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urticarial vasculitis

Etiology: 1) serum sickness 2) drug induced: ACE inhibitors, penicillin, sulfonamides, fluoxetine, & thiazides 2) connective tissue disease (hypocomplementemic form) - systemic lupus erythematosus 3) viral infection - hepatitis B, hepatitis C, infectious mononucleosis 4) idiopathic (majority of cases) Epidemiology: 1) male:female ratio is 1:2 2) median age: 43 years, range: 15-90 years 3) in serum sickness a) occurs 8-14 days after antigen exposure in non- sensitized individuals b) occurs within 36 hours in sensitized individuals 4) relatively rare [6] Pathology: 1) histologically, changes of leukocytoclastic vasculitis 2) perivascular neutrophilic infiltrate involving postcapillary venules 3) expansion of the vessel wall occurs 4) endothelium is intact 5) deposition of complement & fibrin in the blood vessels &, occasionally, IgM, IgG, & IgA along the basement membrane 6) extravasation of red blood cells 7) eosinophils may be noted early 8) later in the lesion's course, infiltrate may become a mixture of lymphocytes & neutrophils 9) pulmonary vasculitis has NOT been demonstrated Clinical manifestations: 1) urticarial lesions a) erythematous wheals, circumscribed areas of edema b) occasionally indurated c) sometimes purpuric, petechiae d) pruritus or burning e) painful more than pruritic [6] e) individual lesions last up to 5 days f) wheals hurt, burn or sting rather than itch & leave residual pigmentation (bruising) after they resolve [4,5] 2) lymphadenopathy 3) arthralgias (60%), arthritis 28% 4) fever [5] 5) abdominal pain (25%) 6) glomerulonephritis (15%) Laboratory: 1) serum complement levels a) CH50, serum C3, serum C4, serum Clq b) may be diminished or normal 2) autoantibodies to C1q & vascular endothelial cells (hypocomplementemic form) 3) antineutrophilic cytoplasmic antibodies rare 4) renal function tests & urinalysis to evaluate effects of vasculitis on kidneys 5) skin biopsy: (recent lesion, < 48 hours after onset) a) confirms leukocytoclastic vasculitis [4] a) direct immunofluorescence may show deposition of vascular C3, fibrin, & immunoglobulins Radiology: - chest x-ray for patients with hypocomplementemia & pulmonary symptoms Differential diagnosis: - urticaria - lesions resolve faster (< 24 hours) & are less livid [4,8] - itch more common than burning & stinging - wheals resolve without hyperpigmentation - no systemic symptoms, such as fever or joint pain [8] Complications: - greater incidence of coexisting disease in hypocomplementemic form: a) angioedema b) connective-tissue disease [primarily SLE] c) COPD in 62% with hypocomplementemic form - many of these had been smokers - nephritis Management: 1) treatment is based on a) systemic effects of the disease b) extent of cutaneous involvement c) previous response to treatment 2) for patients with cutaneous involvement only a) antihistamines b) nonsteroidal anti-inflammatory drugs (NSAIDs) c) for persistent cutaneous manifestations - hydroxychloroquine, colchicine, or dapsone 3) for patients with systemic involvement or refractory disease a) glucocorticoids b) azathioprine c) mycophenolate mofetil d) rituximab e) cyclophosphamide 4) prognosis: good, most cases resolve in months to years

Related

urticaria (hives)

General

leukocytoclastic vasculitis; hypersensitivity vasculitis; trisymptome

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 783
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 94
  3. eMedicine http://www.emedicine.com/derm/topic449.htm
  4. Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: 19501230
  5. Medical Knowledge Self Assessment Program (MKSAP) 16, 18, 19. American College of Physicians, Philadelphia 2012, 2018, 2022
  6. Stigall LE, Sigmon JR, Leicht SS. Urticarial vasculitis: a unique presentation. South Med J. 2009 May;102(5):531-3 PMID: 19373142
  7. Venzor J, Lee WL, Huston DP. Urticarial vasculitis. Clin Rev Allergy Immunol. 2002 Oct;23(2):201-16. Review. PMID: 1222186
  8. Davis MD, van der Hilst JC. Mimickers of urticaria: urticarial vasculitis and autoinflammatory diseases. J Allergy Clin Immunol Pract. 2018;6:1162-70. PMID: 29871797