DERMATOMYOSITIS AND HEAR LOSS: TWO MANIFESTATIONS OF ONE DISEASE?

Introduction: Dermatomyositis (DM) is an idiopathic inflammatory myopathy, characterized by features of proximal skeletal muscle weakness, evidence of muscle inflammation and characteristic skin manifestations. Caucasian male 18 years, previously healthy, was referred to our department due to a history with 5 months of evolution of proximal muscular weakness (especially in upper limbs). The patient reported fatigue, difficulty climbing stairs and difficulty swallowing. Simultaneously mentioned height loss (10kg in 3 months), and photosensitive erythema over face, neck, dorsum of metacarpalphalangeal (MCF) and proximal interphalangeal (PIP) joints. In the physical examination it stands out periorbital heliotrope rash and Gottron's papules over the dorsal PIP and MCP. In the neurological examination, muscle strength was 3/5 proximal at upper limbs, Growers sign was present. No arthritis was identified. Blood analysis exhibited a discrete elevation of creatine phosphokinase: 181 U/L, aldolase: 7.8 U/L (Normal< 7,6). The inflammatory markers, antinuclear antibody, myositis-specific, and myositis-associated autoantibody were negative. Electromyography presented lesion of muscular fibers. The diagnosis of DM was made, and the patient started 60mg/day of prednisolone. A muscular biopsy confirmed the diagnosis of DM (muscle with lesions of inflammatory myopathy). During the following period, the patient showed clinical improvement (improvement of muscle strength was 5/5 proximal at upper limbs, Growers sign was negative, allowing reduction of dosage of prednisolone. After 4 months of treatment, the patient presented bilateral hearing loss. An MRI was made which revealed «presence of an expansive formation centered on the posterior wall of the oropharynx, obliterating the nasopharynx, exerting a mass effect on the adjacent structures». A biopsy of the lesion was executed by otorhinolaryngologist, and histology revealed EBV-associated non-keratinizing epidermoid carcinoma whose staging was T2N2M0. The patient started radiotherapy with chemotherapy with cisplatin 100mg/m2, remaining in treatment with clinical improvement. Conclusion: The association between malignancy and inflammatory myopathy has been supported by numerous epidemiologic studies, with the strongest association occurring in those with DM2. The clinical implications of this association, irrespective of the pathophysiologic mechanisms involved, are that it is imperative to screen for tumors in patients with DM, particularly if the symptoms do not respond to conventional immunosuppressive treatment. The types of malignancies vary and include not only hematologic malignancies such as lymphoma but also solid tumors such3. Ethnic backgrounds should be considered in screening, because cancer types vary according to race, with lung and nasopharyngeal cancers being more frequent in Asians than Caucasians. The authors highlight this case due to the rarity of the association between DM and nasopharyngeal cancer especially in a young Caucasian patient. [ABSTRACT FROM AUTHOR]