventing the majority of the alterations induced by the periprostatic bundle injury.

Fibroelastolytic Papulosis of the Chest / 대한피부과학회지

Fibroelastolytic papulosis is an acquired elastic tissue disorder that includes the clinicopathological features of white fibrous papulosis of the neck (WFPN) and pseudoxanthoma elasticum-like papillary dermal elastosis (PXE-PDE). It is clinically characterized by discrete white papules occurring on the neck in middle-aged women and is histopathologically characterized by elastolysis and/or fibrosis in the upper dermis. An 80-year-old woman presented with multiple white papules on her anterior chest, which occurred 6 months prior to the presentation. Histopathological examination of a biopsy specimen revealed some degree of fibrosis and elastolysis in the upper dermis. Thus, the final diagnosis in this case was compatible with fibroelastolytic papulosis considering that this patient showed clinicopathological features of both WFPN and PXE-PDE. Notably, this is the first case report that describes fibroelastolytic papulosis confined exclusively to the anterior chest.

Morphological analysis of the elastic and collagen fibers in the ram penis / Análise morfológica de fibras elásticas e colágenas no pênis de ovinos

The penis represents the organ of the male's copulation. It is essential to know the reproductive biology and the morphology of the reproductive organs to increase animal production. In order to contribute to this knowledge and provides information on the ram reproductive morphology, the purpose of this work was to describe the distribution, based on light microscopy, of the collagen and elastic fibers in the ram penis. For that, were collected transverse fragments of the penis (root, sigmoid flexure, body and glans) of seven rams. The specimens were fixed in paraformaldehyde for 24h and destined for the histological routine. The extracellular matrix of the ram penis was composed of collagen and elastic fibers. The penis was enveloped by the tunica albuginea, consisting essentially of collagen fibers, which were arranged in two layers: an outer longitudinal and an inner circular. This tunic emitted septa that penetrated the corpus cavernosum. The elastic fibers appeared transversely and longitudinally in the corpus cavernosum, corpus spongiosum, and next to the neurovascular bundle of the penis. This structure was not different to that reported for other domestic ruminants such as cattle and buffaloes.(AU)

O pênis representa o órgão da cópula do macho. É fundamental que se conheça a biologia reprodutiva e a morfologia dos órgãos reprodutores para o incremento da produção animal. Com o objetivo de contribuir para este conhecimento e fornecer informações sobre a morfologia reprodutiva de ovinos, o propósito deste trabalho foi descrever a distribuição, com base na microscopia de luz, das fibras colágenas e elásticas no pênis de ovinos. Para tanto, foram coletados fragmentos transversais do pênis (raiz, flexura sigmoide, corpo e glande) de sete ovinos. Os exemplares foram fixados em paraformaldeído por 24h e destinados à rotina histológica. A matriz extracelular do pênis de ovinos estava constituída por fibras colágenas e elásticas. O pênis estava envolvido pela túnica albugínea, formada essencialmente por fibras colágenas, que estavam arranjadas em duas camadas: longitudinal externa e circular interna. Esta túnica emitiu septos que penetraram no corpo cavernoso. As fibras elásticas apareceram de modo transversal e longitudinal nos corpos cavernoso e esponjoso e junto ao feixe vásculo-nervoso do pênis de ovinos. Esta estrutura mostrou-se similar ao encontrado em outros ruminantes domésticos como bovinos e bubalinos.(AU)

Papular elastorrhexis: a rare and benign disease

Abstract: Papular elastorrhexis is an acquired disease of elastic tissue; considered rare, its etiology and pathogenesis remain unknown. The vast majority of cases occur in women in the first or second decade of life. The disease manifests as multiple uniformly-sized, circumscribed, hypochromic and achromic papules located predominantly on the trunk and upper extremities. The lesions are generally asymptomatic and have a stable evolution over years. Its diagnosis is based on clinical and histopathological findings. The benignity of papular elastorrhexis and the subtlety of its clinical changes make the expectant treatment perfectly viable. The authors present one case in a young female patient.

Acquired elastotic hemangioma, a little-known entity: report of a case with 15 years of evolution

Abstract: Acquired elastotic hemangioma is a hemangioma variant first described in 2002. It is characterized by being a benign, solitary, slow-growing lesion, that appears in adulthood and is associated with solar exposure. It is a rare hemangioma variant with few cases reported in the literature. We present a case of acquired elastotic hemangioma on the back of the right hand and forearm in a male adult. Acquired elastotic hemangioma is a benign vascular proliferation associated with solar exposure, usually assymptomatic. It affects adults between 59 and 65 years of age. Histopatologically it is characterized by proliferation of small vessels in the upper dermis that are disposed parallel to the epidermis, and significant solar elastosis. The treatment is surgical, with no relapses reported.

Anetodermia secundaria a sífilis / Anetoderma secondary to syphilis. Case report and literature review

La anetodermia es una entidad cutánea benigna, rara e infrecuente, cuya característica es la pérdida localizada de fibras elásticas a nivel de la dermis. Suele observarse en pacientes con síndrome antifosfolípidico, lupus eritematoso sistémico, acné y varicela y de manera inusual como manifestación de una sífilis secundaria. Comunicamos el caso de una mujer con secundario o sifilítico que desarrolló anetodermia posterior a este proceso infeccioso.

Anetoderma is a benign, rare and infrequent cutaneous entity whose characteristic is the localized loss of elastic fibers at the level of the dermis. It is usually observed in patients with antiphospholipid syndrome, systemic lupus erythematosus, acne and varicella and an unusual way as a manifestation of secondary syphilis. We report a case of a woman with syphilitic secondary disease who developed an anetoderma after this infectious process.

Histomorphological changes in the common carotid artery of the male rat in induced hypogonadism / 대한해부학회지

The role of androgens in the development of cardiovascular diseases remains controversial. The current study therefore sought to determine the changes in the histomorphology of the common carotid artery of the male rat in orchidectomy-induced hypogonadism. Twenty-two Rattus norvegicus male rats aged 2 months were used. The rats were randomly assigned into baseline (n=4), experimental (n=9), and control (n=9) groups. Hypogonadism was surgically induced in the experimental group by bilateral orchiectomy under local anesthesia. At experiment weeks 3, 6, and 9, three rats from each group (experimental and control) were euthanized, their common carotid artery harvested, and routine processing was done for paraffin embedding, sectioning, and staining. The photomicrographs were taken using a digital photomicroscope for morphometric analysis. Orchidectomy resulted in the development of vascular fibrosis, with a significant increase in collagen fiber density and decrease in smooth muscle and elastic fiber density. Moreover, there was development of intimal hyperplasia, with fragmentation of medial elastic lamellae in the common carotid artery of the castrated rats. Orchidectomy induces adverse changes in structure of the common carotid artery of the male rat. These changes may impair vascular function, therefore constituting a possible structural basis for the higher incidences of cardiovascular diseases observed in hypogonadism.

A Case of Nodular Colloid Degeneration in a 40-year-old Woman / 대한피부과학회지

Colloid degeneration of the skin is a rare, distinct clinicopathological entity characterized by the deposition of colloid material in the dermis. Nodular colloid degeneration (NCD) is a clinical variant of colloid degeneration of the skin. Herein, we report the case of a 40-year-old woman who presented with a 3-year history of multiple asymptomatic yellowish-brown, indurated papules and plaques on her forehead. Histopathologic examination of the lesion revealed deposition of amorphous pale pink homogenous colloid material with irregular clefts, fissures, and scattered pyknotic nuclei in the upper dermis. The material was negative for Congo red staining, and periodic acid-Schiff and Masson's trichrome staining revealed positive reactivity. Scattered fragmented elastic fibers were present in the colloid material on Verhoeff—Van Gieson staining. The patient was diagnosed with NCD. We propose that NCD should be considered in the differential diagnosis of long-standing facial nodules and plaques.