Research Article

A case report of Hypocomplementemic urticarial vasculitic syndrome presenting with Renal failure

Amy M Hopkins, Angela M Gibbs, Ryan S Griffiths, Rupali S Avasare and Firas G Khoury*

Published: 09/12/2018 | Volume 2 - Issue 3 | Pages: 039-043


We present a case of hypocomplementemic urticarial vasculitic syndrome (HUVS) who developed severe renal failure requiring ICU-level care. Our patient is a 66-year-old man who presented with abdominal pain, rash, confusion, oliguria, and shortness of breath. He was found to be in acute renal failure with leukocytosis and elevated lactate. Work-up for infectious, autoimmune, and hematologic malignant diseases was negative. The presence of chronic urticaria, abdominal pain, hypocomplementemia, and leukocytoclastic vasculitis on skin biopsy confirmed the diagnosis of HUVS. He required hemodialysis for renal failure as well as gastrostomy tube placement for nutritional support secondary to the development of mucosal ulcers, a rare finding in HUVS. He recovered with several months of high-dose steroids and hemodialysis. This case highlights the effectiveness of steroids for initial treatment of HUVS, and the relapsing and remitting nature of the disease. Providers should also be aware of the broad range of presenting symptoms such as mucosal lesions that may require nutritional support. Interestingly, unlike many previously reported cases of HUVS, our patient had not yet developed signs and symptoms of systemic lupus erythematosus, which often overlaps with HUVS.

Read Full Article HTML DOI: 10.29328/journal.jcn.1001017 Cite this Article


  1. Park C, Choi SW, Kim M, Park J, Lee JS, et al. Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report. J Med Case Rep. 2014;8: 352.  Ref.:
  2. McDuffie FC, Sams Jr. WM, Maldonado JE, Andreini PH, Conn DL, et al. Hypocomplementemia with cutaneous vasculitis and arthritis. Possible immune complex syndrome. Mayo Clin Proc. 1973; 48: 340–348. Ref.:
  3. Aydogan K, Karadogan SK, Adim SB, Tunali S. Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus. Int J Dermatol. 2006; 45: 1057–1061. Ref.:
  4. Buck A, Christensen J, McCarty M. Hypocomplementemic urticarial vasculitis syndrome: a case report and literature review. J Clin Aesthet Dermatol. 2012; 5: 36-46. Ref.:
  5. Carbonella A, Mancano G, Gremese E, et al. An autosomal recessive DNASE1L3-related autoimmune disease with unusual clinical presentation mimicking systemic lupus erythematosus. Lupus. 2017; 26: 768–772. Ref.:
  6. Davis MD, Brewer JD. Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am. 2004; 24: 183–213. Ref.:
  7. Her MY, Song JY, Kim DY. Hypocomplementemic urticarial vasculitis in systemic lupus erythematosus. J Korean Med Sci. 2009; 24:184–186. Ref.:
  8. Wisnieski JJ1, Baer AN, Christensen J, Cupps TR, Flagg DN, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Med. 1995; 74: 24–41. Ref.:
  9. Schwartz HR, McDuffie FC, Black LF, Schroeter AL, Conn DL. Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease. Mayo Clin Proc. 1982; 57: 231–238. Ref.:
  10. Grotz W, Baba HA, Becker JU, Baumgartel MW. Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge. Dtsch Arztebl Int. 2009; 106: 756–763. Ref.:
  11. Santiago M, Machicado V. IMAGES IN CLINICAL MEDICINE. Jaccoud’s Arthropathy. N Engl J Med. 2015; 373: e1. Ref.:
  12. Grimbert P, Schulte K, Buisson C, Desvaux D, Baron C, et al. Renal transplantation in a patient with hypocomplementemic urticarial vasculitis syndrome. Am J Kidney Dis. 2001; 37: 144–148. Ref.:
  13. Pasini A, Bracaglia C, Aceti A, Vivarelli M, Lavacchini A, et al. Renal involvement in hypocomplementaemic urticarial vasculitis syndrome: a report of three paediatric cases. Rheumatol. 2014; 53: 1409–1413. Ref.:
  14. Salim SA, Yousuf T, Patel A, Fulop T, Agarwal M. Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis. Am J Med Sci. 2018; 355: 195–200. Ref.:
  15. Toprak O, Cirit M, Uzunel H, Ersoy R, Ermete M, et al. Hypocomplementaemic urticarial vasculitis syndrome and acute renal failure with cryoglobulin (–) hepatitis C infection. Nephrol Dial Transplant. 2004;19: 2680–2682. Ref.:
  16. Wisnieski JJ, Naff GB. Serum IgG antibodies to C1q in hypocomplementemic urticarial vasculitis syndrome. Arthritis Rheum. 1989; 32: 1119–1127. Ref.:
  17. Habif TP. Urticaria, angioedema, and pruritis. In: Clinical Dermatology. Elsevier Inc; 2015; 178–217.
  18. Fortson JS, Zone JJ, Hammond ME, Groggel GC. Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone. J Am Acad Dermatol. 1986; 15: 1137–1142. Ref.:
  19. Worm M, Sterry W, Kolde G. Mycophenolate mofetil is effective for maintenance therapy of hypocomplementaemic urticarial vasculitis. Br J Dermatol. 2000; 143: 1324. Ref.:
  20. Davis MD, Daoud MS, Kirby B, Gibson LE, Rogers RS 3rd. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol. 1998; 38: 899–905. Ref.:
  21. Trendelenburg M1, Courvoisier S, Späth PJ, Moll S, Mihatsch M, et al. Hypocomplementemic urticarial vasculitis or systemic lupus erythematosus? Am J Kidney Dis. 1999; 34: 745–751. Ref.: