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 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.
Department of Pediatric Immunology and Rheumatology, Paris Descartes University, Necker Hospital, AP-HP, Paris, France. brigitte.bader-meunier@nck.aphp.fr
Brigitte
B
Bader-Meunier
Dominique
D
Monnet
Christine
C
Barnerias
Isabelle
I
Halphen
Karen
K
Lambot-Juhan
Martin
M
Chalumeau
Nathalie
N
Costedoat-Chalumeau
Jean-Antoine
JA
Ribeil
Christine
C
Bodemer
Romain
R
Gherardi
D001707:Biopsy, Needle; D002648:Child; D003882:Dermatomyositis / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010956:Plasmapheresis / Q000379:methods; D011241:Prednisone / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D012164:Retinal Diseases / Q000175:diagnosis* / Q000473:pathology; D042262:Retinoscopy / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
D011241:Prednisone