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.

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.

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.

Acquired perforating dermatosis in a patient with chronic renal failure

Abstract Perforating dermatoses are a group of skin diseases characterized by transepidermal elimination of dermal material. The disease is divided into two groups: the primary group and the secondary group. The classical or primary perforating dermatoses are subdivided into four types according to the eliminated dermal materials: Kyrle disease, perforating reactive collagenosis, elastosis perforans serpiginosa, and perforating folliculitis. The secondary form is known as acquired perforating dermatosis. The term was proposed in 1989 by Rapini to designate the perforating dermatoses affecting adult patients with systemic disease, regardless of the dermal materials eliminated. This report describes a case of the disease with elimination of collagen and elastic fibers in a patient with chronic renal failure.

Elastoma: clinical and histopathological aspects of a rare disease

Abstract Elastoma is a connective tissue nevus characterized by changes in elastic fibers. It can be congenital or acquired, and is usually diagnosed before puberty. Associated with osteopoikilosis, it is known as Buschke-Ollendorff syndrome. Histopathology with specific staining for elastic fibers is critical for a diagnostic conclusion. This report describes the case of a 7-year-old male patient with lesions diagnosed as elastoma, with absence of bone changes in the radiological imaging. This study aims to report the clinical presentation and histological examination of such unusual disease.

Sudden death, aortic rupture in horses, literature review, case studies reported and risk factors / Morte súbita por ruptura da aorta em cavalos, literatura, estudos de casos relatados e fatores de risco

Sudden deaths of horses in multiple equestrian disciplines have been attributed to acute and chronic respiratory and cardiovascular diseases. The aim of this study was to perform a review of aortic rupture in horses analyzing, case studies and assessing risk factors. The literature has reported a total of 137 cases of aortic rupture in horses for 28 years (1986-2014), with approximately five horses dying of aortic rupture per year. Histopathologically, there are observed discrete macroscopic degenerative changes in the intima layer only in the aorta. The histological evaluation in the beginning portion of the aorta of the heart evidenced degenerative changes with loss of continuity and distribution of elastic fibers. Risk factors for the rupture of the aorta are: spontaneous rupture associated with hypertension, preexisting vascular injury (aneurysm), dilated or hypertrophic cardiomyopathy, copper levels in the endothelium, genetic factors such as inbreeding, toxicology or pharmacological factors. Aortic rupture shows similarity with pulmonary hemorrhage induced by exercise especially under the locomotors induced trauma theory of exercise that can induce pulmonary hemorrhage. In conclusion, degenerative changes to discrete elastic fiber of the intima of the aorta in the emergence of the heart seem to predispose the aorta wall rupture at the time of maximum blood pressure during exercise and the consequent collapse and athletic horses death...

As mortes súbitas de cavalos em várias provas equestres têm sido atribuídas a doenças respiratórias e cardiovasculares agudas e crônicas. O objetivo deste estudo foi efetuar uma revisão de literatura da ruptura da aorta em cavalos analisando estudos de caso e estabelecendo os possíveis fatores de risco. Na revisão da literatura no período de 28 anos (1986-2014) foram localizados 137 casos de ruptura da aorta em cavalos com aproximadamente cinco cavalos morrendo por essa causa por ano. Histologicamente, são observadas alterações macroscópicas discretas degenerativas na camada íntima da aorta. A avaliação histológica na porção inicial da aorta do coração evidencia alterações degenerativas com perda de continuidade e distribuição das fibras elásticas. Fatores de risco para a ruptura da aorta dos cavalos são: ruptura espontânea associada com hipertensão, lesão vascular pré-existente (aneurisma), cardiomiopatia dilatada ou hipertrófica, níveis de cobre no endotélio, fatores genéticos, tais como a consanguinidade na criação, toxicologia e aspectos farmacológicos. A ruptura aórtica mostra semelhança com hemorragia pulmonar induzida pelo exercício. Em conclusão, alterações degenerativas discretas das fibras elásticas da íntima da aorta parecem predispor a ruptura da parede da aorta, no momento da pressão máxima de sangue durante o exercício determinando o consequente colapso e morte do cavalo atleta...

Nova contribuição ao estudo do remodelamento ventricular e dos anéis valvares na cardiomiopatia dilatada: avaliação anátomo-histopatológica / New contribution to the study of ventricular remodeling and valve rings in dilated cardiomyopathy: anatomical and histological evaluation

Introdução: A cardiomiopatia dilatada idiopática (CMDId) é causadora de grande impacto, porém aspectos de sua fisiopatologia são desconhecidos. Objetivo: Avaliar aspectos anatomo-histológicos de corações com CMDId comparando-os a corações normais, com medidas perimetrais dos anéis atrioventriculares direito (AVD) e esquerdo (AVE) e dos ventrículos direito (VD) e esquerdo (VE) e a porcentagem de fibras colágenas e elásticas dos anéis. Métodos: Foram avaliados 13 corações de cadáveres portadores de CMDId e 13 corações normais, que foram dissecados mantendo-se os anéis atrioventriculares e a massa ventricular, com laminação em segmentos correspondentes a 20%, 50% e 80% da distância entre o sulco atrioventricular e o ápice ventricular. Os cortes foram submetidos à digitalização fotográfica, sendo comparadas as medidas. Os anéis foram dissecados, medidos e enviados ao laboratório de anatomia patológica, sendo realizadas colorações por meio de hematoxilina-eosina, picrossírius e resorcina fuccina oxidada. Resultados: Com relação aos ventrículos, no grupo CMDId ocorre dilatação nos segmentos apical, equatorial e basal. A medida do AVD foi maior no grupo CMDId, não havendo diferença no AVE entre os grupos. Com relação ao percentual de fibras colágenas, há diminuição no grupo CMDId em relação ao grupo normal. Com relação às fibras elásticas, não houve diferença entre os grupos. Conclusão: Ocorre alteração da geometria ventricular com dilatação no grupo CMDId. Na CMDId observou-se aumento no perímetro do AVD. Não se observou aumento do perímetro do AVE. Houve diminuição percentual na área total de colágeno tanto no AVD quanto no AVE em corações com CMDId. .

Introduction: Idiopathic dilated cardiomyopathy causes great impact but many aspects of its pathophysiology remain unknown. Objective: To evaluate anatomical and histological aspects of hearts with idiopathic dilated cardiomyopathy and compare them to a control group, evaluating the behavior of the perimeters of the atrioventricular rings and ventricles and to compare the percentage of collagen and elastic fibers of the atrioventricular rings. Methods: Thirteen hearts with cardiomyopathy and 13 normal hearts were analysed. They were dissected keeping the ventricular mass and atrioventricular rings, with lamination of segments 20%, 50% and 80% of the distance between the atrioventricular groove and the ventricular apex. The sections were subjected to photo scanning, with measurement of perimeters. The atrioventricular rings were dissected and measured digitally to evaluate their perimeters, later being sent to the pathology laboratory, and stained by hematoxylin-eosin, picrosirius and oxidized resorcin fuccin. Results: Regarding to ventricles, dilation occurs in all segments in the pathological group, and the right atrioventricular ring measurement was higher in idiopathic dilated cardiomyopathy group, with no difference in the left side. With respect to collagen, both sides had lower percentage of fibers in the pathological group. With respect to the elastic fibers, there was no difference between the groups. Conclusion: There is a change in ventricular geometry in cardiomyopathy group. The left atrioventricular ring does not dilate, in spite of the fact that in both ventricles there is lowering of collagen. .

Effects of prepubertal corticosterone treatment on urinary bladder

PURPOSE: The aim of this work was to analyze the bladder wall modifications after a chronic treatment with high doses of corticosterone in prepubertal rats. METHODS: This study included 26 male rats assigned into four groups: T30 was treated with corticosterone until 29 days of age and killed at day 30, while T65 group received the same treatment but was killed at day 65. Each group had its own control group (C30 and C65). For treated animals, daily intraperitoneal injections of corticosterone (20 mg/Kg) were administered between 7th and 29th day of life. Bladders were removed and collagen, smooth muscle, elastic fibers system, vascular density and epithelium were analyzed by morphometrical methods, immunofluorescence, and biochemistry. RESULTS: Vascular density in lamina propria was reduced by 40% (p<0.05) in group T65. Collagen organization was altered in T30 and T65, although total collagen concentration was unchanged. The T65 group had an increase in elastic system fibers. There was no difference in epithelial height and cell density between the groups. Concerning the smooth muscle fibers density we observed a 19% increase (p<0.05) in the T65 group. CONCLUSION: Prepubertal administration of corticosterone induces structural modifications in the bladder of rats in a medium term analysis. .

Structural analysis of the phimotic prepuce in patients with failed topical treatment compared with untreated phimosis

To evaluate histological alterations in prepuce of patients with phimosis submitted to topic treatment with betamethasone in association with hyaluronidase.

We studied sixty patients (mean age 4.5), presenting true phimosis and treated with a topical treatment with betamethasone cream (0.2%) + hyaluronidase. The parents of seven of these patients opted for circumcision (control group). The other fifty-three patients were submitted to clinical treatment. The samples were stained with Weigert's resorcin-fuchsin (analysis of the elastic fibers) and Picro-Sirius Red, for analysis of the collagen. The volumetric density of the elastic fibers was determined by stereological methods.

Only eight (15%) of the fifty-three patients submitted to topical treatment presented failure, being indicated for circumcision (histological analysis). We observed an increase of the collagen type III of the patients submitted to topical treatment. The quantification showed a reduction of the volumetric density of the prepuce's elastic fibers of the patients submitted to the cream treatment, when compared to the control group (p = 0.056). The volumetric density of the elastic fibers of the prepuce at the group not submitted to topical treatment showed an average of 14.60% (11.06 to 21.64%); in the group submitted to the cream treatment, the volumetric density of the elastic fibers of the prepuce showed an average of 10.34% (3.45 to 17.9%).

The topical treatment of phimosis with betamethasone 0.2% + hyaluronidase had a success rate of 85%. Patients with failure of the topical treatment with steroid had histological alterations in the prepuce. .

Structural alterations of foreskin caused by chronic smoking may explain high levels of urethral reconstruction failure using foreskin flaps

OBJECTIVES: The aim of the present study was to perform a stereological and biochemical analysis of the foreskin of smoker subjects. MATERIALS AND METHODS: Foreskin samples were obtained from 20 young adults (mean = 27.2 years old) submitted to circumcision. Of the patients analyzed, one group (n = 10) had previous history of chronic smoking (a half pack to 3 packs per day for 3 to 13 years (mean = 5.8 ± 3.2). The control group included 10 nonsmoking patients. Masson's trichrome stain was used to quantify the foreskin vascular density. Weigert’s resorcin-fucsin stain was used to assess the elastic system fibers and Picrosirius red stain was applied to study the collagen. Stereological analysis was performed using the Image J software to determine the volumetric densities. For biochemical analysis, the total collagen was determined as µg of hydroxyproline per mg of dry tissue. Means were compared using the unpaired t-test (p < 0.05). RESULTS: Elastic system fibers of smokers was 42.5% higher than in the control group (p = 0.002). In contrast, smooth muscle fibers (p = 0.42) and vascular density (p = 0.16) did not show any significant variation. Qualitative analysis using Picrosirius red stain with polarized light evidenced the presence of type I and III collagen in the foreskin tissue, without significant difference between the groups. Total collagen concentration also did not differ significantly between smokers and non-smokers (73.1µg/mg ± 8.0 vs. 69.2µg/mg ± 5.9, respectively, p = 0.23). CONCLUSIONS: The foreskin tissue of smoking patients had a significant increase of elastic system fibers. Elastic fibers play an important role in this tissue’s turnover and this high concentration in smokers possibly causes high extensibility of the foreskin. The structural alterations in smokers’ foreskins could possibly explain the poor results in smoking patients submitted to foreskin fasciocutaneous flaps in urethral reconstruction surgery.

Imunofenotipagem e remodelamento da matriz extracelular na sarcoidose pulmonar e extrapulmonar / Immunophenotyping and extracellular matrix remodeling in pulmonary and extrapulmonary sarcoidosis

OBJETIVO: Investigar o significado de marcadores de imunidade celular e de componentes elásticos/colágeno da matriz extracelular em estruturas granulomatosas em biópsias de pacientes com sarcoidose pulmonar ou extrapulmonar. MÉTODOS: Determinações qualitativas e quantitativas de células inflamatórias, de fibras de colágeno e de fibras elásticas em estruturas granulomatosas em biópsias cirúrgicas de 40 pacientes com sarcoidose pulmonar e extrapulmonar foram realizadas por histomorfometria, imuno-histoquímica, e técnicas de coloração com picrosirius e resorcina-fucsina de Weigert. RESULTADOS: A densidade de linfócitos, macrófagos e neutrófilos nas biópsias extrapulmonares foi significativamente maior do que nas biópsias pulmonares. Os granulomas pulmonares apresentaram uma quantidade significativamente maior de fibras de colágeno e menor densidade de fibras elásticas que os granulomas extrapulmonares. A quantidade de macrófagos nos granulomas pulmonares correlacionou-se com CVF (p < 0,05), ao passo que as quantidades de linfócitos CD3+, CD4+ e CD8+ correlacionaram-se com a relação VEF1/CVF e com CV. Houve correlações negativas entre CPT e contagem de células CD1a+ (p < 0,05) e entre DLCO e densidade de fibras colágenas/elásticas (r = -0,90; p = 0,04). CONCLUSÕES: A imunofenotipagem e o remodelamento apresentaram características diferentes nas biópsias dos pacientes com sarcoidose pulmonar e extrapulmonar. Essas diferenças correlacionaram-se com os dados clínicos e espirométricos dos pacientes, sugerindo que há duas vias envolvidas no mecanismo de depuração de antígenos, que foi mais eficaz nos pulmões e linfonodos.

OBJECTIVE: To investigate the significance of cellular immune markers, as well as that of collagen and elastic components of the extracellular matrix, within granulomatous structures in biopsies of patients with pulmonary or extrapulmonary sarcoidosis. METHODS: We carried out qualitative and quantitative evaluations of inflammatory cells, collagen fibers, and elastic fibers in granulomatous structures in surgical biopsies of 40 patients with pulmonary and extrapulmonary sarcoidosis using histomorphometry, immunohistochemistry, picrosirius red staining, and Weigert's resorcin-fuchsin staining. RESULTS: The extrapulmonary tissue biopsies presented significantly higher densities of lymphocytes, macrophages, and neutrophils than did the lung tissue biopsies. Pulmonary granulomas showed a significantly higher number of collagen fibers and a lower density of elastic fibers than did extrapulmonary granulomas. The amount of macrophages in the lung samples correlated with FVC (p < 0.05), whereas the amount of CD3+, CD4+, and CD8+ lymphocytes correlated with the FEV1/FVC ratio and VC. There were inverse correlations between TLC and the CD1a+ cell count (p < 0.05), as well as between DLCO and collagen/elastic fiber density (r = -0.90; p = 0.04). CONCLUSIONS: Immunophenotyping and remodeling both showed differences between pulmonary and extrapulmonary sarcoidosis in terms of the characteristics of the biopsy samples. These differences correlated with the clinical and spirometric data obtained for the patients, suggesting that two different pathways are involved in the mechanism of antigen clearance, which was more effective in the lungs and lymph nodes.

Hereditary Sclerosing Poikiloderma

Hereditary sclerosing poikiloderma (HSP) is a very rare disease. The clinical features are principally widespread poikiloderma and linear hyperkeratotic and sclerotic bands. We report an 18-yr-old male who presented reticular hyperpigmented lesions on the trunk and extremities since 2-yr-old. Also, linear sclerosing bands appeared on both antecubital and popliteal fossae after yr. Histopathologic finding showed dense sclerotic collagen fibers with telangiectasia in the upper dermis and fragmentations of damaged elastic fibers in the elastic stain, consistent with HSP. We report the first Korean case of HSP.

Granuloma anular elastolítico gigantocelular: relato de caso / Annular elastolytic giant cell granuloma: case report

O granuloma anular elastolítico gigantocelular é dermatose granulomatosa rara, caracterizada, histologicamente, por fagocitose das fibras elásticas por células gigantes multinucleadas e, clinicamente, por placas anulares com bordas elevadas e centro atrófico, com preferência por áreas expostas. A patogênese é controversa. Descreve-se um caso de mulher de 44 anos, com achados clínicos e histológicos clássicos, que apresentou boa resposta à hidroxicloroquina, após o período de 1 mês.

Annular elastolytic giant cell granuloma is a rare granulomatous skin disease characterized histologically by phagocytosis of elastic fibers by multinucleated giant cells and clinically by annular patches with elevated borders and atrophic centers found mainly on sun-exposed skin. The pathogenesis of the disease is controversial. We report the case of a 44-year-old woman with classical clinical and histological findings of the disease whose condition improved when hydroxychloroquine was administered over a four-week period.

Granuloma actínico / Actinic granuloma

El granuloma actínico es una rara afección de la piel que se desarrolla en general en áreas expuestas al sol. Su patogénesis no es clara.La teoría más aceptada es la implicancia de la radiación solar como factor disparador. Típicamente la enfermedad se presenta en individuos mayores de 30 años con historia de exposición solar. Se manifiesta con lesiones anulares de bordes elevados y atrofia central,asintomáticas, con poca tendencia a la involución. Se han probado múltiples terapéuticas con éxito variado. Esta entidad poco frecuente fue descripta por primera vez en 1975 por O`Brien. Presentamos dos pacientes con granuloma actínico. El primero correspondió a una mujer de 83 años de edad con una lesión en pómulo izquierdo. El segundo a un varón de 70 años con una gran lesión que se extendía desde el epigastrio hasta las clavículas.

Actinic granuloma is a rare skin disorder usually developing uponsun-exposed areas. While its pathogenesis is unclear, the acceptedhypothesis maintains that solar radiation is the triggering factor.Typically the disease develops in individuals over 30 years of age witha history of sun exposure, showing annular lesions with raised bordersand central atrophy, asymptomatic, with little tendency to regression.Multiple therapies have been tested with variable response. Thisuncommon entity was described for the first time in 1975 by O’Brien.We present two patients with actinic granuloma. The first onecorresponded to an 83 years-old woman with a lesion on the leftcheek. The second one was a 70 years-old male with a large lesion thatextended from the epigastrium to the collar bones.

Morphological and ultrastructural characteristics of extracellular matrix changes in oral squamous cell carcinoma.

Background: The biology of oral squamous cell carcinoma (OSCC), including its progression from dysplasia to carcinoma, "field effects", genetic changes in tumor associated mucosa (TAM) and effect of matrix metalloproteinases in breaking down of matrix proteins to facilitate invasion, has been well documented. However, what remains to be done is to extrapolate this knowledge to improve patient care. Aim: The aim of this study was to observe the extracellular matrix (ECM) changes with the routine histochemical stains available to most histopathologists. Materials and Methods: The study includes 72 cases of OSCC in which the tumor and adjacent normal appearing areas were sampled to study the ECM changes with hematoxylin and eosin (H and E) and Verhoeff's-Van Gieson elastic stain (VVG). Results: Basophilic fragmentation of collagen (H and E) and clumped short elastic fibers (VVG) were seen in 12 (16.7%) cases. Of the remaining cases, 18 (25%) had a dense lymphocytic infiltrate and had no demonstrable elastic fibers. Those cases with H and E changes were further studied and compared with normal mucosa for ultrastructural changes. The ultrastructural study demonstrated an increase in oxytalan, elaunin and elastic fibers and decrease in collagen fibers with some transformation changes associated with OSCCs and lymph node metastasis. Conclusion: Changes in transformation of collagen to elastic fibers and also the loss of both the fibers in areas of lymphocytic infiltration possibly indicate degradation of ECM fibers by factors released from the lymphocytes or tumor cells and the limiting effect on the tumor by ECM remodeling.