Dystrophic calcinosis in a child with a thumb sucking habit: case report

We present an uncommon case of a 3-year-old boy with a finger sucking habit who developed dystrophic calcification in his left thumb. Two years after excision, there was no recurrence, and the thumb retained full range of motion. We also discuss its probable pathogenesis and present a brief review of the literature about orthopedic complications in the hand due to this habit.

MDCT imaging of calcinosis in systemic sclerosis.

Calcinosis is a typical feature of systemic sclerosis (SSc) and can be found in many different tissues including the superficial soft tissues, periarticular structures, muscles, and tendons. It can also provoke erosive changes on bones. Investigation is conducted most often with plain radiographs. However, when a more detailed assessment is necessary, multidetector computed tomography (MDCT) is helpful owing to its multiplanar reformat (MPR) ability. The purpose of this review is to provide an overview of the various appearances of calcinosis in SSc patients as visualized at MDCT.

Calcinose tumorale, à propos d'un cas pédiatrique [Tumoral calcinosis in a child: a case report and review]

Tumoral calcinosis (Ct) is a rare pathology of unknown origin.We present the clinical, imaging ( including CT and MRI), surgical and pathology findings of Ct in a 9 year-old boy who presented with an incidental finding of a large elbow mass. The MR aspect of Ct has been reported only once and this case is the second description in a child. The association of Ct and dermatomyositis, as reported hereby, has also been described only once. With respect to treatment alternatives, we believe that it is important for radiologists to recognise this rare pathology in pediatric patients.

Arterial diffuse intimal thickening associated with enzootic calcinosis of sheep

Morphometric, immunohistochemical and ultrastructural studies were carried out on the diffuse intimal thickening (DIT) in arteries of 7 sheep with clinical signs of naturally occurring enzootic calcinosis due to ingestion of the plant Nierembergia veitchii. Arterial lesions consisted of medial deposition of calcium salts and DIT. Calcification of the intima was rare, mild and located near the elastic lamina. By immunohistochemistry a-actin was detected in cells of the media and in cells forming the intimal thickening. Receptors for 1,25(OH)2 vitamin D3 were detected in nuclei of intimal, medial and endothelial cells. DIT was irregularly distributed and was neither proportionally related to the intensity of the underlying mineralization area nor to the thickening of the remaining media. Ultrastructural morphometry in smooth muscle cells (SMCs) of the media and thickened intima revealed, in the latter, an increase of 318% in the volumetric fraction of those organelles involved in synthesis and a proportional decrease in contractile elements when compared to normal values of media cells. There were histological and ultrastructural evidences of modification of SMCs and their migration to the intima, where they proliferated causing DIT. It was concluded that DIT is a consistent component of arteriosclerotic lesions in N. veitchii induced calcinosis of sheep and that the predominant cell in this process is the SMCs originated from its predecessors of the media. It is suggested that the inducing factor for the arterial changes is 1,25(OH)2 D3 present in N. veitchii.

Arterial diffuse intimal thickening associated with enzootic calcinosis of sheep

Foram feitos estudos morfométrico, imunoistoquímico e ultra-estrutural do espessamento intimal difuso (DIT) das artérias de 7 ovinos com sinais clínicos de calcinose enzoótica espontânea causada pela ingestão da planta Nierembergia veitchii. As lesões caracterizavam-se por deposição de sais de cálcio na média como placas e estrias que, com frequência faziam saliência para a luz, criando rugosidades e irregularidades da íntima. Não foram observadas calcificações na artéria pulmonar e no sistema venoso. Microscopicamente, as calcificações das artérias restringiam-se quase que exclusivamente à média. Na imunoistoquimica, foi verificada a-actina nas células da média e nas do espessamento intimal. Receptores para 1,25(OH)2D3 foram evidenciados nos núcleos das células musculares da média, da íntima e das células endoteliais. As análises morfométricas em microscopia ótica revelaram, nas artérias, DIT irregularmente distribuido sem relação com a intensidade dos processos de calcificação subjacente nem com a espessura da média remanescente. A morfometria das alterações ultra-estruturais das células musculares lisas da média e da íntima espessada, mostrou que nessas últimas foi verificado aumento de até 318% na fração volumétrica das organelas de síntese em detrimento dos elementos contráteis, quando comparados com os valores das células da média. Essas modificações indicam a transformação das células musculares lisas de um tipo contrátil para células de tipo sintético. Entre essses dois extremos foram evidenciadas diversas formas intermediárias. Foram observadas evidências histológicas e ultra-estruturais da transformação e migração de células da média através das fenestras da lâmina elástica interna, para a íntima, onde proliferam e formam o DIT. Foi concluído que o DIT é um componente constante nas lesões arterioscleróticas nas calcinoses dos ovinos induzidas pela planta N. veitchii e que as células predominantes são células musculares lisas provenientes de predecessoras da média. Foi constatado que o DIT só excepcionalmente apresenta calcificação, vascularização, presença de células espumosas e fendas de colesterol. Não foram vistas áreas de necrose. Sugere-se que o fator indutor das alterações arteriais é o 1,25(OH)2D3 contido em N. veitchii.

A locus on chromosome 7 determines myocardial cell necrosis and calcification (dystrophic cardiac calcinosis) in mice.

Dystrophic cardiac calcinosis, an age-related cardiomyopathy that occurs among certain inbred strains of mice, involves myocardial injury, necrosis, and calcification. Using a complete linkage map approach and quantitative trait locus analysis, we sought to identify genetic loci determining dystrophic cardiac calcinosis in an F2 intercross of resistant C57BL/6J and susceptible C3H/HeJ inbred strains. We identified a single major locus, designated Dyscalc, located on proximal chromosome 7 in a region syntenic with human chromosomes 19q13 and 11p15. The statistical significance of Dyscalc (logarithm of odds score 14.6) was tested by analysis of permuted trait data. Analysis of BxH recombinant inbred strains confirmed the mapping position. The inheritance pattern indicated that this locus influences susceptibility of cells both to enter necrosis and to subsequently undergo calcification.

Extensive Calcinosis in an Adult Nigerian Woman with Dermatomyositis

Objectives: Extensive calcinosis in a patient with dermatomyositis is a rare clinical presentation in adults. The aim of this report is to present the clinical and radiographic features of a 25-year-old woman with dermatomyositis and extensive calcinosis. Case Presentation: A 25-year-old woman with extensive calcinosis on background dermatomyositis is described. Results: The clinical and radiographic features of the patient were consistent with dermatomyositis complicated by extensive calcinosis in an adult. Conclusion: Dermatomyositis with extensive calcinosis is a rare clinical presentation in adults.

Photoletter to the editor: Calcinosis cutis in a burn scar

Calcinosis cutis is a rare condition characterized by the deposition of insoluble calcium salts in the skin and subcutaneous tissue. Dystrophic calcinosis cutis appears as a result of local tissue damage or abnormalities, such as alterations in extra-cellular matrix proteins or subcutaneous tissue with normal calcium and phosphate serum levels. It has been rarely described as a late complication of burns. Latency periods of 15-54 years have been reported. We describe the case of a 57-year-old man with dystrophic calcinosis cutis in a burn scar, which developed 42 years after the skin injury. The condition was successfully treated with surgical excision.

Diagnostic agreement between panoramic radiographs and color doppler images of carotid atheroma

The aim of this study was to investigate the agreement between diagnoses of calcified atheroma seen on panoramic radiographs and color Doppler images. Our interest stems from the fact that panoramic images can show the presence of atheroma regardless of the level of obstruction detected by color Doppler images. Panoramic and color Doppler images of 16 patients obtained from the archives of the Health Department of the city of Valença, RJ, Brazil, were analyzed in this study. Both sides of each patient were observed on the images, with a total of 32 analyzed cervical regions. The level of agreement between diagnoses was analyzed using the Kappa statistics. There was a high level of agreement, with a Kappa value of 0.78. In conclusion, panoramic radiographs can help detecting calcifications in the cervical region of patients susceptible to vascular diseases predisposing to myocardial infarction and cerebrovascular accidents. If properly trained and informed, dentists can refer their patients to a physician for a cardiovascular evaluation in order to receive proper and timely medical treatment.

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

Objective To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry. Methods Inclusion 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. Results Of 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%. Conclusion Evaluation 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.

Clinicopathological evaluation of in vivo epidermal nuclear fluorescence

Nuclear fluorescence in keratinocytes is an occasional phenomenon, often present in autoimmune diseases, especially in connective-tissue disease (CTD); however, its clinical significance remains unclear. To investigate the profile of patients with positive nuclear staining on direct immunofluorescence (DIF) of skin samples. A retrospective analysis of 28 patient records from our immunodermatology laboratory was performed between May 2003 and June 2006. Inclusion criteria were the presence of autoantibodies (IgG, IgA or IgM) or complement (C3) binding keratinocyte nuclei on DIF. The most prevalent diseases related to the nuclear keratinocyte DIF staining were systemic lupus erythematosus (n = 9), mixed CTD (n = 3), overlap syndrome (n = 3), Sjogren`s syndrome (n = 1), and CREST (calcinosis, Raynaud`s phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia) syndrome (n = 1). Serum antinuclear antibody (ANA) was positive in 20 of 28 patients, with titres varying from 1 : 160 to 1 : 1280. Of the 20 patients with positive anti-nuclear antibodies (ANA), 17 were positive for anti-extractable nuclear antigen antibodies, 12 had anti-SSA/Ro, 11 had anti-SSB/La and 8 had anti-ribonucleoprotein. Eight patients were negative for ANA. Positive predictive value of in vivo ANA for systemic CTDs was 75%. The present data suggest that in vivo ANA evaluation is an additional and feasible auxiliary tool for diagnosing CTDs.

Comparison of clinical features and drug therapies among European and Latin American patients with juvenile dermatomyositis

Objective To compare the demographic features, presenting manifestations, diagnostic investigations, disease course, and drug therapies of children with juvenile dermatomyositis (JDM) followed in Europe and Latin America. Methods Patients were inception cohorts seen between 1980 and 2004 in 27 paediatric rheumatology centres. The following information was collected through the review of patient charts: sex; age at disease onset; date of disease onset and diagnosis; onset type; presenting clinical features; diagnostic investigations; course type; and medications received during disease course. Results Four hundred and ninety patients (65.5% females, mean onset age 7.0 years, mean disease duration 7.7 years) were included. Disease presentation was acute or insidious in 57.1% and 42.9% of the patients, respectively. The course type was monophasic in 41.3% of patients and chronic polycyclic or continuous in 58.6% of patients. The more common presenting manifestations were muscle weakness (84.9%), Gottron`s papules (72.9%), heliotrope rash (62%), and malar rash (56.7%). Overall, the demographic and clinical features of the 2 continental cohorts were comparable. European patients received more frequently high-dose intravenous methylprednisolone, cyclosporine, cyclophosphamide, and azathioprine, while methotrexate and antimalarials medications were used more commonly by Latin American physicians. Conclusion The demographic and clinical characteristics of JDM are similar in European and Latin American patients. We found, however, several differences in the use of medications between European and Latin American paediatric rheumatologists.

Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients

Objective. To investigate the long-term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study. Methods. Patients consisted of inception cohorts seen between 1980 and 2004 in 27 centers in Europe and Latin America. Predictor variables were sex, continent, ethnicity, onset year, onset age, onset type, onset manifestations, course type, disease duration, and active disease duration. Outcomes were muscle strength/endurance, continued disease activity, cumulative damage, muscle damage, cutaneous damage, calcinosis, lipodystrophy, physical function, and health-related quality of life (HRQOL). Results. A total of 490 patients with a mean disease duration of 7.7 years were included. At the cross-sectional visit, 41.2-52.8% of patients, depending on the instrument used, had reduced muscle strength/endurance, but less than 10% had severe impairment. Persistently active disease was recorded in 41.2-60.5% of the patients, depending on the activity measure used. Sixty-nine percent of the patients had cumulative damage. The frequency of calcinosis and lipodystrophy was 23.6% and 9.7%, respectively. A total of 40.7% of the patients had decreased functional ability, but only 6.5% had major impairment. Only a small fraction had decreased HRQOL. A chronic course, either polycyclic or continuous, consistently predicted a poorer outcome. Mortality rate was 3.1%. Conclusion. This study confirms the marked improvement in functional outcome of juvenile DM when compared with earlier literature. However, many patients had continued disease activity and cumulative damage at followup. A chronic course was the strongest predictor of poor prognosis. These findings highlight the need for treatment strategies that enable a better control of disease activity over time and the reduction of nonreversible damage.