Thrombotic microangiopathy and Purtscher-like retinopathy as a rare presentation of juvenile dermatomyositis

Juvenile dermatomyositis is a rare systemic vasculopathy that may sometimes present with acute complications. We report here the case of a 7-year-old boy with severe dermatomyositis associated with thrombocytopenia and blurry vision. The presence of schistocytosis and the secondary occurrence of hem...

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Bibliographic Details
Published in:Pediatrics (Evanston) 2012-03, Vol.129 (3), p.e821-e824
Main Authors: Bader-Meunier, Brigitte, Monnet, Dominique, Barnerias, Christine, Halphen, Isabelle, Lambot-Juhan, Karen, Chalumeau, Martin, Costedoat-Chalumeau, Nathalie, Ribeil, Jean-Antoine, Bodemer, Christine, Gherardi, Romain
Format: Article
Language:English
Subjects:
Biopsy, Needle
Care and treatment
Causes of
Child
Children & youth
Dermatomyositis
Dermatomyositis - diagnosis
Dermatomyositis - pathology
Diagnosis
Diagnosis, Differential
Eyes & eyesight
Follow-Up Studies
Humans
Immunohistochemistry
Male
Medical diagnosis
Pediatrics
Plasmapheresis - methods
Prednisone - therapeutic use
Prevention
Purpura, Thrombotic Thrombocytopenic - diagnosis
Purpura, Thrombotic Thrombocytopenic - pathology
Purpura, Thrombotic Thrombocytopenic - therapy
Retina
Retinal diseases
Retinal Diseases - diagnosis
Retinal Diseases - pathology
Retinoscopy - methods
Risk Assessment
Severity of Illness Index
Thrombocytopenic purpura
Treatment Outcome
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Summary:Juvenile dermatomyositis is a rare systemic vasculopathy that may sometimes present with acute complications. We report here the case of a 7-year-old boy with severe dermatomyositis associated with thrombocytopenia and blurry vision. The presence of schistocytosis and the secondary occurrence of hemolytic anemia were consistent with a diagnosis of thrombotic thrombocytopenic purpura (TTP). Further investigations demonstrated the association of TTP with muscular microangiopathy and Purtscher-like retinopathy. Retinal and hematologic involvements dramatically improved after the initiation of plasma exchange in emergency. This report emphasizes that early recognition of TTP and prompt plasmapheresis are important in a child with severe juvenile dermatomyositis associated with thrombocytopenia.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.2011-0338