@entity8817 is a rare, chronic @entity74 of unknown etiology, characterized by a typical @entity1910 and proximal @entity425 . A retrospective study from the medical records of @entity1 diagnosed as @entity8817 was performed at Queen Sirikit National Institute of @entity1 Health from 1988 to 1998. There were seven cases of @entity8817 diagnosed according to the criteria of Bohan and Peter. Six cases were female and one case was male. The age of diagnosis ranged from 2.5 years to 11 years. (mean age was 7 +/- 3.6 years). The presenting symptoms were @entity425 (6 cases), @entity925 (2 cases) and @entity1910 (4 cases). All of the @entity1 developed proximal @entity425 of the lower extremities varying from grade 3 to grade 4. The cutaneous manifestations were heliotrope signs (6 cases), gottron's papules (2 cases), photosensitivity (2 cases) and @entity730 cutis (4 cases). Electromyography (EMG) was performed in 6 cases and revealed typical change of myopathic type. Elevated muscle enzymes were noted in all cases. Muscle biopsy was performed in 6 cases and was compatible with @entity5442 . Oral @entity617 (1-2 mg/kg/day) was given in 6 cases and the @entity425 improved. There was no mortality in this study. Four cases developed @entity730 cutis 1 to 3 years after @entity425 and did not respond to any treatment. In conclusion, @entity8817 is a disease which causes @entity717 in @entity1 . Early diagnosis and treatment can prevent morbidity and mortality. Calcification at the skin usually occurs after the onset of @entity425 several months to years after diagnosis.

@entity8817 in Thai XXXX .

@entity1 :: ('9606', 'Species') :: ['Child', 'children', 'patients'] @entity5442 :: ('MESH:D009220', 'Disease') :: ['myositis'] @entity1910 :: ('MESH:D005076', 'Disease') :: ['skin rashes', 'skin rash'] @entity425 :: ('MESH:D018908', 'Disease') :: ['muscle weakness'] @entity8817 :: ('MESH:C538250', 'Disease') :: ['juvenile dermatomyositis', 'Juvenile dermatomyositis'] @entity730 :: ('MESH:D002114', 'Disease') :: ['calcinosis'] @entity74 :: ('MESH:D030342', 'Disease') :: ['multisystemic inflammatory disorder'] @entity717 :: ('MESH:D009069', 'Disease') :: ['chronic disability'] @entity925 :: ('MESH:D063806', 'Disease') :: ['muscle pain'] @entity617 :: ('MESH:D011239', 'Chemical') :: ['prednisolone']
@entity1 :: (9606,Species) :: ['children']
