Juvenile dermatomyositis: A report of three cases.
Ultrastruct Pathol
; 40(2): 83-5, 2016.
Article
in En
| MEDLINE
| ID: mdl-26886841
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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Muscle, Skeletal
/
Endothelial Cells
/
Dermatomyositis
Type of study:
Diagnostic_studies
/
Prognostic_studies
/
Screening_studies
Limits:
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Language:
En
Journal:
Ultrastruct Pathol
Year:
2016
Document type:
Article
Affiliation country:
Italia