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      Dermatomiositis como síndrome paraneoplásico en el contexto de un cáncer de ovario bilateral: reporte de un caso y revisión bibliográfica Translated title: Paraneoplastic dermatomyositis in the context of bilateral ovarian cancer: A case report and a literature review

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          Abstract

          Resumen ANTECEDENTES: La dermatomiositis, como síndrome paraneoplásico, puede asociarse con cáncer de ovario de 9 a 21.4% de las ocasiones. Debido a la afectación muscular que implica, supone un gran ensombrecimiento en la morbilidad de estas pacientes. Para identificar el tumor primario hay que tener un alto nivel de sospecha diagnóstica, lo que supone hacer pruebas complementarias aún no claramente establecidas. CASO CLÍNICO: Paciente de 62 años, sin alergias conocidas, que consultó debido a la aparición de una erupción pruriginosa de 20 días de evolución en la región facial, el cuello, el escote y la cara externa de miembros acompañada de mialgias y cierta astenia. En la exploración se comprobó el exantema maculopapular eritematoso en las regiones corporales principalmente fotoexpuestas y pápulas en la región dorsal de las articulaciones metacarpofalángicas. El estudio anatomopatológico definitivo reportó: carcinoma seroso de alto grado, con patrón sólido multinodular en el ovario izquierdo y carcinoma seroso de alto grado con patrón sólido-papilar multinodular en el ovario derecho; ambos anejos con infiltración paratubárica. CONCLUSIONES: Establecer un correcto y rápido diagnóstico de la dermatomiositis es fundamental debido a su fuerte asociación con neoplasias ocultas; esto hace que ante una paciente con datos de dermatomiositis sea decisivo confirmar el diagnóstico y llevar a cabo un exhaustivo estudio de extensión, sobre todo en pacientes de alto riesgo.

          Translated abstract

          Abstract BACKGROUND: Dermatomyositis, as a paraneoplastic syndrome, can be associated with ovarian cancer 9 to 21.4% of the time. Due to the muscle involvement involved, it is a major overshadowing in the morbidity of these patients. To identify the primary tumor, it is necessary to have a high level of diagnostic suspicion, which means performing complementary tests not yet clearly established. CLINICAL CASE: A 62-year-old patient, with no known allergies, consulted due to the appearance of a pruritic rash of 20 days of evolution in the facial region, neck, neckline and external face of the limbs accompanied by myalgia and some asthenia. Examination revealed erythematous maculopapular exanthema in the mainly photoexposed body regions and papules in the dorsal region of the metacarpophalangeal joints. The definitive anatomopathologic study reported: high-grade serous carcinoma with multinodular solid pattern in the left ovary and high-grade serous carcinoma with multinodular solid-papillary pattern in the right ovary; both adnexae with paratubal infiltration. CONCLUSIONS: Establishing a correct and rapid diagnosis of dermatomyositis is fundamental due to its strong association with occult neoplasms; this makes it decisive to confirm the diagnosis and carry out an exhaustive extension study, especially in high-risk patients, when faced with a patient with data of dermatomyositis.

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          Paraneoplastic syndromes: an approach to diagnosis and treatment.

          Recent medical advances have improved the understanding, diagnosis, and treatment of paraneoplastic syndromes. These disorders arise from tumor secretion of hormones, peptides, or cytokines or from immune cross-reactivity between malignant and normal tissues. Paraneoplastic syndromes may affect diverse organ systems, most notably the endocrine, neurologic, dermatologic, rheumatologic, and hematologic systems. The most commonly associated malignancies include small cell lung cancer, breast cancer, gynecologic tumors, and hematologic malignancies. In some instances, the timely diagnosis of these conditions may lead to detection of an otherwise clinically occult tumor at an early and highly treatable stage. Because paraneoplastic syndromes often cause considerable morbidity, effective treatment can improve patient quality of life, enhance the delivery of cancer therapy, and prolong survival. Treatments include addressing the underlying malignancy, immunosuppression (for neurologic, dermatologic, and rheumatologic paraneoplastic syndromes), and correction of electrolyte and hormonal derangements (for endocrine paraneoplastic syndromes). This review focuses on the diagnosis and treatment of paraneoplastic syndromes, with emphasis on those most frequently encountered clinically. Initial literature searches for this review were conducted using PubMed and the keyword paraneoplastic in conjunction with keywords such as malignancy, SIADH, and limbic encephalitis, depending on the particular topic. Date limitations typically were not used, but preference was given to recent articles when possible.
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            Polymyositis and dermatomyositis.

            The inflammatory myopathies, commonly described as idiopathic, are the largest group of acquired and potentially treatable myopathies. On the basis of unique clinical, histopathological, immunological, and demographic features, they can be differentiated into three major and distinct subsets: dermatomyositis, polymyositis, and inclusion-body myositis. Use of new diagnostic criteria is essential to discriminate between them and to exclude other disorders. Dermatomyositis is a microangiopathy affecting skin and muscle; activation and deposition of complement causes lysis of endomysial capillaries and muscle ischaemia. In polymyositis and inclusion-body myositis, clonally expanded CD8-positive cytotoxic T cells invade muscle fibres that express MHC class I antigens, which leads to fibre necrosis via the perforin pathway. In inclusion-body myositis, vacuolar formation with amyloid deposits coexists with the immunological features. The causative autoantigen has not yet been identified. Upregulated vascular-cell adhesion molecule, intercellular adhesion molecule, chemokines, and their receptors promote T-cell transgression, and various cytokines increase the immunopathological process. Early initiation of therapy is essential, since both polymyositis and dermatomyositis respond to immunotherapeutic agents. New immunomodulatory agents currently being tested in controlled trials may prove promising for difficult cases.
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              Cancer risks of dermatomyositis and polymyositis: a nationwide cohort study in Taiwan

              Introduction The association of idiopathic inflammatory myositis (IIM) and malignancies has been reported, but rarely in Asian countries. Our aim was to investigate the risk of cancer among IIM patients without a prior history of malignancies, in Taiwan. Methods We conducted a nationwide cohort study of 1,012 patients with dermatomyositis (DM) and 643 patients with polymyositis (PM), but without prior history of malignancies, utilizing the National Health Insurance Database from 1997 to 2007. Standardized incidence ratios (SIRs) of cancers were analyzed. Results A total of 95 cancers (9.4%) in DM and 33 cancers (4.4%) in PM were identified. Overall cancer risk was significantly elevated in DM patients (SIR = 5.11, 95% confidence interval [CI] = 5.01 to 5.22) and PM patients (SIR = 2.15, 95% CI = 2.08 to 2.22). Most cancers were detected in the first year of observation. The risk of cancer decreased with observation time, yet remained elevated compared with the general population in both study groups after 5 years of follow-up. DM was associated with sustained elevated risk of cancers in every age group, whereas the risk of cancer in PM was highest in younger patients and decreased with age. DM patients were at the greatest risk of cancers of the nasopharynx, lungs and hematopoietic malignancies. Conclusions Patients with IIM are at increased risk for cancer and should receive age-appropriate and gender-appropriate malignancy evaluations, with additional assessment for nasopharyngeal, lung and hematologic malignancy following diagnosis, and with continued vigilance for development of cancers in follow-up.
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                Author and article information

                Journal
                gom
                Ginecología y obstetricia de México
                Ginecol. obstet. Méx.
                Edición y Farmacia S.A. de C.V. (Ciudad de México, Ciudad de México, Mexico )
                0300-9041
                2021
                : 89
                : 7
                : 561-566
                Affiliations
                [2] Sevilla orgnameHospital Universitario Virgen Macarena orgdiv1Servicio de Anatomía Patológica España
                [1] Sevilla orgnameHospital Universitario Virgen Macarena orgdiv1Unidad de gestión clínica de Obstetricia y Ginecología España
                [3] Sevilla orgnameHospital Universitario Virgen Macarena orgdiv1Unidad de gestión clínica de Dermatología España
                Article
                S0300-90412021000700009 S0300-9041(21)08900700009
                10.24245/gom.v89i7.4894
                431e4867-508f-413e-9f2f-89a0275b7422

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : November 2020
                : December 2020
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 13, Pages: 6
                Product

                SciELO Mexico

                Categories
                Casos clínicos

                Allergies,Dermatomiositis,síndrome paraneoplásico,cáncer de ovario,alergias,eritema,mialgia,exantema maculopapular,Dermatomyositis,Paraneoplastic syndrome,Ovarian cancer,Rash,Myalgia,Maculopapular exanthema

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