Calcinosis in juvenile dermatomyositis, a therapeutic challenge

Calcinosis is a common and debilitating complication of dermatomyositis. It is a hallmark of the disease, occurring mainly in pediatric patients. Little is known about its pathophysiology, and there is no universally recognized treatment. This is a report of two children with juvenile dermatomyositis and severe calcinosis who showed improvement with the use of alendronate and diltiazen in one patient, and intravenous immunoglobulin in the other.A calcinose é uma complicação comum e incapacitante da dermatomiosite. Ela constitui uma característica marcante da doença, ocorrendo principalmente em pacientes pediátricos. Pouco se sabe a respeito de sua fisiopatologia e não existe um tratamento reconhecido universalmente. Este trabalho relata dois casos de dermatomiosite juvenil e calcinose grave com melhora clínica com o uso do alendronato e do diltiazen em um paciente e da imunoglobulina endovenosa em outro.UNIFESP-EPM Departamento de PediatriaUNIFESP, EPM, Depto. de PediatriaSciEL

Risk factors associated with calcinosis of juvenile dermatomyositis

OBJETIVO: Identificar fatores de risco associados à calcinose em crianças e adolescentes com dermatomiosite juvenil. MÉTODOS: Prontuários de 54 pacientes com dermatomiosite juvenil foram estudados. Foram avaliados dados demográficos; características clínicas: grau de força muscular (I a V do Medical Research Council), presença de comprometimentos pulmonar (distúrbio ventilatório restritivo com presença ou ausência do anticorpo anti-Jo-1), gastrointestinal (refluxo gastroesofágico) e cardíaco (pericardite e/ou miocardite); exames laboratoriais: elevação de enzimas musculares (creatinoquinase, aspartato aminotransferase, alanina aminotransferase e desidrogenase lática) e terapias utilizadas: corticoterapia isolada ou associada à cloroquina e/ou imunossupressor. Os pacientes foram divididos em dois grupos de acordo com a presença ou ausência de calcinose e foram avaliados através de análise univariada e multivariada. RESULTADOS: Calcinose foi evidenciada em 23 (43%) pacientes, sendo em seis (26%) antes do diagnóstico e em 17 (74%) após. A análise univariada revelou que comprometimentos cardíaco (p = 0,01) e pulmonar (p = 0,02) e necessidade da utilização de um ou mais imunossupressores (metotrexato, ciclosporina A e/ou pulsoterapia com ciclofosfamida endovenosa) no tratamento da dermatomiosite juvenil (p = 0,03) foram associados com uma maior incidência de calcinose. A análise multivariada mostrou que comprometimento cardíaco (OR = 15,56; IC95% 1,59-152,2) e uso de um ou mais imunossupressores (OR = 4,01; IC95% 1,08-14,87) foram as únicas variáveis independentes associadas à presença de calcinose. CONCLUSÕES: O aparecimento da calcinose foi freqüente na dermatomiosite juvenil, habitualmente na evolução da doença. A calcinose foi associada aos casos mais graves, que apresentaram envolvimento cardíaco e necessitaram da utilização de imunossupressores no seu tratamento.OBJECTIVE: To identify risk factors associated with calcinosis in children and adolescents with juvenile dermatomyositis. METHODS: A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale), pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies), gastrointestinal problems (gastroesophageal reflux) and/or heart disease (pericarditis and/or myocarditis); laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase); and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses. RESULTS: Calcinosis was identified in 23 (43%) patients, and in six (26%) patients it had emerged prior to diagnosis while in 17 (74%) it was post diagnosis. The univariate analysis revealed that cardiac (p = 0.01) and pulmonary (p = 0.02) involvement and the need for one or more immunosuppressor (methotrexate, cyclosporine A and/or pulse therapy with intravenous cyclophosphamide) to treat juvenile dermatomyositis (p = 0.03) were all associated with an increased incidence of calcinosis. The multivariate analysis then demonstrated that only cardiac involvement (OR = 15.56; 95%CI 1.59-152.2) and the use of one or more immunosuppressor (OR = 4.01; 95%CI 1.08-14.87) were independently associated with the presence of calcinosis. CONCLUSIONS: Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment

Dermatomyositis and Breast Calcinosis

The authors report a rare case of dermatomyositis diagnosed at the Mastology Sector of the Division of Gynecology of the Federal University of São Paulo - Escola Paulista de Medicina, which caused breast deformity due to formation of bilateral dystrophic calcifications.Os autores relatam caso raro de dermatomiosite diagnosticado no Setor de Mastologia da Disciplina de Ginecologia da Universidade Federal de São Paulo (UNIFESP) - Escola Paulista de Medicina, causando deformidade mamária devido à formação de calcificações distróficas bilateralmente.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Setor de MastologiaUNIFESP, EPM, Setor de MastologiaSciEL

Calcinose pseudo-tumorale primitive chez l’enfant

La calcinose pseudo-tumorale est une tumeur bénigne relativement rare, elle se caractérise par un dépôt de matériel calcique dans les tissus mous péri-articulaires. Nous rapportons un cas de calcinose tumorale primitive de la hanche gauche découvert chez un enfant de 15 ans avec une revue de la littérature. La patiente a été opérée avec une exérèse totale de la masse, les suites postopératoires étaient simples. 6 mois après l’intervention il n’y a pas de récidive tumorale.Mots clés: Calcinose tumorale primitive, masse du tissu mou, enfantEnglish Title: Primary pseudotumoral calcinosis in childrenEnglish AbstractPseudotumoral calcinosis is a relatively rare benign tumor, characterized by calcium deposition in peri-articular soft tissues. We here report the case of a 15-year old boy with primary tumoral calcinosis of the left hip as well as a literature review. The patient underwent total mass resection. The postoperative course was simple. The patient showed no recurrence at 6-month follow-up.Keywords: Primary tumoral calcinosis, soft tissue mass, chil

Calcinose rebelle avec préjudice fonctionnel et esthétique

Les calcinoses cutanées sont un groupe de maladies dans lesquelles des dépôts calciques se forment dans la peau. Elles peuvent être localisées ou diffuses, avec un retentissement fonctionnel, et peuvent altérer la qualité de vie. Le traitement des formes diffuses ne fait pas consensus et reste souvent décevant. Nous rapportons un nouveau cas. Il s'agissait d'un jeune homme âgé de 18 ans, aux antécédents d'une dermatomyosite ayant survenu à l'âge de 5 ans, en rémission, sans traitement, depuis l'âge de 8 ans. Après un intervalle libre de 6 ans post guérison de sa dermatomyosite, le patient avait présenté des zones d'induration cutanée peu douloureuses. Elles étaient observées pour la première fois au niveau des coudes, des poignets et des mains. Ensuite, il y avait une apparition d'autres lésions au niveau de la face, avec préjudice esthétique. L'examen trouvait des lésions à type de papules, de plaques et de nodules infiltrés, durs à la palpation, de couleur blancjaunâtre. Les radiographies des membres avaient montrées des images radio-opaques, de tonalité calcique. Une biopsie de l'une de ces lésions, avait confirmé le diagnostic de calcinose cutanée. L'évolution spontanée était marquée par la survenue de plusieurs épisodes d'extrusion d'un matériel crayeux, blanchâtre, plus ou moins liquide à travers des ulcérations cutanées. Une corticothérapie orale associée à la colchicine a été administrée durant une année, mais cela n'avait pas empêché l'apparition de nouvelles calcifications. Le patient est actuellement traité par colchicine et inhibiteurs calciques, avec persistance des lésions de calcinose, mais sans aggravation récente.Pan African Medical Journal 2015; 2

Calcinose tumorale chez un hémodialysé

Tumoral calcinosis is a rare benign condition, characterized by calcium deposition in the soft tissue in extra-articular location which progresses toward a tumoral form. It can be primary or secondary to chronic renal insufficiency. We report a new case of tumoral calcinosis in a 27 years old young man. This patient with renal failure secondary to a malformative uropathy over a period of 15 years, was treated initially with peritoneal dialysis and hemodialysis. The patient presented since 5 months a gradual swelling of the hip causing difficulty walking. Examination revealed a stony consistency infiltration. There was no abnormal skin lesion. Pelvic radiograph highlighted significant diffuse calcium deposits in the Ssubcutaneous tissue. Bone scan showed increased uptake of the hip. MRI showed tumoral calcinosis of the posterior surface of the root of the thigh, measuring 14x13x12 cm, well limited, heterogeneous with fine peripheral and septal contrast, located at the level of large gluteal muscle, of the adductor magnus muscle and taking the proximal part of the conjoint tendon of the biceps femoris and the semitendinosus. This mass came into contact with the sciatic nerve without affecting the back. Laboratory tests found calcemy at 2,33 mmol/L, phosphoremy at 3,1 mmol/L, parathyroid hormone (PTH) at 770 ng/L and creatinine at 977 mol/L. The patient was offered a surgical procedure but he refused it. Pan African Medical Journal 2016; 2

Calcinose tumoral cervical em cão

Calcinose circunscrita ou calcinose tumoral é uma mineralização ectópica que apresenta-se na forma de depósito de cálcio em tecidos moles e pode manifestar-se em diversas espécies. Alguns autores recomendam usar o termo calcinose tumoral quando a deposição dos sais de cálcio ocorre no tecido periarticular e calcinose circunscrita quando localizada no tecido subcutâneo. Pode ocorrer na coluna vertebral e levar à compressão da medula espinhal. O quadro neurológico, quando se instala é progressivo e provoca distúrbios de leves a moderados. Com exceção da calcinose metastática, o tratamento indicado é a remoção cirúrgica, com baixo índice de recidiva da lesão e redução e/ou resolução das lesões neurológicas. O trabalho teve o objetivo de relatar um caso de compressão de medula espinhal cervical causada por uma calcinose tumoral entre o atlas e o axis em um cão Malamute do Alaska de oito meses de idade, o qual foi submetido a procedimento cirúrgico para remoção do tecido alterado. As alterações histológicas classificaram a massa como uma calcinose tumoral. Devido ao grau de compressão na medula, natureza progressiva da doença e prognóstico favorável, foi feita a opção pela remoção cirúrgica do tumor. Neste caso, a calcinose tumoral determinou compressão da medula espinhal com sinais neurológicos de dor e deficiência funcional leve, assimétrica, da locomoção e de reações posturais

Dermatomyositis

Dermatomiosite é doença idiopática inflamatória crônica que afeta a musculatura estriada, a pele e outros órgãos. Apresenta critérios diagnósticos definidos por Bohan & Peter, podendo os pacientes ser classificados em cinco grupos: dermatomiosite juvenil, dermatomiosite primária idiopática, dermatomiosites amiopáticas, dermatomiosite associada a neoplasias e dermatomiosite associada a outras doenças do tecido conectivo. O sexo feminino é mais afetado, e a idade média do diagnóstico é 40 anos. Manifestações cutâneas são observadas em todos os pacientes. Das alterações sistêmicas, a manifestação muscular mais freqüente é a perda de força proximal, e a manifestação pulmonar mais comum é a pneumopatia intersticial. Podem ser observadas neoplasias durante o seguimento da doença, sendo mais freqüentes nos pacientes acima de 60 anos. A desidrogenase lática é a enzima muscular alterada na maioria dos casos. Para diagnóstico da dermatomiosite, pode ser realizado exame anatomopatológico de biópsia cutânea e biópsia muscular, além de eletroneuromiografia. Os corticóides são a terapia mais utilizada. As causas de óbito mais freqüentes são a neoplasia maligna, a septicemia e a infecção pulmonar.Dermatomyositis is a chronic idiopathic inflammatory disorder that affects striated skeletal muscles, the skin, and other organs. Diagnostic criteria were established by Bohan & Peter and patients may be classified into five groups: juvenile dermatomyositis, primary dermatomyositis, amyopatic dermatomyositis, dermatomyositis associated with malignancies and dermatomyositis associated with other connective tissue disorders. Females are more affected and the mean age of diagnosis is 40 years. Skin manifestations are observed in all patients. Loss of proximal strength is the most common systemic alteration and lung involvement is most often manifested as interstitial pneumopathy. Neoplasms may be detected during the course of the disease specially in patients over 60. Lactic dehydrogenase serum levels are altered in the majority of cases and diagnosis can be established or the basis of skin and muscle biopsies and electroneuromiography. Corticosteroids are the first line drugs. The most common causes of death are malignant neoplasms, sepsis and pulmonary infection

Renal Abnormalities During Calcinosis Primary Tumor

Primary tumour calcinosis is a rare condition of unknown etiopathogeny. They are transmitted in an autosomal dominant or recessive mode and predominate in the black race. Calcinoses are manifested by hydroxyapathite deposits in the dermis or hypodermis. The authors report an observation of primary tumour calcinosis associated with renal abnormalities and staturoweight retardation in an 11-year-old child

Hypertrophie bilatérale du canal du Wharton révélatrice d’un Syndrome de Gougerot Sjögren

Le canal de Wharton draine les secrétions de la glande sousmaxillaire. Les étiologies de la dilatation de ce canal sont nombreuses: mégacanaux salivaires idiopathiques, lithiase salivaire, dilatation canalaire résultant d'une sténose tumorale ou traumatique et calcinose salivaire. Cette dernière est due à la présence de multiples concrétions parenchymateuses bilatérales dans un groupe de glandes. Il existerait une relation entre calcinose et Syndrome de Gougerot Sjögren (SGS). Patiente de 55 ans sans antécédents pathologiques notables était hospitalisée pour exploration d'une hypertrophie du canal de Wharton et une sécheresse buccale et oculaire évoluant depuis un an. L'examen somatique notait une hypertrophie des glandes sous maxillaires, une hypertrophie des canaux de Wharton surtout à droite sans calcul palpable sur leur trajet et l'orifice de Wharton était libre. Le reste de l'examen était sans particularités. La scintigraphie salivaire montrait une perfusion et une captation faible des glandes parotides et sous maxillaires, cet aspect est en faveur d'un syndrome sec. L'échographie cervicale était sans anomalies. Le bilan immunologique (anticorps antinucléaires, anti SSA, anti SSB, facteur rhumatoïde) était positif. Le «Break up time» (BUT) était altéré à 3'' en faveur d'une xérophtalmie. La biopsie des glandes salivaires accessoires objectivait une sialadénite stade III de Chisholm confirmant le diagnostic du SGS et la patiente a été mise sous traitement symptomatique.Pan African Medical Journal 2015; 2