Diagnosis and treatment of dermatomyositis, a review

Abstract

Introduction.  Dermatomyositis (DM) is a rare chronic autoimmune disease with primary features of symmetrical proximal muscle weakness and skin involvement with differentiated skin lesions and a clinically heterogeneous constellation of systemic manifestations that may affect other organs mainly heart, lung, and digestive system. In the absence of characteristic dermatological findings or myopathy, DM can be difficult to diagnose; therefore, due to its low incidence and the complexity of its diagnosis, it is a topic of interest to motivate other colleagues in the health area to approach this pathology. Objective. To analyze the current scientific evidence of dermatomyositis and to elaborate a detailed review of the pathology. Methodology.  The present work is a systematic review of the existing literature, of publications in scientific journals compiling information from the databases of Medline, Intra Med, PubMed, The Cochrane, SciELO and other journals with quality scientific evidence in which descriptors such as: Dermatomyositis, paraneoplastic syndrome, cancer are used. The records range between 30 and 50, after the combination of the different keywords with date restriction within the last five years, in Spanish and English. Results. Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of systemic acquired diseases characterized by chronic progressive muscle weakness and an inflammatory cellular infiltrate in skeletal muscle. In this group, polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM) are the most frequent diseases. Diagnosis is based on the cutaneous manifestations of the disease and the degree of muscle involvement, and when there is doubt in the diagnosis, electromyography or skin and muscle biopsy may be used. The goals of treatment of dermatomyositis include improving physical function and preventing the development of disability. Treatment should be instituted early and requires a team approach involving physical therapist, dermatologist, and family physician. Involvement of other specialists may be necessary, depending on the manifestations of the disease. Conclusion. The diagnosis of dermatomyositis is given largely by the clinical manifestations of the disease, but in certain cases it represents a diagnostic challenge in patients who suffer from it in the absence of cutaneous manifestations, requiring complementary studies such as skin biopsy, electromyography and even muscle biopsy. Dermatomyositis is an autoimmune disease that generally appears as a paraneoplastic syndrome associated with an underlying malignant neoplasm in a subgroup of patients. Because of this, comprehensive management and timely diagnosis is necessary to prevent possible complications. The goals of dermatomyositis treatment include improving physical function and avoiding the development of disability. Among the therapeutic options that help to control this disease, we find pharmacological therapies such as the use of systemic corticosteroids, immunoglobulins, monoclonal antibodies and immunomodulators.