Dermatomiositis asociada con carcinoma hepatocelular relacionado con el virus de la hepatitis B: un reporte de caso en Perú / Dermatomyositis associated with hepatitis B virus-related hepatocellular carcinoma: a case report in Peru

La dermatomiositis (DM) es una miopatía inflamatoria de causa desconocida caracterizada por inflamación muscular, debilidad músculo-esquelética proximal y manifestaciones cutáneas típicas. Se ha asociado a malignidades como un síndrome paraneoplásico. Reportamos el caso de un paciente varón de 33 años, diagnosticado de hepatitis B, VHB crónico inactivo, que presentó lesiones papulares, pruriginosas y descamativas en cara, manos, zona inguinal y pies. Al examen físico se evidenció pápulas de Gottron, signo del heliotropo, debilidad muscular simétrica proximal. Se realizó una biopsia de piel donde se encontraron hallazgos compatibles con DM. Tras una ecografía abdominal se encontró una tumoración hepática, cuyo resultado en biopsia fue de carcinoma hepatocelular moderadamente diferenciado. Posteriormente se le realiza segmentectomía con lo cual síntomas de DM disminuyen. Es un caso infrecuente, y de sumo interés por lo que se decide reportar.

Dermatomyositis is an idiopathic inflammatory myopathie characterized by proximal skeletal muscle weakness, typical skin manifestations and muscle inflammation. This disease has been associated with malignancies as a paraneoplastic syndrome. We present a patient of thirty-three years diagnosed with hepatitis B, chronic inactive HBV who presents papular, pruritic and desquamative lesions on the face, hands, inguinal area and feet. At the physical examination is evidentiated Gottron's papules, heliotrope sign and proximal symmetric muscular weakness. Findings compatible with DM were found in a skin biopsy. An abdominal ultrasound revealed a liver tumor whereby a biopsy was performed and the result was a moderately differentiated hepatocellular carcinoma. Subsequently, a segmentectomy has been made and consequently the DM symptoms decreased. This case is of great interest and rare reason why we decided to reported it.

Miopatías inflamatorias idiopáticas: una mirada actualizada al diagnóstico y el manejo / Idiopathic inflammatory myopathies: a review

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired immune-mediated diseases, which typically involve the striated muscle with a variable involvement of the skin and other organs. Clinically, they are characterized by proximal muscle weakness, elevation of muscle enzymes, myopathic changes on electromyography and an abnormal muscle biopsy. The different IIM have been classified according to their distinctive histopathologic features in dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM). Several myositis-specific antibodies are associated with the different phenotypes, as well as with different risk of neoplastic disease and systemic complications. The basis for the treatment of DM, PM, and IMNM is immunosuppression. For IBM there are only symptomatic treatments. Steroids, associated or not with other immunosuppressant drugs, are the first line of treatment. Biologic drugs will allow future individualized therapies. The 10-year survival of DM, PM and IMNM is 62 to 90%. The leading causes of death are neoplastic, lung and cardiac complications. IBM does not impair survival, although it affects the quality of life.

Dermatomiositis asociada al autoanticuerpo anti-MDA5 / Dermatomyositis associated with anti-MDA5 autoantibody

La dematomiositis es una miopatía inflamatoria idiopática con espectro clínico variable. En los últimos años se ha identificado un número de autoanticuerpos específicos de miositis útiles para el diagnóstico, la clasificación y el pronóstico de las diversas formas de la enfermedad, entre los que se encuentra el anti-MDA5. Este anticuerpo se asocia al desarrollo de úlceras cutáneas, enfermedad intersticial pulmonar rápidamente progresiva, mortalidad temprana y mal pronóstico por lo que la detección del mismo, en un contexto clínico adecuado, plantea la necesidad de un tratamiento inmunosupresor agresivo. Describimos un caso de dermatomiositis hipomiopática, (es decir, con afección muscular leve) que presentaba compromiso cutáneo específico, enfermedad pulmonar intersticial y anticuerpo anti-MDA5 que respondió favorablemente al tratamiento combinado con ciclofosfamida, gamaglobulina y corticoides.

Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is us eful for diagnosis, prognosis and classification of the diverse clinical forms of the disease. This antibody is associated with cutaneous ulcers, rapidly progressive interstitial lung disease, early mortality and poor prognosis, so the detection of this antibody in a suitable clinical context, raises the need for an aggressive immunosuppressive treatment. We describe a case of dermatomyositis classified as hypomyopathic (i.e. involving mild muscle weakness), presenting specific skin lesions, interstitial lung disease, and presence of anti-MDA5 antibody that had a favorable response to combined treatment with cyclophosphamide, gamma globulin and corticosteroids.

Panniculitis associated with amyopathic dermatomyositis

Abstract: Panniculitis is a rare clinical finding in dermatomyositis, with less than 30 cases reported and there is only one case associated with the amyopathic subtype described in the literature. The present report describes a 49-year-old female patient that one year after being diagnosed with amyopathic dermatomyositis, presented indurated, painful, erythematous to violaceous nodules located on the upper limbs, thighs and gluteal region. Skin biopsy revealed lobular panniculitis with a lymphocytic infiltrate. The patient was treated with prednisone and methotrexate, but remained unresponsive to treatment.

Miopatías inflamatorias con evolución recidivante / Relapses in inflammatory myopathies

La mayoría de los estudios de tratamiento de las miopatías inflamatorias son de corte y no permiten establecer su eficacia en largo plazo. En este trabajo, describimos el seguimiento de siete pacientes con miopatías inflamatorias, 5 polimiositis y 2 dermatomiositis. Determinamos su presentación, su seguimiento clínico mediante el examen físico, las enzimas musculares y la respuesta al tratamiento. Esta última la definimos como cursos de tratamiento, donde cada curso termina al aumentar los corticoides o al colocar una nueva medicación inmunosupresora debido al empeoramiento clínico o aumento sostenido de las enzimas musculares. El tratamiento instaurado puede remitir, controlar parcialmente, o fracasar en controlar la enfermedad en cuanto se normalicen, estabilicen, o no modifiquen respectivamente tanto la clínica como las enzimas musculares. Se analizaron 20 ciclos, en 14 se logró la remisión, en cinco se controló parcialmente y en uno fracasó el tratamiento. La remisión se logró en un tiempo promedio de 139 ± 98 días y el control en un promedio de 160 ± 100 días. Excepto en una ocasión, todos los ciclos de tratamiento, independientemente del que fuera, remitieron o controlaron los síntomas, pero en el tiempo todos los pacientes recidivaron en su enfermedad.

Most studies about treatment of inflammatory myopathies consist of cross-sectional analyses that do not assess long-term efficacy. In the present study we describe the follow-up of seven patients with inflammatory myopathies, 5 polymyositis and 2 dermatomyositis. We describe their clinical features, follow-up, muscle enzyme levels, and treatment responses. We define the latter as treatment cycles, every one of which end when steroid doses need to be increased or a new immunosuppressive drug has to be added because of clinical worsening or sustained increases in muscle enzyme levels. Treatment can cause remission, partially control, or fail in achieving myositis improvement when it normalizes, stabilizes, or does not affect muscle enzymes or clinical features, respectively. We analyzed 20 cycles, in which remission was achieved in 14 cases, partial control in 5 instances, and treatment failure in one case. Remission occurred after an average of 139 ± 98 days, whereas partial control took place in 160 ± 100 days. Except in one case, all treatment cycles controlled or remitted the symptoms. However, in all patients the illness recurred with time.

Autoantibodies in adult patients with idiopathic inflammatory myopathies in Buenos Aires

The idiopathic inflammatory myopathies(IIM) are a heterogeneous group of diseases of the skeletal muscle. On the basis of clinical, serologic and histological differences, they are classified in dermatomyositis (DM), polymyositis (PM), inclusion body myositis and immunomediated necrotizing myopathy. Autoantibodies directed against nuclear and cytoplasmic antigens are present with variable frequencies among studies. Myositis-specific antibodies (MSAs) are useful in IIM because they contribute to the diagnosis, help to identify different clinical subsets, and have prognostic value. This study aimed to explore the frequency of autoantibodies, especially MSAs, and their relationship with clinical features in adult patients with DM, PM and overlap syndrome. Medical records were reviewed. Myositis-associated antibodies (non-specific) and MSAs (anti Jo-1, PL-7, PL-12, Mi-2 and SRP) were measured using commercial kits. Twelve patients had MSAs, an overall frequency similar to those of international series, but PL-12 and Mi-2 were more frequent than Jo-1, which is the most frequently observed elsewhere. All five patients with Mi-2 had classical DM with a favorable response to treatment. Interstitial pneumonia (n: 4) and/or treatment-refractory disease (n: 3) were found in the presence of anti-PL-12, alone or associated with anti-SRP and/or Jo-1. In conclusion, the coexistence of AEM, a rare finding, was found in three patients. The presence of MSAs aided to the diagnosis of IIM, in particular in those patients without available or conclusive biopsy results.

Las miopatías inflamatorias idiopáticas (MII) comprenden un grupo heterogéneo de enfermedades adquiridas del músculo esquelético. Según sus características clínicas, serológicas e histológicas se las clasifica en dermatomiositis (DM), polimiositis (PM), miopatía necrotizante autoinmune y miositis por cuerpos de inclusión. Los anticuerpos específicos de miositis (AEMs) contribuyen al diagnóstico, permiten distinguir formas clínicas y tienen valor pronóstico. Con el objetivo de explorar la frecuencia de autoanticuerpos, en particular AEMs, y su relación con las características clínicas de las MII del adulto, se revisaron las historias clínicas de 25 pacientes con DM, PM y síndromes de superposición, asistidos en nuestro centro entre 1999 y 2013. La presencia de autoanticuerpos asociados a miositis (no específicos) y AEMs (anti Jo-1, PL-7, PL-12, Mi-2, SRP) se investigó utilizando kits comerciales. Doce pacientes presentaron AEMs, frecuencia global similar a la encontrada en series internacionales, pero a diferencia de lo observado en otros países, anti-PL-12 y anti-Mi-2 fueron más frecuentes que anti-Jo-1. Los cinco pacientes con anti-Mi-2 tuvieron DM clásica y buena evolución clínica. Anti-PL-12, ya sea solo o asociado a anti-SRP y/o anti-Jo-1, estuvo presente en pacientes con neumonía intersticial (n:4) y/o enfermedad refractaria al tratamiento (n: 3). En conclusión, la coexistencia de AEM, hallazgo raro, se encontró en tres pacientes. La presencia de AEMSs contribuyó al diagnóstico de MII, en particular en aquellos casos sin resultados concluyentes de biopsia de músculo.

Dermatomiositis asociada a malignidad / Dermatomyositis associated with malignancy

La dermatomiositis es una enfermedad sistémica que se caracteriza fundamentalmente por la presencia de alteraciones inflamatorias de piel y músculo estriado. En ciertos casos constituye un síndrome paraneoplásico, por lo que su diagnóstico obliga a una exhaustiva búsqueda de la probable asociación con un cáncer. Presentamos tres casos de dermatomiositis asociados a neoplasias, dos mujeres con cáncer ginecológico y un varón con cáncer de estómago. Una de las mujeres también presentaba un síndrome mielodisplásico. En dos casos la dermatomiositis fue posterior al diagnóstico del cáncer y en uno los hallazgos fueron simultáneos.

Dermatomyositis is a systemic disease characterized primarily by the presence of inflammatory skin disorders and striated muscle. In some cases it constitutes a paraneoplastic syndrome, so diagnosis requires a thorough search of the likely association with cancer. We present three cases of dermatomyositis associated with malignancies, two women with gynecologic cancer and a man with stomach cancer. One of the women also had a myelodysplastic syndrome. In two cases dermatomyositis was after the diagnosis of cancer and in one, the findings were simultaneous.

Skeletal muscle major histocompatibility complex class I and II expression differences in adult and juvenile dermatomyositis

OBJECTIVE: To analyze major histocompatibility complex expression in the muscle fibers of juvenile and adult dermatomyositis. METHOD: In total, 28 untreated adult dermatomyositis patients, 28 juvenile dermatomyositis patients (Bohan and Peter's criteria) and a control group consisting of four dystrophic and five Pompe's disease patients were analyzed. Routine histological and immunohistochemical (major histocompatibility complex I and II, StreptoABComplex/HRP, Dakopatts) analyses were performed on serial frozen muscle sections. Inflammatory cells, fiber damage, perifascicular atrophy and increased connective tissue were analyzed relative to the expression of major histocompatibility complexes I and II, which were assessed as negatively or positively stained fibers in 10 fields (200X). RESULTS: The mean ages at disease onset were 42.0±15.9 and 7.3±3.4 years in adult and juvenile dermatomyositis, respectively, and the symptom durations before muscle biopsy were similar in both groups. No significant differences were observed regarding gender, ethnicity and frequency of organ involvement, except for higher creatine kinase and lactate dehydrogenase levels in adult dermatomyositis (p<0.050). Moreover, a significantly higher frequency of major histocompatibility complex I (96.4% vs. 50.0%, p<0.001) compared with major histocompatibility complex II expression (14.3% vs. 53.6%, p=0.004) was observed in juvenile dermatomyositis. Fiber damage (p=0.006) and increased connective tissue (p<0.001) were significantly higher in adult dermatomyositis compared with the presence of perifascicular atrophy (p<0.001). The results of the histochemical and histological data did not correlate with the demographic data or with the clinical and laboratory features. CONCLUSION: The overexpression of major histocompatibility complex I was an important finding for the diagnosis of both groups, particularly for juvenile dermatomyositis, whereas there was lower levels of expression of major histocompatibility complex II than major histocompatibility complex I. This finding was particularly apparent in juvenile dermatomyositis.

Dermatomiosite na infância / Juvenile dermatomyositis

A dermatomiosite juvenil é uma doença autoimune que acomete crianças e adolescentes entre 2 e 17 anos de idade, com média de 7 anos ao diagnóstico. É caracterizada por vasculopatia sistêmica e suas manifestações principais são fraqueza muscular proximal simétrica, elevação de enzimas musculares séricas e lesões cutâneas, sendo que o heliótropo e as pápulas de Gottron são patognomônicas. Sua identificação precoce e instituição rápida de tratamento adequado podem prevenir o aparecimento de calcinose e possibilitam melhor prognóstico e qualidade de vida ao paciente. Embora a base medicamentosa da terapia seja o uso de glicocorticoide, o metotrexato, a ciclosporina, a azatioprina e a ciclofosfamida, dependendo da gravidade, são os imunossupressores mais frequentemente utilizados. Imunoglobulina endovenosa pode ser útil nos casos graves e atualmente o uso de imunobiológicos representa uma nova perspectiva para os casos refratários.

Paniculitis en paciente con dermatomiositis / Panniculitis in a patient with dermatomyositis

La paniculitis es una manifestación cutánea infrecuente de la dermatomiositis. Puede preceder hasta en 14 meses a otras manifestaciones de la dermatomiositis. En todos los casos, la miositis y la paniculitis presentan mejoría simultánea durante el tratamiento. Describimos una paciente de sexo femenino de 30 años que presenta lesiones clínica e histológicamente compatibles con paniculitis luego de 2 meses de que el compromiso muscular y cutáneo permitió el diagnóstico de dermatomiositis. Las lesiones cutáneas remitieron con el tratamiento esteroideo.

Panniculitis is a rarely reported clinical finding in dermatomyositis. It may precede the other manifestations associated with dermatomyositis by as much as 14 months. In all cases, myositis and panniculitis improve simultaneously during treatment. The present report describes the case of a 30-year-old female patient with clinical and histopathological findings consistent with panniculitis two months after the onset of the muscle and cutaneous symptoms that permitted diagnosis of dermatomyositis. The skin lesions regressed following steroid treatment.

Atualização em dermatomiosite / Updating in dermatomyositis

JUSTIFICATIVA E OBJETIVOS: A dermatomiosite é umadoença sistêmica crônica, de etiologia desconhecida, que se caracterizapor acometimento inflamatório da pele e dos músculos.O diagnóstico baseia-se nos achados clínicos e laboratoriais. Os corticoides são a terapia mais utilizada. As causas de óbito maisfrequentes são neoplasia maligna, septicemia e fibrose pulmonar.O objetivo deste estudo foi efetuar uma ampla revisão de literatura com foco no reconhecimento dos principais achados clínicos e no tratamento desta doença.CONTEÚDO: A dermatomiosite é uma doença sistêmica crônica que se caracteriza por acometimento inflamatório da pele e dos músculos. Possui duas formas principais: miopática, maisfrequente, onde se encontram lesões musculares e cutâneas; e amiopática, somente com lesões cutâneas. O sexo feminino é o 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 frequente 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 frequentes nos pacientes acima de 60 anos. A desidrogenase lática é a enzima muscular alterada na maioria dos casos. O diagnóstico da dermatomiositepode ser realizado por exame anatomopatológico de biópsia muscular,além de eletroneuromiografia. Os corticoides são os mais utilizados. As causas de óbito mais frequentes são neoplasia maligna,septicemia e fibrose pulmonar. Não há causa conhecida. Odiagnóstico baseia-se nos achados clínicos e laboratoriais.CONCLUSÃO: Através da analise dos dados bibliográficos foi possível concluir que a dermatomiosite é uma doença de diagnóstico predominantemente clínico. Os exames laboratoriais e de imagem constituem importantes confirmadores do quadro, mas nunca são identificadores isolados, sendo sempre a clínica, soberana no diagnóstico desta doença.(...)

BACKGROUND AND OBJECTIVES: The dermatomyosits is a systemic chronic disease, of unknown etiology, which is characterized for inflammatory attack of the skin and muscles. The diagnosis is based on clinical and laboratory finds. The corticosteroids are the most used therapy. The most frequent causes of death are: the malignant neoplasia, the septicemia and the pulmonary fibrosis. A bibliographical revision was carried out, with the objective of attracting attention of the doctors for the recognition of the principal clinical finds of this illness. CONTENTS: The dermatomyositis is a systemic chronic disease that is characterized by the inflammatory attack of the skin and muscles. It has two principal forms: miopatic that is more frequent, and presents muscular and skin injuries; and the amiopatic that presents skin injuries only. The feminine sex is more affected mainly at the middle age of 40s. Skin manifestations are observed in all the patients. Among the systemic alterations,the most frequent muscular demonstration is the loss of strength proximal, and the commonest pulmonary demonstration is interstitial pneumopathy. Neoplasis can be observed during the continuation of the disease, and is more frequent in the patients above 60 years old. The Lactic dehydrogenase means that the muscular enzyme is altered in most of the cases. The diagnosis of the dermatomyositis can be done for the muscular biopsy applyinganatomopatologic exam, besides electroneuromiography examination. The corticosteroids are the most used therapy. The most frequent causes of death are the malignant neoplasis, sepsis, and the pulmonary fibrosis and there is no known cause. The diagnosis is based on the clinical and laboratories finds.CONCLUSION: Through the analyzes of the bibliographical facts it is possible to conclude that dermatomiositis is a disease of predominantly clinical diagnosis.(...)

Dermatomiositis paraneoplásica asociada a carcinoma de células pequeñas de pulmón: a propósito de un caso / Paraneoplastic dermatomysitis associated with small cell lung carcinoma: case report

La dermatomiositis es una rara enfermedad autoinmune que afecta principalmente músculos estriado y piel. Su causa es habitualmente desconocida pero en algunas oportunidades puede estar asociada a diversas neoplasias internas. Presentamos un paciente con dermatomiositis asociada a carcinoma de células pequeñas de pulmón.

Association of anti-glomerular basement membrane antibody disease with dermatomyositis and psoriasis: case report / Associação de doença do anticorpo anti-membrana basal glomerular com dermatomiosite e psoríase: relato de caso

CONTEXT: Anti-glomerular basement membrane (anti-GBM) antibody syndrome is characterized by deposition of anti-GBM antibodies on affected tissues, associated with glomerulonephritis and/or pulmonary involvement. This syndrome has been described in association with other autoimmune disorders, but as far as we know, it has not been described in association with dermatomyositis and psoriasis. CASE REPORT: A 51-year-old man with a history of dermatomyositis and vulgar psoriasis presented with a condition of sensitive-motor polyneuropathy of the hands and feet, weight loss of 4 kg, malaise and fever. On admission, he had been making chronic use of cyclosporin and antihypertensive drugs for three months because of mild arterial hypertension. Laboratory tests showed anemia and leukocytosis, elevated serum urea and creatinine and urine presenting proteinuria, hematuria, leukocyturia and granular casts. The 24-hour proteinuria was 2.3 g. Renal biopsy showed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) deposits on the glomerular basement membrane by means of direct immunofluorescence, which were suggestive of anti-GBM antibodies. The patient was then treated initially with methylprednisolone and with monthly cyclophosphamide in the form of pulse therapy.

CONTEXTO: A síndrome do anticorpo anti-membrana basal glomerular (anti-MBG) é caracterizada pela deposição de anticorpos anti-MBG em tecidos afetados, associada à glomerulonefrite e/ou ao envolvimento pulmonar. Essa síndrome já foi descrita em associação a outras doenças autoimunes, mas até onde conhecemos, não há relatos de sua associação com dermatomiosite e psoríase. RELATO DE CASO: Um homem de 51 anos com antecedentes de dermatomiosite e psoríase vulgar apresentou quadro de polineuropatia sensitivo-motora de mãos e pés, perda de 4 kg, adinamia e febre. À admissão estava em uso crônico de ciclosporina e de anti-hipertensivos há três meses devido a hipertensão arterial leve. Exames laboratoriais mostraram anemia e leucocitose, creatinina e ureia séricas elevadas e urina com proteinúria, hematúria, leucocitúria e cilindros granulosos. A proteinúria de 24 horas foi de 2,3 g. A biópsia renal revelou uma glomerulonefrite crescêntica necrotizante com depósitos lineares de imunoglobulina G (IgG) na MBG à imunofluorescência, sugestivos de anticorpos anti-MBG. O paciente foi então tratado inicialmente com metilprednisolona e com ciclofosfamida mensalmente na forma de pulsoterapia.

Dermatomiositis y neoplasias / Dermatomyositis and malignancy

La dermatomiositis es una miopatía inflamatoria infrecuente que se asocia a múltiples neoplasias, siendo las más frecuentes el cáncer de mama, ovario y pulmón. Los factores de riesgo más importantes para el desarrollo de neoplasia son el sexo masculino, la edad avanzada, la presencia de vasculitis/necrosis cutánea y la ausencia de anticuerpos específicos de miositis. La historia clínica y el examen físico parecen ser las mejores herramientas para diagnosticar las neoplasias; sin embargo, la tomografía computarizada de tórax, abdomen y pelvis representa el examen de elección si no existen hallazgos orientadores de una neoplasia específica. En el presente artículo se analizan la evidencia respecto a la asociación de dermatomiositis y cáncer, los factores de riesgo y las estrategias necesarias para descartar malignidad en estos pacientes.

Dermatomyositis is a rare inflammatory myopathy associated with multiple neoplasias, the most frequent are breast, ovary and lung cancer. The most important risk factors are male gender, old age, the presence of cutaneous vasculitis/necrosis, and the absence of myositis specific antibodies. Clinical history and a physical examination seem to be the best tools to diagnose neoplasias; nevertheless, of thorax, abdomen and pelvis computer tomography is the exam of choice when findings do not lead to specific myositis. We present an analysis of the evidence with respect to the association between dermatomyositis and cancer, risk factors and necessary strategies to discard malignancy in these patients.

A study of morphometric and histopathological features of muscle biopsies from patients of myopathy.

The aim of this study is to analyse the morphological pattern of different types of myopathies including morphometric data. The cases were diagnosed as myopathy on the basis of clinical details, EMG findings, serum CK values. Muscle biopsies were performed and hematoxylin & eosin stain and Masson's trichrome stain were done. Muscle fiber diameters were measured using an eye piece micrometer of 100 fibers in each biopsy, these values were plotted and histograms were constructed. From this, mean fiber diameter (MFD), standard Deviation (SD), atrophy factor (AF), hypertrophy factor (HF) and variability coefficient (VC) were calculated. Degree of inflammation was scored semiquantitatively and presence of degenerating fibers, regenerating fibers, perifascicular atrophy, perivascular lymphocytic infiltration and vasculitis were noted. Out of 25 patients, 9 patients of inflammatory myopathy were adults, of the 16 patients of dystrophy 9 patients were adults. Along with weakness of limbs, skin rash was seen in 2 patients of dermatomyositis. Degree of inflammation was more in the patients of inflammatory myopathy than in the patients of dystrophy. Necrotic and regenerating fibers were seen in both groups. Perifascicular atrophy was seen in 1 case of dermatomyositis. Atrophy factor was higher in cases of dystrophy and so was hypertrophy factor. Variability coefficient >250 was found on 90% of dystrophy cases and it was <250 in the cases of inflammatory myopathy. Morphometry provides valuable data, which helps in distinguishing dystrophy from cases of myopathy with inflammation.

Inflammatory myopathies in childhood: correlation between nailfold capillaroscopy findings and clinical and laboratory data

OBJECTIVE: Nailfold capillaroscopy is an important tool for the diagnosis and follow-up of patients with rheumatic diseases, in particular dermatomyositis and scleroderma. A relationship has been observed in adults between improved capillaroscopic findings and reduced disease activity. Our aim was to correlate disease activity (clinical and laboratory data) and nailfold capillaroscopy findings in 18 patients with inflammatory myopathies. METHODS: This prospective study included 13 juvenile dermatomyositis patients (Bohan and Peter criteria) (mean age of 8.8 years) and five patients with overlap syndrome (mean age of 15.7 years). We evaluated disease activity (skin abnormalities and muscle weakness, muscle enzymes and acute phase reactants) and its correlation with nailfold capillaroscopy findings (dilatation of isolated loops, dropout of surrounding vessels and giant capillary loops). We used a microscope with special light and magnification of 10 to 16X. RESULTS: Eighteen patients underwent a total of 26 capillaroscopic examinations, seven of them on two or more occasions (13 were performed during the active disease phase and 13 during remission). Twelve of the 13 examinations performed during the active phase exhibited scleroderma pattern and 8 of the 13 examinations performed during remission were normal. Therefore, in 20 of the 26 examinations clinical and laboratory data and nailfold capillaroscopy findings correlated (p = 0.01). CONCLUSIONS: Nailfold capillaroscopy is a non-invasive examination that offers satisfactory correlation with disease activity and could be a useful tool for the diagnosis and follow-up of inflammatory myopathies.

Dermatomiositis: revisión bibliográfica y descripción de cinco casos clínico / Dermatomyositis: a report of five cases and bibliographic review

La Dermatomiosis es una enfermedades miopáticas inflamatorias que presentan manifestaciones cutáneas características, tales como rash heliotropo, pápulas de Gottron, eritema o poiquilodermia con fotodistribución, hipertrofia cuticular y telangiectasias periungueles. Otras manifestaciones cutáneas pueden estar presente y no son características de la enfermedad, pudiendo estar en otras patologías. Se trata de una patología de baja incidencia en la consultas dermatológica; sin embargo, durante el año 2001 se observó un aumento de la incidencia de los casos de Dermatomiosits diagnosticados en el Servicio de Dermatología del Hospital Clínico de la Universidad de Chile. Por este motivo, se presentan cinco de los casos más estudiados, destacando en dos de ellos su asociación con neoplasia (cáncer de páncreas y adenocarcinoma mamario oculto).

Correlações da expressão de MHC-I e II, C5b-9 e fenotipagem de células inflamatórias em tecido muscular na dermatomiosite juvenil (DMJ) / Correlations of the expression of MHC-I and II, C5b-9 and inflammatory cells phenotyping in juvenile dermatomyositis

37 fragmentos musculares de 35 pacientes com o diagnóstico de DMJ foram estudados com o objetivo de avaliar a expressão de MHC classes I e II, C5b-9 e fenotipagem das células inflamatórias CD4, CD8, CD20 e CD68 em tecido muscular e correlacionar com os principais parâmetros clínicos, laboratoriais, histológicos e terapêuticos desta doença. Os achados foram comparados à expressão em oito casos de polimiosite (PM), cinco de dermatomiosite (DM) e quatro de / Thirty seven muscle biopsy specimens from patients with JDM were analyzed in order to assess the expression of MHC-I and II, C5b-9, CD4, CD8, CD20 and CD68 and to correlate with the clinical, laboratorial, histological and therapeutical parameters. These findings were compared to the expression in five dermatomyositis (DM), eight polymyositis (PM) and four dystrophy cases...