Urticarial Vasculitis

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Contents 1 Etiology 2 Epidemiology 3 Pathology 4 Clinical-manifestations 5 Laboratory 6 Radiology 7 Complications 8 Differential-diagnosis 9 Management 10 More General Terms 11 References

Etiology

• serum sickness
• drug induced: ACE inhibitors, penicillin, sulfonamides, fluoxetine, & thiazides
• connective tissue disease ( hypocomplementemic form)
• viral infection

• hepatitis B, hepatitis C, infectious mononucleosis

• idiopathic (majority of cases)

Epidemiology

• male:female ratio is 1:2
• median age: 43 years, range: 15-90 years
• in serum sickness

• occurs 8-14 days after antigen exposure in non- sensitized individuals
• occurs within 36 hours in sensitized individuals

Pathology

• histologically, changes of leukocytoclastic vasculitis
• perivascular neutrophilic infiltrate involving postcapillary venules
• expansion of the vessel wall occurs
• endothelium is intact
• deposition of complement & fibrin in the blood vessels &, occasionally, IgM, IgG, & IgA along the basement membrane
• extravasation of red blood cells
• eosinophils may be noted early
• later in the lesion's course, infiltrate may become a mixture of lymphocytes & neutrophils
• pulmonary vasculitis has NOT been demonstrated

Clinical-manifestations

• urticarial lesions

• erythematous wheals, circumscribed areas of edema
• occasionally indurated
• sometimes purpuric, petechiae
• pruritis or burning
• individual lesions last up to 5 days
• wheals hurt rather than itch & leave residual pigmentation after they resolve [4]

• lymphadenopathy
• arthralgias (60%)
• arthritis 28%
• abdominal pain (25%)
• glomerulonephritis (15%)

Laboratory

• serum complement levels

• CH50, serum C3, serum C4, serum Clq
• may be diminished or normal

• autoantibodies to C1q & vascular endothelial cells ( hypocomplementemic form)
• antineutrophilic cytoplasmic antibodies rare
• renal function tests & urinalysis to evaluate effects of vasculitis on kidneys
• skin biopsy: (recent lesion, < 48 hours after onset)

• confirms leukocytoclastic vasculitis [4]
• direct immunofluorescence may show deposition of vascular C3, fibrin, & immunoglobulins

Radiology

• chest x-ray for patients with hypocomplementemia & pulmonary symptoms

Complications

• greater incidence of coexisting disease in hypocomplementemic form:

• angioedema
• connective-tissue disease [primarily SLE]
• COPD in 62% with hypocomplementemic form

• many of these had been smokers

Differential-diagnosis

• lesions last longer & are more livid than urticaria [4]

Management

• treatment is based on

• systemic effects of the disease
• extent of cutaneous involvement
• previous response to treatment

• for patients with cutaneous involvement only

• antihistamines
• nonsteroidal anti-inflammatory drugs ( NSAIDs)
• for persistent cutaneous manifestations

• colchicine, hydroxychloroquine, or dapsone

• for patients with systemic involvement or refractory disease

• glucocorticoids
• azathioprine
• mycophenolate mofetil
• rituximab

• prognosis: good, most cases resolve in months to years

More General Terms

• leukocytoclastic vasculitis; hypersensitivity vasculitis

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 [1]
4. Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: [2]

urticarial vasculitis