Dermatomiositis: estudio clínico - epidemiológico.

La dermatomiositis (DM) pertenece al grupo de dermatomiopatias inflamatorias idiopaticas (DMII); es una enfermedad inflamatoria cronica que afecta la piel y el musculo esqueletico. Se denomina Dermatomiositis clinicamente amiopatica (DMCA) a un subgrupo que exhibe las manifestaciones cutaneas caracteristicas de la DM por mas de 6 meses sin desarrollar debilidad muscular. Objetivo: identificar las caracteristicas clinicas y epidemiologicas de los casos de DM diagnosticados en el Servicio de Dermatologia del Instituto de Biomedicina Dr. Jacinto Convit del Hospital Vargas de Caracas, en el periodo comprendido entre Enero 2009 y Diciembre 2013. Metodos: se realizo un estudio retrospectivo, descriptivo y transversal. Se revisaron las historias con diagnostico de DM y se analizaron las caracteristicas clinicas y epidemiologicas. Resultados: Se identificaron 40 casos, la mayoria de sexo femenino (67.5%). La edad promedio al momento del diagnostico fue de 42.8 anos. La mayoria de los casos (57.5%) presentaron diagnostico de DM clasica, 32.5% DMCA, 7.5% DM de inicio juvenil y 2.5% DM asociada a cancer. Eritema en heliotropo fue el signo cutaneo mas reportado (75%), seguido de papulas de Gottron (70%) y poiquilodermia en areas fotoexpuestas (70%) de la cual predomino el signo de la “V” (47.5%). Se documentaron alteraciones periungueales en el 50% de los casos. La calcinosis y el fenomeno de Raynaud estuvieron presentes en un 15% y 7.5%, respectivamente. El 75% contaba con reporte de la creatin quinasa (CK). Conclusiones: Es importante conocer las caracteristicas clinicas de la DM para realizar un correcto diagnostico, sabiendo que dicha patologia conlleva a una alta morbilidad y discapacidad funcional. Dermatomyositis: clinical- epidemiological study Summary. Dermatomyositis (DM) belongs to a group of idiopathic inflammatory dermatomyositis (IIDM). Is a chronic inflammatory disease that affects the skin and muscles. Clinically amyopathic dermatomyositis (CADM) is a subgroup that develops the same cutaneous manifestatios for more than 6 months without muscle involvement. Objective: to identify the clinical and epidemiological characteristics of patients diagnosed with DM at the Department of Dermatology, in Instituto de Biomedicina Dr. Jacinto Convit, Hospital Vargas de Caracas, in the period between January 2009 and December 2013. Methods: A retrospective, descriptive and transversal study was conducted. The medical records of all DM cases were reviewed, and the main clinical and epidemiological characteristics were analyzed. Results: 40 cases were identified, most of which were female (67.5%). Average age of diagnosis was 42.8 years old. Most cases (57.5%) were diagnosed with classic DM, 32.5% CADM, 7.5% juvenile DM and 2.5% DM associated with cancer. Heliotrope rash was the predominant reported cutaneous manifestation (75%), followed by Gottron papules (70%) and poikiloderma in sun-exposed areas (70%) of which, the “V sign” was the most common (47.5%). Periungueal alterations were found in 50% of cases. Calcinosis and Raynaud phenomenon were present in 15% and 7.5% respectively. Seventy five percent (75%) of patients had creatine kinase (CK) values reported. Conclusions: in order to make an accurate diagnosis, is important to know the clinical characteristics of DM, knowing that this disease is related to high morbidity and disability.