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.
Medical Research Service, VA Medical Center, Cleveland, OH 44106.
J J
JJ
Wisnieski
S N
SN
Emancipator
N J
NJ
Korman
J H
JH
Lass
T M
TM
Zaim
E R
ER
McFadden
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