Clinical and histopathological spectrum of genital lichen sclerosus in 133 cases: Focus on the diagnosis of pre-sclerotic disease

Background: Early inflammatory lesions of lichen sclerosus are histopathologically difficult to diagnose until the hallmark of the disease i.e., papillary sclerosis becomes visible in histological sections. Pre-sclerotic and late or resolved phases of the disease have not been extensively studied. Methods: We retrospectively reviewed all cases diagnosed as genital lichen sclerosus over a ten-year period from 2006 to 2016, correlating the clinical findings with the histological features. Results: A total of 133 cases of genital lichen sclerosus (90 males and 43 females) were identified. Both genders demonstrated a similar histological spectrum. Fifty eight (44%) cases were identified as having pre-sclerotic lichen sclerosus, 64 (48%) as having progressive disease and 11 (8%) cases were classified as fully resolved with atrophy. Asymptomatic vitiligoid lesions were identified in 19 (14%) cases of which 12 were male. Low-grade squamous cell carcinoma was seen within the areas affected by long-standing lichen sclerosus, in four patients (3%, 2 male). Limitations: We studied only haematoxylin and eosin stained sections. The presence of basement membrane thickening could have been better illustrated with the periodic acid–Schiff stain. Conclusion: The pathogenesis of lichen sclerosus probably involves an immune reaction to the basement membrane at the epidermal interface and around the adnexa. The initial band of inflammation shifts gradually downwards from the epidermal interface into the dermis destroying the vascular channels and appendages, resulting in excessive deposition of altered extracellular matrix. Basilar infiltration of lymphocytes along with a grossly vacuolated or thickened basement membrane is proposed as the characteristic diagnostic feature of the pre-sclerotic stage. Greater awareness of the clinicopathological spectrum of lichen sclerosus should enable early diagnosis and treatment, thereby preventing structural damage and possible malignant transformation in chronic cases

Cromoblastomicosis: primer caso alóctono tratado en Chile / Chromoblastomycosis: first allochthonous case treated in Chile

Resumen La cromoblastomicosis es una infección fúngica de la piel y del tejido subcutáneo, de evolución crónica, causada por hongos dematiáceos que se caracterizan por presentar melanina en su pared celular. La enfermedad se presenta en todo el mundo, principalmente en regiones tropicales y subtropicales. En Chile, solo hay un reporte de caso humano hace más de 30 años. Se presenta el caso de un varón de 46 años, haitiano, residente en Chile, con placas verrucosas en la zona tibial anterior de un año de evolución. El diagnóstico de cromoblastomicosis se confirmó al observar células muriformes en la histopatología y colonias dematiáceas en el cultivo micológico; además, en la miscroscopía directa se observaron conidias compatibles con Fonsecaea spp. Luego de seis meses de tratamiento con antimicóticos sistémicos y crioterapia, se logró la remisión completa de las lesiones.

Abstract Chromoblastomycosis is a fungal infection of the skin and subcutaneous tissue, of chronic evolution, caused by dematiaceous fungi. The disease occurs worldwide, mainly in tropical and subtropical regions, but in regions like Chile there is only one report of a human case more than 30 years ago. We present the case of a 46-year-old Haitian man, resident in Chile, with verrucous plaques in the right anterior tibial area of one year of evolution. The diagnosis of chromoblastomycosis was confirmed when muriform cells and dematiaceous colonies were observed in the histopathological analysis and the direct microscopy, respectively. After six months of treatment with systemic antimycotics and cryotherapy, complete remission of the lesions was achieved.

Effects of various surface treatments on the bonding efficacy of noncarious cervical sclerotic lesions / 华西口腔医学杂志

Noncarious cervical sclerotic lesions (NCSL) are dental cervical lesions with noncarious sclerotic dentine (NCSD), which appears smooth, hard, and either light yellow or dark brown. Most NCSLs are wedge or dish shaped and commonly occur in canines and premolars, leading to dental hypersensitivity and aesthetic defect. The principal treatment is composite resin restoration; however, many clinical problems, such as retention loss, should not be ignored. NCSL's bonding interface includes NCSD and enamel, and interface pre-treatment can promote the bonding effect. This review summarizes current surface treatment methods and their influence on the bonding effectiveness of NCSL to provide guidance for clinical practice.

Erdheim-Chester disease with multisystemic involvement: a diagnostic challenge

Erdheim–Chester disease (ECD) is a rare, non-inherited, non- Langerhans form of histiocytosis of unknown origin, first described in 1930. This entity is defined by a mononuclear infiltrate consisting of lipid laden, foamy histiocytes that stain positively for CD68. Individuals affected by this disease are typically adults between their 4th and 6th decades of life. The multi systemic form of ECD is associated with significant morbidity, which may arise due to histiocytic infiltration of critical organ systems. Among the more common sites of involvement are the skeleton, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum) and skin. The most common presenting symptom of ECD is bone pain. Bilateral symmetric increased tracer uptake on 99mTc bone scintigraphy affecting the periarticular regions of the long bones is highly suggestive of ECD. However, definite diagnosis of ECD is established only once CD68(+), CD1a(−) histiocytes are identified within a biopsy specimen with aid of clinical and radiological data. Here we present a rare case of Erdheim-Chester disease in a 46 year male patient based on clinical data, radiological data, histopathological and immunohistochemistry findings.

Effectiveness of autologous lipoinjection into the hand to improve function in patients with scleroderma: pilot study

Background: Scleroderma is a rare disease of the tissues that is characterized for being inflammatory and developing fibrosis in the skin. Typically, this disease affects middle-aged women.Methods: A study was conducted in which 10 patients with scleroderma with involvement in the sclerotic stage were included. One of the hands was chosen randomly for treatment with fatty graft processed by Coleman technique and the other hand, physiological solution was placed. Patients were evaluated at 45 and 90 days after the procedure using the Cochin Hand Functional Scale (CHFS) questionnaire, modified Rodnan scale for the skin (mRSS), grip strength with dynamometer and measurement of fingertip to palm of hand in flexion.Results: The average of CHFS before treatment was 42.30 and 25.70 at 90 days p=0.007. The average strength in the experimental hand before treatment was 11.67 and 14.58 at 90 days p=0.007, in the control hand p=0.873. The mean finger-palm tip distance before treatment was 44.80 and from 36.00 to 90 days p=0.019, in the control hand p=0.149. There is a significant difference in the degree of severity at 90 days of the mRSS of the back of the hands p=0.011 and phalanges p=0.000 between the patients with lipoinjection and physiological solution.Conclusion: Significant improvement was observed in patients with scleroderma treated with autologous lipoinjection.

Alteraciones de la dentina con el envejecimiento / Dentin alterations with aging

Diferentes estudios han demostrado que después de la tercera década de vida hay una transición en la microestructura de la dentina. Dada la importancia de ésta como sustrato para la adhesión de materiales de restauración utilizados en operatoria y rehabilitación oral, ha sido objetivo de este trabajo realizar una búsqueda bibliográfica de las publicaciones existentes en inglés y español de los últimos 15 años, haciendo uso de buscadores científicos como Pubmed, Google Schoolar y LILACS que permitieran actualizar la información existente ayudando a entender los efectos biológicos del envejecimiento de la dentina, identificando los cambios de mayor relevancia a nivel histológico. De la búsqueda realizada se concluye que el envejecimiento de la dentina está asociado con aumento de dentina secundaria, formación de dentina esclerótica opaca, variaciones en la composición química de la matriz y disminución del número y actividad de los odontoblastos. Los conocimientos sobre el envejecimiento de la dentina deben tenerse en cuenta frente a estudios relacionados con materiales de restauración dental, ya que los cambios en la microestructura y capacidad funcional de la dentina con el envejecimiento requieren que éstos se adapten a dichas variaciones (AU)

Different studies have shown that aafter the third term of life there is a transition in the microstructure of dentin. Given the importance of dentin as a substrate for the adhesion of restorative materials used in operative and oral rehabilitation, the aim of the present work was to conduct a search of the scientific literature in English and Spanish, published in the last 15 years, using search engines such as Pubmed, Google Schoolar and LILACS, for an update on the biological effects of dentin aging, to identify the most relevant age-related histological changes in dentina. The data obtained from the literature review allow concluding that dentin aging is associated with an increase in secondary dentina, opaque sclerotic dentin formation, variations in the chemical composition of the matrix and a decrease in odontoblast number and activity. Updated information on dentin aging should be taken into account in studies on dental restoration materials, since the latter must adapt to aging-related changes in the microstructure and functional capacity of dentin (AU)

Hemangioma cases and treatment / Шинэ санаа Шинэ нээлт

Hemangiomas are common benign vascular tumors which about 70% to 80% of the lesions are found in the head and neck region. This study was conducted on 36 children with hemangioma who were referred to Orofacial Surgery Cabinet of Dental School of Health Sciences University of Mongolia and Orofacial Surgery Cabinet of National Center for Maternal and Child Health of Mongolia between 2013 and 2015.Of the 36 children referred for assessment, 72% were female, 55% were infants of 4 to 9 months old, and 83% of them were citizens. 58% of patients underwent cryotherapy,and 57% of patients underwent cryotherapy had 2 therapy sessions. 25% of patients receiver sclerotic therapy, and 67% of them had 2 therapy sessions. According to the locations of hemangiomas 30% of them were localized in infraorbital area. 72% of the participants are women and 55% are children age of 4-9 month which 83% of all participants were living in city. 58% of children with hemangioma are treated with cryotherapy which 57%of treatment finished by second visit. And 25% were treated by dehydration treatment which 67% were finished by second visit.

Tuberous sclerosis with giant renal angiomyolipoma and sclerotic skeletal lesions in a geriatric patient: role of imaging.

Tuberous sclerosis is a rare inherited neurocutaneous syndrome, which has multisystem involvement. We present a geriatric female patient with chronic abdominal pain who was diagnosed with giant renal angiomyolipoma (AML) and multiple sclerotic skeletal lesions. Radiological imaging played an important role in identifying these two features and diagnosing tuberous sclerosis in our case.

Carcinoma of Mandible Involving The Floor of The Mouth - A Case Report.

Carcinoma of the floor of mouth represents 8% to 12% of all head and neck cancers. 85 to 95% of patients are males; the mean age is 58 years for men and 65 years for women. Oral carcinoma of the mandibular region has been defined as carcinoma of the mandibular alveolar ridge, lower buccal sulcus, sublingual sulcus and mandibular retro molar trigone . Lesions occurring in this area often involve mandible mostly by direct extension and seldom by other routes . The prevalence of mandibular bone involvement ranges from 12 to 56%.Classical clinical symptoms can be discomfort or pain under the mobile tongue, difficulty with protraction or swallowing, speech impairment, but more frequently, the dentist or the family physician discovers the disease

Long-term Follow-up of Metaphyseal Sclerotic Lines in Children Treated with Pamidronate

PURPOSE: Bisphosphonates are widely used for the management steroid-induced osteoporosis (SIO) in children. With the increasing use of bisphosphonates, there have been reports of abnormal radiological findings in the growing skeleton. Therefore, their use in pediatric patients remains controversial. The present study was conducted to evaluate the long-term follow-up radiographic features, particularly metaphyseal sclerotic lines, in children who receive pamidronate therapy for nephropathy. METHODS: Twenty-four children with nephropathy treated with oral calcium and pamidronate (mean duration, 9 months; dose, 100 mg daily), were evaluated retrospectively. All patients had SIO secondary to chronic glucocorticoid therapy for treating nephropathy. Long bone radiographic imaging was performed before treatment with pamidronate, and at follow-up, several years later. Physeal growth rates were estimated by measuring the distance that the sclerotic lines moved on the radiographs during the corresponding time intervals. RESULTS: The mean follow-up period was 138 months. Long bone radiographs showed well-defined sclerotic lines at the metaphyseal ends, progressively moving from the physeal plate to the diaphysis, in all patients. The mean rate of movement of the sclerotic line was 6.21 mm per year. In 12 patients, the lines disappeared. The mean rate of growth in height was 7.33 cm per year. CONCLUSIONS: Results of long-term follow-up suggest that the metaphyseal sclerotic lines associated with pamidronate treatment tend to disappear without affecting overall skeletal growth. Bisphosphonate treatment for SIO in children with nephropathy seems to be safe, although further studies in larger number of patients are needed.

The application of Chaperon guiding catheter system in endovascular treatment of intracranial aneurysms / 介入放射学杂志

Objective To discuss the application of Chaperon guiding catheter system in endovascular treatment of intracranial aneurysms. Methods A total of 20 patients with intracranial aneurysms were enrolled in this study. The patients hadⅡorⅢtype of aortic arch (n=11) or sclerotic plague at the orifice of internal carotid or vertebral artery (n = 9). Endovascular embolization of the intracranial aneurysm was carried out in all patients. By using Cordis guiding catheter system the catheter was placed into the target artery. Chaperon guiding catheter system was used during the procedure in order to determine whether the Chaperon guiding catheter could be smoothly placed into the target artery or not. Results When the Chaperon guiding catheter system was employed in the endovascular procedure, the difficulties of catheterization caused by the distortion of the aorta or by the plagues on the walls of arteries could be basically overcome. The Guiding catheter could be smoothly placed into the target arteries. Conclusion The Chaperon guiding catheter system can be successfully used in the endovascular treatment for the intracranial aneurysms, especially when the patient has tortuous aorta or there is sclerotic plague on the artery wall. (J Intervent Radiol, 2014, 23:281-283).

Chromoblastomycosis in Kerala, India.

Background: We are reporting 35 new cases of Chromoblastomycosis from Central Kerala. A majority of the cases from India are reported from the Sub Himalayan belt and South India. The disease scenario in India and abroad is briefly reviewed. Aims: To study chromoblastomycosis in Central Kerala including the demographic and clinico- investigative profile. Methods: This report is a retrospective record analysis of 35 cases of chromoblastomycosis who presented to the Dermatology Outpatient department of our tertiary center from January 2003 to July 2010 after obtaining Institutional Review Board approval. Results: The disease was found to be more common among male agriculturists. The majority of cases were from the central districts of Kerala in and around the Western Ghats. The lower extremity (60%) was more affected with 40% of the subjects remembering a prior history of trauma. Sclerotic bodies were demonstrable in scrapings from black dots in 42.8%. The characteristic mixed mycotic granuloma was demonstrable in 77.1% of cases. The most common species isolated was Fonsecaea pedrosoi. Conclusions: Chromoblastomycosis is very common in Central Kerala. The disease mainly affects male agriculturists especially those employed in rubber plantations. The most common organism is F. pedrosoi.

Concurrent mycetoma and chromomycosis.

Chromoblastomycosis and Madura foot are chronic localised mycotic infection of the skin and subcutaneous tissue that follows the implantation of the fungi through minor trauma, mainly found in persons working outdoors on bare foot. In cases where both Madura and chromoblastomycosis are present, the treatment becomes difficult with low cure rates and frequent relapses. Here, we present such a very rare case of a 38-year-old cattle farmer who presented with verrucose nodules, tumefaction and multiple discharging nodules on the left lower 1/3 rd leg and foot since last 9 years. Direct KOH mount of the verrucose tissue showed Fonsecaea pedrosoi sclerotic muriform bodies and a biopsy of one granule discharging nodule demonstrated fungal mycetoma. He was put on tab. Itraconazole 200 mg o.d. and cotrimoxazole bid for 6 months with very little improvement. The rarity of this combination is most probably due to different geographical distribution.

Pedunculated Sclerotic Fibroma Resembling Soft Fibroma / 대한피부과학회지

Sclerotic fibroma is a rare skin neoplasm that can occur seen sporadically or in association with Cowden's disease. Clinically, it presents as asymptomatic flesh-colored or pinkish slow-growing papules or nodules with a wide anatomical distribution. We report a rare case of pedunculated type of sclerotic fibroma resembling soft fibroma, which has not been reported in Korea.

'Unstained' and 'de stained' sections in the diagnosis of chromoblastomycosis: A clinico-pathological study.

Background: Diagnosis of chromoblastomycosis is frequently missed for many reasons: (1) rarity of the lesion (2) requirement of careful search for diagnostic 'sclerotic' bodies which are often sparse in clinical material such as tissue and exudates (3) often they elicit tissue reactions such as verrucous lesion and micro abscesses, misleading the diagnosis (4) lack of 'clinical suspicion'. Aims: (1) To compare the feasibility of 'unstained', 'stained' and 'de stained' sections in identification of the diagnostic ' sclerotic' bodies (2) To study histopathological features of chromoblastomycosis, and (3) To highlight the importance of clinical suspicion in the diagnosis of chromoblastomycosis. Design : This is a retrospective study conducted on six clinically unsuspected, histopathologically diagnosed cases of chromoblastomycosis. Results: Most common clinical presentation was verrucous lesion over extremities affecting adult men engaged in outdoor works. Sclerotic bodies and their characteristic features were easily identified in both 'unstained' and 'de stained' sections. Special stains such as Fontana Masson and Gomori's methenamine silver nitrate are useful for demonstrating hyphal forms in keratinized layers and for illustration purposes. Conclusion: Both 'unstained' and 'de stained' sections can be used for rapid screening of sclerotic bodies. 'De stained' sections can be used as a suitable substitute for special stains for confirmation and for retrospective review of all verrucous lesions to diagnose the missed lesion. Clinical suspicion is very important in the diagnosis of chromoblastomycosis as it helps pathologist to screen for diagnostic sclerotic bodies.

Linhas escleróticas metafisárias em crianças e adolescentes em uso de alendronato / Sclerotic metaphyseal lines in children and adolescents treated with alendronate

INTRODUÇÃO: Os bisfosfonatos inibem a reabsorção óssea pela interferência na ação dos osteoclastos. Dentre os efeitos adversos, as linhas escleróticas em metáfise de ossos longos são descritas como principal alteração radiográfica na faixa etária pediátrica. OBJETIVO: Avaliar a frequência de alterações radiográficas causadas pelo alendronato utilizado em crianças e adolescentes com baixa densidade óssea ou calcinose. PACIENTES E MÉTODOS: Foi realizado um estudo do tipo coorte retrospectiva analisando-se prontuários de 21 pacientes que fizeram uso de alendronato semanal por no mínimo 10 meses. Os pacientes realizaram radiografias de ossos longos antes do início do alendronato e aproximadamente um ano após o seu uso. RESULTADOS: Onze pacientes (52,3 por cento) apresentaram linhas escleróticas em metáfise dos ossos longos. A localização mais frequente foi em tíbia (8/11 pacientes), seguida de fêmur (7/11), úmero (6/11), rádio (4/11), ulna (3/11) e fíbula (2/11). Nenhum paciente apresentou regressão das alterações radiográficas durante o tempo de evolução (até 1,1 ano após a suspensão do alendronato). CONCLUSÃO: Se usado com critério, o alendronato é seguro e as alterações radiográficas não mostraram ter um significado mais importante.

INTRODUCTION: Bisphosphonates inhibit bone resorption by interfering with the action of osteoclasts. Among the adverse effects, sclerotic lines observed in the metaphysis of long bones have been described as the main imaging finding in pediatric patients. OBJECTIVE: To evaluate the frequency of radiographic changes caused by alendronate in children and adolescents with low bone density or calcinosis. PATIENTS AND METHODS: We conducted a cross-sectional study with 21 patients who were treated with once-weekly alendronate for at least 10 months. Patients underwent x-rays of long bones before the start of alendronate and approximately one year after its use. RESULTS: Eleven patients (52.3 percent) had sclerotic lines in the metaphysis of long bones. The most frequent site was the tibia (8/11 patients), followed by the femur (7/11), humerus (6/11), radius (4/11), ulna (3/11), and fibula (2/11). Regression of radiographic changes during the study period (up to 1.1 years after discontinuation of alendronate) was not observed. CONCLUSION: If used carefully, alendronate is safe and radiographic changes have not been shown to be clinically relevant.

Cowden Syndrome Accompanied by Multiple Sclerotic Fibroma on the Face / 대한피부과학회지

Cowden syndrome (CS) is a rare genodermatosis that is characterized by multiple hamartomatous tumors of an ectodermal, mesodermal and endodermal origin. CS is associated with an increased risk of malignancy and especially breast cancer, thyroid cancer, uterine cancer and renal cell carcinoma. The characteristic features of the disease are mucocutaneous findings, including multiple facial trichilemmomas, oral mucosal papillomatosis and acral keratoses. Multiple sclerotic fibroma is also known to be a clue to diagnose CS. We herein emphasize that multiple sclerotic fibromas of the skin are an important marker of CS, which may also be helpful for making an early diagnosis.

Estudo comparativo in vitro das estruturas orgânicas e inorgânicas da dentina saudável e esclerosada humana e bovina: nanodureza, concentração de Ca e P e análise morfológica / In vitro comparative study of organic and inorganic components analysis of health and sclerotic human and bovine dentin: nanohardness, Ca and P concentration and morphological analysis

A dentina esclerosada é um substrato comumente encontrado em pacientes idosos. No entanto, existem poucos estudos comparativos entre dentina humana e bovina esclerosadas. O objetivo deste estudo foi comparar os componentes inorgânicos e orgânicos da dentina saudável e esclerosada humana e bovina, através de cinco parâmetros: nanodureza, módulo de elasticidade, análise quantitativa da concentração de Cálcio (Ca) e Fósforo (P), densidade tubular e morfologia do colágeno. Trinta dentes humanos e 30 bovinos foram distribuídos em 4 grupos experimentais (n=15 por grupo): dentina humana saudável (DHS), esclerosada humana (DEH), bovina saudável (DBS), e bovina esclerosada (DBE). Os dentes saudáveis foram preparados na mesma altura e inclinação dos dentes esclerosados expondo níveis similares da dentina e obtendo fragmentos com 2mm de espessura. Foram realizadas 3 medições por espécime em 3 áreas pré determinadas de dentina intertubular com a utilização do Nanoindentador (carga de 500N por 5 s). Cinco espécimes de cada grupo foram preparados para Microscopia Eletrônica de Varredura (MEV). Com o auxílio da Energia Dispersiva por Raios-X EDX foram obtidos os valores (em percentagem) das concentrações de Ca e P e calculada a relação Ca:P. A contagem dos túbulos por área foi realizada em todas as eletromicrografias. Após descalcificação e preparo, o restante dos espécimes foi analisado em Microscopia Eletrônica de Transmissão (MET)...

The sclerotic dentin has been commonly found in elderly patients. However, there are scarce reports in the literature comparing on the use of human and bovine sclerotic dentins. The objective of this study was to compare inorganic and organic components of healthy and sclerotic dentins from human and bovine. Five parameters were analyzed: nanohardness, elastic modulus, quantitative analysis of Calcium (Ca) and Phosphorous (P) concentrations, tubular density and ultrastructural morphology. Thirty human teeth plus 30 bovine teeth were distributed in 4 experimental groups (n=15 per group): human healthy dentin (HHD), human sclerotic dentin (HSD), bovine healthy dentin (BHD) and bovine sclerotic dentin (BSD). Healthy teeth were cut in the same level and inclination of the sclerotic superficial dentins. The nanohardness and elastic modulus (GPa) of three pre determined areas of each exposed dentin was measured using a nanoindenter (500N for 5s). Five samples of each group were prepared for scanning electron microscopy (SEM) examination. Energy Dispersive X-ray (EDX) was used for obtaining the Ca/P ratio. The tubular density was obtained by counting the tubules in scanning electron micrographs taken in the same magnification and work distance...

Experimental murine chromoblastomycosis obtained from Fonsecaea pedrosoi isolate cultured for a long periodt

The present study aimed to describe F. pedrosoi propagules capable of causing chronic murine disease. Several changes in F. pedrosoi hyphae were identified in fungal cells cultured for a long period. Optical microscopy found many rounded cells with double-rigid melanin-rich walls. Terminal and intercalary chlamydoconidia were also frequently observed. Analyses of images from transmission electron microscopy (TEM) revealed several cells with walls composed of at least three layers and an outer layer enriched with melanin. Two groups of twenty BALB/c mice were subcutaneously infected in their footpads with F. pedrosoi cells at an inoculum concentration of approximately 1 x 10(4) cells/mL. In one group, long-term cultured F. pedrosoi cells were inoculated in one footpad, whereas in the other group, both footpads were infected. Active lesions were observed up to seven months post-infection, particularly in mice inoculated at two sites. After this period, animals were killed. Histological sections revealed characteristics bearing a strong resemblance to the human form of the disease such as tissue hyperplasia, granulomas with microabscesses and sclerotic cells. Based on this study, we identified fungal cells from old cultures capable of provoking chronic chromoblastomycosis under experimental conditions, especially when more than one site is infected.