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

• Published in: Pediatrics (Evanston) Vol. 129; no. 3; p. e821
• 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: Journal Article
• Language: English
• Published: United States 01.03.2012
• Subjects: Biopsy, Needle; Child; Dermatomyositis - diagnosis; Dermatomyositis - pathology; Diagnosis, Differential; Follow-Up Studies; Humans; Immunohistochemistry; Male; Plasmapheresis - methods; Prednisone - therapeutic use; Purpura, Thrombotic Thrombocytopenic - diagnosis; Purpura, Thrombotic Thrombocytopenic - pathology; Purpura, Thrombotic Thrombocytopenic - therapy; Retinal Diseases - diagnosis; Retinal Diseases - pathology; Retinoscopy - methods; Risk Assessment; Severity of Illness Index; Treatment Outcome
• ISSN: 1098-4275
• DOI: 10.1542/peds.2011-0338

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.