RESUMO
Inflammatory myopathies are a group of diseases whose common pathway is immune-mediated muscle damage, one of which is polymyositis. The definition of polymyositis is controversial, with proponents advocating a definition based on immunohistochemical and histopathological findings in muscle biopsies, while other proponents advocate a definition based on clinical manifestations and histopathological findings. Polymyositis is a quite rare disease that is clinically characterized by progressive proximal muscle weakness with a symmetric distribution. Within the diagnostic approach, laboratory studies show elevation of sarcoplasmic enzymes; nerve conduction tests are performed, which may aid in distinguishing myopathic causes of weakness from neuropathic disorders; and muscle biopsy is considered the gold standard to diagnose inflammatory myopathy and to distinguish the subclasses. We report the case of a 61-year-old male patient who presented generalized symmetrical weakness, predominantly in the upper extremities, and dysphagia, whose laboratory studies, autoantibodies, and muscle biopsy were confirmatory of this entity.
RESUMO
Resumen ANTECEDENTES: La enfermedad de Paget mamaria es una alteración poco frecuente, caracterizada por cambios en el área del pezón-areola, que puede asociarse con carcinoma de mama in situ o invasor. CASO CLÍNICO: Paciente de 69 años, con lesión mamaria de tres años de evolución, cambios en la coloración del pezón y descamación, acompañados de prurito, de aspecto eritematoso y escamoso en el área del pezón-areola izquierda, con retracción del pezón; se obtuvo una biopsia del área del pezón-areola. El estudio de patología reportó células neoplásicas grandes, con infiltración a la epidermis, aisladas o en pequeños grupos, con citoplasma claro, vacuolado, núcleo agrandado y nucléolo prominente, característico de la enfermedad de Paget mamaria. El estudio de inmunohistoquímica reportó positividad para CK7 y HER2, y negatividad para p53 a células neoplásicas, con lo que se estableció el diagnóstico definitivo de enfermedad de Paget mamaria. La mastografía no mostró alteraciones y la mastectomía reportó carcinoma intraductal. CONCLUSIÓN: El conocimiento de la enfermedad de Paget mamaria tiene importancia clínica, puesto que representa una afección poco frecuente, que debe considerarse en el diagnóstico diferencial de lesiones benignas del complejo areola-pezón.
Abstract BACKGROUND: The Paget´s disease mammary is an uncommon malignancy that presents with changes in the nipple-areolar region that may be associated with an underlying breast cancer. CLINICAL CASE: A 69-year-old patient with a breast lesion of three years of evolution, changes in nipple, characterized by coloration and scaling, pruritus, with an erythematous and scaly appearance in the nipple-areola left region, and nipple retraction. A biopsy of the nipple-areola was obtained. The Pathology service reported large neoplastic cells, with infiltration to the epidermis, isolated or in small groups, with clear cytoplasm, vacuolated, enlarged nucleus and prominent nucleolus, characteristic of Paget's disease mammary. The immunohistochemistry study reported positivity for CK7 and HER2, and negativity for p53 to neoplastic cells, which established the definitive diagnosis of Paget's disease of the breast. The mammography was normal and the mastectomy reported intraductal carcinoma. CONCLUSION: The knowledge of mammary Paget's disease is of clinical importance, since it represents a rare condition, which must be considered in the differential diagnosis of benign lesions of the areola-nipple complex.
RESUMO
We report the case of a 44-year-old, who was resected of a vulvar tumor. The pathological report was leiomyoma with focal myxoid degeneration, which is an infrequent benign tumor.
