Juvenile dermatomyositis: A report of three cases |
| |
| Authors: | V Papa B Romanin R Bergamaschi D M Cordelli R Costa L Badiali De Giorgi |
| |
| Institution: | 1. Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italyvalentina.papa@gmail.com;3. Department of Pediatric Emergency, S. Orsola-Malpighi Hospital, Bologna, Italy;4. Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy;5. Operative Unit of Child Neuropsychiatry, S. Orsola-Malpighi Hospital, Bologna, Italy;6. Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy;7. Operative Unit of Anatomy, Pathological Histology, S. Orsola-Malpighi Hospital, Bologna, Italy |
| |
| Abstract: | Juvenile dermatomyositis (JDM), an autoimmune idiopathic myositis, is characterized by rash and proximal muscle weakness. Immunohistopathology typically shows perivascular inflammatory infiltrate with predominance of CD4+ T lymphocytes, perifascicular atrophy, and upregulation of major histocompatibility complex class I. JDM has been attributed to a humoral-driven muscle microangiopathy probably implicating the type I interferon pathway. Tubulo-reticular inclusions present in endothelial cell of muscle are biomarkers of interferon exposure, and so may be an indirect data of this myopathy especially in the absence of rash and inflammatory infiltrate. We report on three patients in which electron microscopy solves the differential diagnosis among infantile myositis showing peculiar inclusions. |
| |
| Keywords: | Dermatomyositis infantile myosytis tubulo-reticular inclusions |
|
|
