Hypocomplementemic urticarial vasculitis syndrome in three siblings

Ozcakar Z. B., Yalcinkaya F., Altugan F. S., Kavaz A., Ensari A., Ekim M.

RHEUMATOLOGY INTERNATIONAL, cilt.33, sa.3, ss.763-766, 2013 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 33 Sayı: 3
  • Basım Tarihi: 2013
  • Doi Numarası: 10.1007/s00296-010-1645-5
  • Dergi Adı: RHEUMATOLOGY INTERNATIONAL
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.763-766
  • Anahtar Kelimeler: Hypocomplementemia, Urticarial vasculitis, Child, Familial, Lupus, LUPUS-ERYTHEMATOSUS
  • Eskişehir Osmangazi Üniversitesi Adresli: Hayır

Özet

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is relatively uncommon and generally seen in the fourth decade of life. There are very few pediatric cases with the diagnosis of HUVS in the literature. In this report, we describe the first familial cases of HUVS in three siblings. The disease onset was during childhood period in all patients. One of them developed severe renal involvement and died. The other two had ongoing skin and eye manifestations and the elder one developed lupus. Presence of these three patients is a strong evidence for the role of genetic factors in the pathogenesis of this rare vasculitis.