Fatal vascular occlusion in juvenile dermatomyositis

Fatal vascular occlusion in juvenile dermatomyositis.

Juvenile dermatomyositis.

Juvenile dermatomyositis (JDMS) is a chronic inflammatory condition characterized by muscle weakness and a distinctive rash caused by underlying vasculopathy. Long-term complications include subcutaneous and muscular calcification, contractures and in some cases the gradual development of a second connective tissue disease. Early aggressive treatment with systemic immunosuppressants and other a...

Juvenile dermatomyositis.

The clinical profile of 7 children and their follow-up is described. There was female preponderance with mate to female ratio of 1:6. The median age of onset was 6 years. All the patients had skin rash, muscle weakness and abnormal enzyme profile. Muscle biopsy was performed in 6 and was abnormal in all of them. The electromyogram (EMG) was performed in 6 and was found abnormal in five. All the...

Juvenile Dermatomyositis in Pregnancy

Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when...

Juvenile dermatomyositis in Western Switzerland

Introduction Juvenile dermatomyositis (JDM) is a rare chronic, autoimmune vasculopathic disease with mainly muscle and skin involvement. The incidence described in USA is 2.5-4.1 cases per million children; the disease is slightly more common in girls. Recent studies describe that two third of the patients followed a chronic disease course, whereas only one third showed monocyclic disease course.