Hypocomplementemic urticarial vasculitis syndrome with severe systemic manifestations.
We describe two children with clinical and laboratory features of hypocomplementemic urticarial vasculitis syndrome. Both patients had severe, life-threatening manifestations: rapidly progressive glomerulonephritis (patient 1) and pulmonary hemorrhage (patient 2). We conclude that this syndrome may be a potentially severe multisystem disease.
Martini
A
A
Clinica Pediatrica, Università di Pavia, Italy.
Ravelli
A
A
Albani
S
S
De Benedetti
F
F
Massa
M
M
Wisnieski
J J
JJ
D000293:Adolescent; D002648:Child; D003165:Complement System Proteins / Q000172:deficiency*; D005921:Glomerulonephritis / Q000209:etiology*; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D008171:Lung Diseases / Q000209:etiology*; D008297:Male; D013577:Syndrome; D014581:Urticaria / Q000150:complications*; D014657:Vasculitis / Q000150:complications*
D003165:Complement System Proteins