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Title

IMPORTANCE OF OCCULT CANCER SCREENING IN PATIENTS WITH DERMATOMYOSITIS

Background

Polymyositis (PM), dermatomyositis (DM) and myositis by inclusion bodies (IBM) are the main subtypes of idiopathic inflammatory myopathies, a group of heterogeneous and acquired systemic diseases. DM is an autoimmune disease that mainly affects the skin, muscles and lungs and is strongly associated with an increased risk of cancer.

Case report

A 64-year-old female presented with erythematous and diffuse desquamative cutaneous lesions in the chest (figure 1), face, ear (figure 2) and limbs for a month. She was admitted for investigation. Lab tests revealed elevated creatine kinase (4800 U/L), aldolase (33U/L) and transaminases (ALT 160U/L, AST 270U/L) as well as nuclear fine speckled antinuclear antibody 1/320 (ANA) and positive anti-SSA (Ro). A general examination revealed typical periocular lesions of heliotrope (figure 3) and Gottron’s papules (figure 4). She developed muscle weakness, severe dysphagia and dysphonia during hospitalization. The diagnosis of dermatomyositis was established. She was treated with a pulse of methylprednisolone 1g for three days followed by oral glucocorticoid with decreasing dose, associated with azathioprine 2 mg/kg and hydroxychloroquine 400 mg/day. A chest tomography showed a tree-in-bud pattern, raising the suspicion of tuberculosis that was excluded later by negative bronchoalveolar lavage. She developed outer ear infection and otitis. Due to the severity of the disease and the associated infectious condition, treatment with monthly human immunoglobulin (1g / kg / day for 2 days) was started. A screening for neoplasia was performed with gynecological and thyroid evaluation, upper digestive endoscopy and colonoscopy, computed tomography (CT) of the face, thorax and abdomen. Prominent gastric lymph nodes were seen in CT, the largest adjacent to the small curvature of the stomach, measuring 1.36 x 1.2 cm (figure 5) with normal upper gastrointestinal endoscopy. An endoscopic ultrasound was performed for aspiration of the lymph node, despite the difficulty of the approaching. The immunohistochemical analysis was compatible with adenocarcinoma, with a probable primary site in the gastrointestinal tract.

Conclusion

Neoplasms of the ovary, lung, pancreas, stomach, esophagus, cervix, colorectal, non-Hodgkin's lymphoma and nasopharyngeal carcinoma are the most commonly associated with dermatomyositis. The case presented reinforces this association and the importance of exhaustive neoplastic screening. The diagnosis, when performed at an early stage, can modify the course of the disease and the prognosis of the patient.

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