Hypocomplementemic urticarial vasculitis syndrome in identical twins.
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a syndrome of recurrent urticarial vasculitis, arthralgia/arthritis, and hypocomplementemia. Angioedema, ocular inflammation, glomerulonephritis, and obstructive lung disease are other clinical findings. Although the etiology of HUVS is unknown, its resemblance to systemic lupus erythematosus (SLE) suggests a similar pathogenesis. SLE is known to occur in identical twins. This is the first report of a pair of identical twins with HUVS. Concordance for HUVS in identical twins suggests that the pathogenesis of the disease involves abnormal genetic immunoregulation.
Wisnieski
J J
JJ
Medical Research Service, VA Medical Center, Cleveland, OH 44106.
Emancipator
S N
SN
Korman
N J
NJ
Lass
J H
JH
Zaim
T M
TM
McFadden
E R
ER
D000328:Adult; D001706:Biopsy; D003165:Complement System Proteins / Q000032:analysis* / Q000172:deficiency; D004200:Diseases in Twins / Q000235:genetics; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000235:genetics; D013577:Syndrome; D014581:Urticaria / Q000097:blood* / Q000150:complications*; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000097:blood* / Q000150:complications*
D003165:Complement System Proteins