Systemic necrotizing vasculitis associated with childhood sarcoidosis.
Childhood sarcoidosis is a rare disorder with protean manifestations. The case of a child with prolonged fever, hepatosplenomegaly, pancytopenia, and systemic necrotizing vasculitis manifesting as fever, rash and skin infarctions, digital pregangrene, and foot drop is reported. This is the first case of systemic necrotizing vasculitis reported in sarcoidosis. The fulminant course of the disease required treatment with intravenous pulsed cyclophosphamide and high doses of corticosteroids. The spectrum of vasculitis in childhood and adult sarcoidosis is reviewed.
Kwong
T
T
Division of Pediatric Rheumatology, Schneider Children's Hospital/Long Island Jewish Medical Center, New Hyde Park, NY 11042.
Valderrama
E
E
Paley
C
C
Ilowite
N
N
D001853:Bone Marrow / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology; D010488:Polyarteritis Nodosa / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D012507:Sarcoidosis / Q000150:complications* / Q000473:pathology; D012867:Skin / Q000473:pathology
D003520:Cyclophosphamide; D011241:Prednisone