Juvenile localized scleroderma: Clinical and epidemiological features in 750 children. An international study

Objective. Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres. Methods. A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS. Results. Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr. Conclusion. This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome. © 2006 Oxford University Press.

Clinical and histologic features of botryoid odontogenic cyst: A case report

Introduction. The lateral periodontal cyst, as the name implies, occurs on a lateral periodontal location and is of developmental origin, arising from cystic degeneration of clear cells of the dental lamina. A botryoid odontogenic cyst is considered to be a rare multilocular variant of a lateral periodontal cyst. Case presentation. We report the clinical and histopathologic features of a rare case of botryoid odontogenic cyst found in an edentulous area corresponding to the right lower canine of a 64-year-old African-American woman. A multilocular radiolucency was observed, and surgical removal of the lesion revealed a nodule of rubber-like consistency measuring about 1.5 cm in diameter. Cross-sectioning of the nodule showed that it consisted of various cystic compartments. Histologically, various voluminous periodic acid-Schiff-negative clear cells randomly distributed throughout the cystic epithelium were observed, as well as cell layers showing thickenings generally formed by oval, sometimes entangled plaques. The capsule consisted of fibrous connective tissue and showed rare and discrete foci of a perivascular mononuclear inflammatory infiltrate and reactive bone-tissue fragments. The final diagnosis was botryoid odontogenic cyst. Conclusion: We provide data that allow the reader to establish the differences between botryoid odontogenic cyst, glandular odontogenic cyst, and lateral periodontal cyst, helping with the differential diagnosis. The reader will have the opportunity to review botryoid odontogenic cyst clinical and histopathologic features, including treatment. © 2010 Farina et al; licensee BioMed Central Ltd.

Clinical, cytologic, and histologic features of a mammary micropapillary carcinoma in a dog

Mammary invasive micropapillary carcinoma is a rare variant of mammary carcinoma that was recently recognized in dogs. The cytologic features and biologic behavior of such neoplasms in dogs have not yet been widely discussed in the veterinary literature. We report the clinical, cytologic, and histologic features of a canine micropapillary carcinoma in a 13-year-old female mongrel dog. The mammary region presented with extreme local pain, severe edema and erythema, and multifocal epidermal ulceration, which is typical for an inflammatory mammary carcinoma. Fine-needle aspirates were highly cellular and consisted of individual cells and papillary cell clusters with characteristics of malignant epithelial cells. Histologic examination revealed neoplastic cells arranged in small papillae without fibrovascular cores, sometimes inside clear lymphatic spaces, indicating lymphovascular invasion. Regional lymph node evaluation revealed metastatic cells. Due to deteriorating clinical condition the dog was euthanatized 5 months after mastectomy. At necropsy, metastatic neoplastic mammary cells were found in popliteal and mediastinal lymph nodes, the right femoral biceps muscle, liver, heart, lungs, and urinary bladder. © 2013 American Society for Veterinary Clinical Pathology.

Chronic reparative changes in medium-sized vessels in a case of primary cutaneous anaplastic large-cell lymphoma with angioinvasive features and cytotoxic phenotype: new histopathological findings in line with indolent clinical behavior

Angioinvasion/angiodestruction has been reported in a small subset of primary cutaneous anaplastic large-cell lymphomas (PCALCL). Recently, PCALCL with angioinvasive features and cytotoxic phenotype has been characterized as a variant associated with good clinical outcomes despite worrisome histopathologic features. We report a case of PCALCL with angioinvasive features and cytotoxic phenotype associated with reparative changes on the wall of medium-sized vessels involved by the neoplasm, including intimal fibroblastic proliferation and luminal obliteration. This vascular pattern, although previously unreported in PCALCL, is in accordance with the indolent behavior observed in this entity and provides a further link with lymphomatoid papulosis type E.

Emergence agitation in pediatric anesthesia: current features

Objective: Postoperative agitation in children is a well-documented clinical phenomenon with incidence ranging from 10% to 67%. There is no definitive explanation for this agitation. Possible causes include rapid awakening in unfamiliar settings, pain (wounds, sore throat, bladder distension, etc.), stress during induction, hypoxemia, airway obstruction, noisy environment, anesthesia duration, child's personality, premedication and type of anesthesia. The purpose of this paper is to discuss the possible causes of postoperative agitation in children, providing a foundation for better methods of identifying and preventing this problem.Sources: MEDLINE and PubMed were searched using the following words: emergence, agitation, incidence, etiology, diagnosis, treatment, children, pediatric, anesthesia.Summary of the findings: This study includes a review of potential agitation trigger factors and a proposal for a standardized diagnostic score system, in addition to measures to improve prevention and treatment.Conclusion: No single factor can identified as the cause of postoperative agitation, which should therefore be considered a syndrome made up of biological, pharmacological, psychological and social components, and which anesthesiologists and pediatric intensive care specialists should be prepared to identify, prevent and intervene appropriately as necessary.

Association between CD31 expression and histopathologic features in stage IB squamous cell carcinoma of the cervix

This study was undertaken to evaluate the association between the expression of CD31 in the tumor and the histopathologic findings in patients with carcinoma of the cervix. This study included prospectively 30 women, aged 46.6 +/- 10.7 years, with stage IB squamous cell carcinoma of the cervix submitted to radical hysterectomy from November 2001 to September 2002. Samples from the tumor were taken and immunohistochemically evaluated by a monoclonal antibody for CD31. Clinicopathologic characteristics such as stage, tumor size, grade of differentiation, lymphatic vascular space invasion (LVSI), parametrial involvement, and status of pelvic lymph nodes were also recorded. The clinical stage (FIGO) was IB1 in 22 patients (73.3%) and IB2 in 8 patients (26.7%). The expression of CD31 was significantly associated with tumor size and the presence of LVSI, but not with grade of differentiation and vaginal or parametrial involvement (P= 0.03, P= 0.032, P= 0.352, P= 0.208, and P= 0.242, respectively). on univariate analysis, the presence of pelvic lymph node metastasis was influenced by LVSI (P= 0.003) and CD31 expression (P= 0.032). However, on multivariate analysis, the presence of LVSI (P= 0.007) was the only independent predictor of pelvic lymph node metastasis. The CD31 expression in tumor is significantly associated with LVSI and tumor size in patients with early-stage squamous cell carcinoma of the cervix.

Hydatidiform Mole in Ectopic Pregnancy Clinical, Imaging, Pathological and Immunohistochemical Characteristics

OBJECTIVE: To determine the clinical, pathological, immunohistochemical and imaging characteristics of hydatidiform mole in ectopic pregnancy (HMEP) in all the cases admitted to the Department of Obstetrics and Gynecology, University Hospital of Caracas (HUC), Central University of Venezuela.STUDY DESIGN: Retrospective and comparative study, based on clinical records review of 2 groups: 10 cases with a diagnosis of HMEP and 20 cases with intrauterine hydatidiform mole (IUHM) admitted to the Obstetrics and Gynecology Department of HUC from 1996 to 2010. Clinical, pathological, immunohistochemical and imaging features were analyzed.RESULTS: The prevalence of HMEP in this study was 0.14:1,000 pregnancies; in this group the mean age was 28.8 years, and the mean gestational age at admission was 8.6 weeks. Both groups (HMEP and IUHM) were comparable in these last variables. Abdominal pain and genital bleeding were the most common clinical symptoms in the HMEP group, while it was vaginal bleeding in the IUHM group. Ultrasound findings were similar to those traditionally described in nonmolar ectopic pregnancy. Histology and immunohistochemistry showed that all cases of HMEP were partial mole.CONCLUSION: Although in this study the prevalence of HMEP was high, the size of the sample limits definitive conclusions. This study concludes that all cases of HMEP are partial mole. (J Reprod Med 2012;57:329-332)

Clinical presentation and salivary gland histopathology of paediatric primary Sjogren's syndrome

ObjectiveExplore the presentation, diagnostic criteria and exocrine gland histopathology of paediatric primary Sjogren's syndrome (PPSjS).MethodsA case series of 8 children is reported and American-European Consensus Group (AECG-2002) criteria were examined, as well as minor labial salivary and lachrymal gland biopsies, which were scored by a pathologist blinded to outcome. For all cases, connective tissue diseases and parotid-related infectious disease were excluded.ResultsAge at onset varied from 5-13 years old; 6 were females, all followed from diagnosis up to the last visit (1-10 years). The main features at presentation were recurrent tender parotid swelling and sialectasis imaging, with decreased salivary function assessed by Tc-99 scintigraphy. Mild sicca symptoms were observed in 4/8 cases. Systemic features, including fatigue, myalgia, arthritis, tenosynovitis, joint contractures, transient Raynaud's and high ESR, were recorded at onset. Autoantibody profile was unremarkable for diagnosis, while lymphocytic infiltration of labial salivary glands and sialectasis were observed in all biopsies (8/8). In lachrymal glands, massive lymphocytic infiltration and lymphocytic gastritis were observed during complementary assessment. Flares were treated with low dose steroids and long-term use of hydroxychloroquine (5/8), although only 318 fulfilled AECG-2002 diagnostic criteria, throughout the disease course.ConclusionPPSjS is rare, slowly progressive and its early presentation is variable. Standardised diagnostic algorithms should include recurrent parotid swelling and early diagnosis should rely mostly on salivary and lachrymal gland histopathology in this age group.

Immunohistochemical and morphological features of a small bowel leiomyoma in a black crested macaque (Macaca nigra)

Background: Spontaneous gastrointestinal neoplasms in non-human primates are commonly seen in aged individuals. Due to genetic similarities between human and non-human primates, scientists have shown increasing interest in terms of comparative oncology studies.Case presentation: The present study is related to a case of an intestinal leiomyoma in a black crested macaque (Macaca nigra), kept on captivity by Mateca a Zoo, Pereira City, Colombia. The animal had abdominal distension, anorexia, vomiting, diarrhea and behavioral changes. Clinical examination showed an increased volume in the upper right abdominal quadrant caused by a neoplastic mass. The patient died during the surgical procedure. Necropsy revealed several small nodules in the peritoneum with adhesion to different portions of the small and large intestines, liver, stomach and diaphragm. Tissue samples were collected, routinely processed and stained by H&E. Microscopic examination revealed a mesenchymal tumor limited to tunica muscularis, resembling normal smooth muscle cells. Neoplastic cells were positive for alpha-smooth muscle actin and vimentin, and negative for cytokeratin AE1/AE3 by immunohistochemistry. Those morphological and immunohistochemical findings allowed to diagnose the intestinal leiomyoma referred above.Conclusion: Neoplastic diseases in primates have multifaceted causes. Their manifestations are understudied, leading to a greater difficulty in detection and measurement of the real impact provides by this disease.

A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases

ObjectiveTo describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry.MethodsInclusion criteria were onset age lower than 18 years and a diagnosis of any idiopathic inflammatory myopathy (IIM) by attending physician. Bohan & Peter (1975) criteria categorisation was established by a scoring algorithm to define JDM and JPM based oil clinical protocol data.ResultsOf the 189 cases included, 178 were classified as JDM, 9 as JPM (19.8: 1) and 2 did not fit the criteria; 6.9% had features of chronic arthritis and connective tissue disease overlap. Diagnosis classification agreement occurred in 66.1%. Medial? onset age was 7 years, median follow-up duration was 3.6 years. Malignancy was described in 2 (1.1%) cases. Muscle weakness occurred in 95.8%; heliotrope rash 83.5%; Gottron plaques 83.1%; 92% had at least one abnormal muscle enzyme result. Muscle biopsy performed in 74.6% was abnormal in 91.5% and electromyogram performed in 39.2% resulted abnormal in 93.2%. Logistic regression analysis was done in 66 cases with all parameters assessed and only aldolase resulted significant, as independent variable for definite JDM (OR=5.4, 95%CI 1.2-24.4, p=0.03). Regarding treatment, 97.9% received steroids; 72% had in addition at least one: methotrexate (75.7%), hydroxychloroquine (64.7%), cyclosporine A (20.6%), IV immunoglobulin (20.6%), azathioprine (10.3%) or cyclophosphamide (9.6%). In this series 24.3% developed calcinosis and mortality rate was 4.2%.ConclusionEvaluation of predefined criteria set for a valid diagnosis indicated aldolase as the most important parameter associated with de, methotrexate combination, was the most indicated treatment.

Clinical, radiographic, biochemical and histological findings of florid cemento-osseous dysplasia and report of a case

A displasia cemento-óssea florida tem sido descrita como uma condição que afeta tipicamente os maxilares de mulheres negras de meia idade. Ela geralmente se manifesta como múltiplas massas radiopacas semelhantes ao cemento distribuídas nos maxilares. Esta condição também tem sido classificada por vários autores como cementoma gigantiforme, osteomielite esclerosante crônica, osteíte esclerosante e massas de cemento escleróticas. Os autores apresentam um caso de displasia cemento-óssea florida não complicada em uma mulher negra de 48 anos de idade. Múltiplas massas escleróticas com bordas radiolúcidas na mandíbula foram identificadas radiograficamente. Os achados histopatológicos revelaram formação de massas escleróticas densas calcificadas semelhantes ao cemento. Todos os aspectos clínicos, radiográficos, bioquímicos e histológicos foram sugestivos do diagnóstico de displasia cemento-óssea florida.

Clinical and histomorphometric characteristics of three different families with hereditary gingival fibromatosis

Objective. To examine the histomorphologic and histomorphometric features of tissue from 3 unrelated families with hereditary gingival fibromatosis (HGF).Study design. Twelve affected individuals from 3 HGF families and 3 control subjects were evaluated. Gingival samples were fixed in formalin and embedded in paraffin for hematoxylin and eosin stain to count the number of fibroblast and inflammatory cells. Sirius red staining was performed to quantitate the amount of collagen present.Results. Histomorphologic analysis of HGF showed extension of epithelial rete ridges into the underlying lamina propria and the presence of collagen bundles in the connective tissue. Analysis of the mean area fraction of collagen showed that there were significant increases in the collagen fraction for all HGF types compared with control subjects (P < .05). There were significant increases in the number of fibroblasts for HGFa and HGFb compared with control subjects (P < .05). The number of fibroblasts for HGFc were similar to that for control subjects.Conclusions. The collagen fraction was significantly greater in all HGF types compared with controls. The number of fibroblasts was significantly increased in 2 of the 3 HGF types compared with controls. These data indicate that different mechanisms may be responsible for tissue enlargement in different forms of HGF.

Experimental lamellar keratoplasty in rabbits using microfibrilar cellulose membrane: clinical, morphological and immunohistochemical findings

Avaliaram-se aspectos clínicos, histopatógicos e imunoistoquímicos da córnes de coelhos da raça Nova Zelândia adultos e machos em ceratoplastias lamelares com membrana de celulose microfibrilar. Trinta animais distribuídos em cinco grupos (n=6) foram estudados por até 60 dias de pós-operatório. A avaliação clínica revelou manifestações moderadas de edema, blefaroespasmo e fotofobia ao segundo dia, evoluindo para formas discretas ou ausentes a partir do sétimo dia, período em que se observou, clinicamente, reparo do defeito corneal. A histopatologia revelou uma fina camada de células escamosas, recobrindo a área lesada já aos sete dias, com discreto infiltrado de células polimorfonucleares. Observaram-se vasos no epitélio a partir do 15o dia, com regressão ao 48o dia. A marcação com o anticorpo Ki67 mostrou aumento de células em proliferação aos 15 dias no epitélio e aos 30 dias no estroma. Nesse período, ocorreram remodelamento e adesão epitealial. Considerando a boa integração do implante, admite-se a membrana de celulose como um bom material a ser utilizado em ceratoplastia lamellar.

Inv dup (15): Is the electroclinical phenotype helpful for this challenging clinical diagnosis?

Objective: To study the electroclinical phenotype in 5 patients with large Supernumerary marker chromosome referred as inv dup (15), in an attempt to analyze the electroclinical spectrum in order to determine if the binomial epilepsy-EEG is stereotyped enough to corroborate this challenging diagnosis.Methods: Five patients with large inv dup (15) were submitted to EEG and/or V-EEG, with a minimum duration of 2 h. Two certified neurophysiologists analyzed all EEG tracings simultaneously, blinded to clinical and molecular data. Epilepsy was characterized by detailed history and a standard questionnaire according to International League Against Epilepsy guidelines and corroborated by V-EEG findings.Results: Epilepsy started during infancy in 4 patients, in 3 with spasms. Spasms were easily controlled in one but not in others. Epilepsy evolved with generalized seizures in two patients and, generalized and focal in one. Currently, 3 patients present refractory epilepsy and two are seizure-free. In one patient, only one isolated episode suggestive of a secondary generalized tonic-clonic event occurred at the age of 12 years without recurrence. Regarding the EEG, patients had distinct features, except for two patients with very high amplitude fast activity, resembling recruiting rhythm. Despite good seizure outcome in 3 patients, EEGs remained remarkably abnormal with frequent epileptiform discharges over poorly organized background.Conclusions: Our data showed a heterogeneous electroclinical phenotype with generalized and partial epilepsy, presenting distinct degrees of severity and refractoriness.Significance: Our findings suggest that it is not possible to delineate an electroclinical phenotype in this neurogenetic syndrome. Therefore, inv dup (15) remains as a diagnostic challenge and epilepsy and EEG features are valuable only when inserted in the proper clinical context. (c) 2006 International Federation of Clinical Neurophysiology. Published by Elsevier B.V.. All rights reserved.

Anti-neutrophil cytoplasmic antibodies (ANCA) in the clinical forms of leprosy

Anti-neutrophil cytoplasmic antibodies (ANCA) are autoantibodies against enzymes present in primary granules of neutrophils and lysosomes of monocytes detected in systemic vasculitis and in other diseases, including infections, ANCA are markers of active Wegener granulomatosis, which presents some anatomo-pathologic and immune response features similar to those of leprosy. Thus, we raised the hypothesis that ANCA may be present in leprosy as markers specifically linked to the presence of vasculitis. The aim of this study was to determine the presence of ANCA in leprosy and its correlation with the clinical forms of the disease. Sera from 60 normal individuals and from 59 patients with different clinical forms of leprosy were studied. The patients were also allocated into reactional and nonreactional groups. By indirect immunofluorescence, ANCA were positive, an atypical pattern A-ANCA, in 28.8% of the patient sera. A-ANCA predominated, although not significantly (p >0,05), in the reactional groups (37.9% vs 20.0%), and in those at the lepromatous pole (41.6% vs 20.0%). There was no correlation between ANCA positivity and either disease duration, disease activity, or therapeutic regimen (p >0.05), An interesting finding was the correlation between ANCA and gender: 94.1% of ANCA-positive patients were males (p <0.01), a feature that so far has not been reported in ANCA-related diseases and for which there is no explanation at the moment. By ELISA, the sera of the lepromatous leprosy patients did not show activity against either PR3, MPO, HLE, the most common ANCA antigens. Because A-ANCA are nonspecific, this finding requires further investigation for the determination of the responsible antigen(s), in conclusion, A-ANCA are present in 28.8% of leprosy patients but are not related to vasculitis in the erythema nodosum leprosum reaction and are not a marker of a specific clinical form.