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Hypersensitivity Vasculitis

Hypersensitivity vasculitis is the most commonly seen form of small-vessel necrotizing vasculitis. The disease may be limited to the skin or may involve many different organs, in which case it is called cutaneoussystemic angiitis.  Histologically there is fibrinoid necrosis of small dermal blood vessels, leukocytoclasis, endothelial cell swelling, and extravasation of red blood cells (RBCs) hypersensitivity vasculitis.

Diagnosis.

Prodromal symptoms include fever, malaise, myalgia, and joint pain. The characteristic lesions are referred to as palpable purpura. The lesions begin as asymptomatic, localized areas of cutaneous hemorrhage that acquire substance and become palpable as blood leaks out of damaged vessels. Lesions may coalesce, producing large areas of purpura leukocytoclastic vasculitis. Nodules and urticarial lesions may appear. Hemorrhagic blisters and ulcers may arise from these purpuric areas and indicate more severe vessel inflammation and necrosis. A few-to-numerous discrete, purpuric lesions

Lesions appear in crops, last for 1 to 4 weeks, and heal with residual scarring and hyperpigmentation. Patients may experience one episode if a drug or viral infection is the cause or multiple episodes.

Systemic disease.

Hypersensitivity vasculitis has many systemic manifestations; the numbers.

An analysis of cutaneous hypersensitivity vasculitis in patients seen by 

Kidneys (50%): Kidney disease is the most common systemic manifestation. Mild vasculitis.
 
Nervous system (40%): Peripheral neuropathy with hypoesthesia or paresthesia is more common
Gastrointestinal tract (36%): Vasculitis of the bowel causes abdominal pain, nausea, vomiting.
 
Lung (30%): Pulmonary vasculitis may be asymptomatic, detected only as nodular or diffuse.
 
Joints (30%): Symptoms vary from pain to erythema.
 
Heart (50%): Myocardial angiitis produces arrhythmias.
Etiology.

(1) Drugs--penicillin, thiazides, aspirin, phenothiazines, sulfonamides, iodides, and many others in single case reports; (2) infections--streptococcal upper respiratory tract infections, Escherichia coli urinary tract infection, and others; (3) connective tissue disease; (4) malignant neoplasms; and (5) other  

Skin biopsy.

The clinical presentation is so characteristic that a biopsy.

SPECTRUM AND INCIDENCE OF 101 CASES OF NECROTIZING VASCULITIS


NECROTIZING VASCULITIS ASSOCIATED WITH COEXISTENT DISEASE
 
Rheumatoid arthritis, 12%

 
Malignancy, 8%

 
Polyarteritis nodosa, 7%

 
Systemic lupus erythematosus, 6%

 
Sjogren's syndrome, 3%

 
Wegener's granulomatosis, 3%

 
Granuloma faciale, 3%

 
Hypergammaglobulinemic purpura, 2%

 
Churg-Strauss syndrome, 2%


NECROTIZING VASCULITIS ASSOCIATED WITH PRECIPITATING EVENT
 
Drug reaction, 13%

 
Bacterial infection, 8%

 
Viral infection, 1%


NECORTIZING VASCULITIS OF UNCERTAIN CAUSE
 
Idiopathic palpable purpura, 13%

 
Chronic urticaria or angioedema, 10%

 
Henoch-Schonlein purpura, 4%

 
Idiopathic vasculitis with nodules, 3%

Treatment.

Identify and remove the offending antigen (i.e., drug, chemical, or infection). No other treatment may be necessary and the disease may clear spontaneously. In other instances the disease persists or becomes recurrent. Short courses of prednisone (40 to 60 mg/day) are effective for most patients. Colchicine, which

The condition is controlled in most patients with 0.6 mg twice daily, effects are seen in 7 to 10 days, and

Azathioprine 150 mg/day was used in patients with 

Dapsone 100 to 150 mg/day controlled three patients in

Hydroxychloroquine and nonsteroidal antiinflammatory drugs are

Cyclophosphamide 2 mg/kg/day induced remissions in patients with multiple-organ involvement who were not controlled with prednisone. The drug had