What Are the Symptoms of Urticarial Vasculitis?

Urticaria vasculitis is characterized by a wind-like rash that lasts for a long time with hypocomplementemia. Inflammatory mediators can damage vascular endothelial cells, so leukocyte fragmentation vasculitis appears. The etiology of this disease is unknown, and may be related to iodine, repeated cold stimuli, and infection factors such as viruses, bacteria, and parasites.

Basic Information

English name
urticarial vasculitis
Visiting department
dermatology
Multiple groups
30 to 40 year old women
Common symptoms
Irregular fever, wind-like skin lesions, itchiness or burning

Causes of urticaria vasculitis

The disease is an immune complex disease with complex etiology and pathogenesis.
1. The factors that induce urticaria vasculitis are unknown. It has been reported that it may be hypersensitive vasculitis caused by pathogens of chemicals and drugs (such as iodine).
2. In low complement urticaria vasculitis, autoantibodies (low-molecular-weight serum complement C1q precipitin) directly anti-C1q collagen-like regions, leading to a decrease in serum C1q, and then activate the complement pathway to produce allergic toxins and neutrophils Granulocyte chemokine. These inflammatory mediators can damage vascular endothelial cells and cause vasculitis. Some scholars have pointed out that the relationship between this disease and connective tissue disease should be paid attention to.

Clinical manifestations of urticarial vasculitis

1. This disease is more common in middle-aged women, and the age of onset is mostly between 30 and 40 years old. Onset is often accompanied by irregular fever, sometimes reaching 38 to 39 ° C.
2. The main feature of the skin is wind-like skin lesions, which are similar to urticaria, but the duration of wind-like skin lesions is long, often 24-72 hours, and does not disappear for several days. Skin lesions are infiltrated, and sometimes purpura is seen at the lesion. A few cases have blisters but no necrosis. After the damage subsided, pigmentation or desquamation remained. Consciously itching or burning.
3. This disease is often accompanied by joint pain and arthritis, which are mainly found in the joints of the extremities, sometimes with joint swelling, abdominal discomfort, and lymphadenopathy. Kidney damage can occur in advanced stages, and epilepsy, meningitis, and unilateral optic neuritis can occur in a few cases.
4. Urticaria vasculitis is often the early symptom of diseases such as dermatomyositis and systemic lupus erythematosus, so the course of disease should be closely monitored.

Urticaria vasculitis test

Laboratory tests showed that erythrocyte sedimentation increased, serum complement levels decreased, autoantibody titers were positive (ANA, dsDNA antibodies, anti-SSA / SSB antibodies), rheumatoid factor positive, and circulating immune complex levels increased.
There are two commonly used auxiliary inspection methods:
1. Routine blood examination can find that the peripheral blood white blood cell count is normal or increased, the proportion of neutrophils increases, and the erythrocyte sedimentation rate is fast. The most common is severe and persistent hypocomplementemia, with a more pronounced decrease in C4.
2. Direct fluorescent examination showed immunoglobulin and complement granular deposits in and around the vessel wall.

Diagnosis of urticaria vasculitis

The diagnosis is mainly based on clinical manifestations and laboratory tests: the clinical manifestations are mainly skin wind masses for more than 24 hours; accompanied by fever, joint pain, abdominal pain, lymphadenopathy, etc. Laboratory tests showed rapid erythrocyte sedimentation and severe and persistent hypocomplementemia. Histopathological examination revealed leukocyte fragmenting vasculitis, and direct fluorescence examination revealed immunoglobulin and granular granular deposits in and around the vessel wall.

Differential diagnosis of urticarial vasculitis

Chronic urticaria
Chronic urticaria usually resolves within a few hours, without joint pain or arthritis, without associated glomerulonephritis, normal blood sedimentation and complement levels, and sometimes histopathologically seen swelling of vascular endothelial cells, but no cellulose-like degeneration and necrosis Features of necrotizing vasculitis.
2. Systemic Lupus Erythematosus (SLE)
Patients with urticarial vasculitis have no light sensitivity and oral ulcers, and have no or only slight visceral damage, which can be distinguished from SLE.

Urticaria vasculitis complications

Complications of this disease include lymphadenopathy, hepatosplenomegaly, nausea, abdominal pain, diarrhea, laryngeal edema, dyspnea, chronic obstructive pulmonary disease, glomerulonephritis, and eye damage.

Urticaria vasculitis treatment

1. Early application of glucocorticoids to prevent systemic complications such as kidney damage. The dosage of glucocorticoids should be determined according to the condition, and can be taken orally or slowly by intravenous drip infusions. After the body temperature returns to normal and most of the skin lesions have subsided, gradually reduce the amount. Because the course of the disease can sometimes last for several months, pay attention to the side effects related to long-term application of glucocorticoids.
2. Non-steroidal anti-inflammatory drugs can treat joint pain symptomatically.
3. Some cases were treated effectively with dapsone, colchicine, hydroxychloroquine, and mycophenolate mofetil.

Prognosis of urticaria vasculitis

Pigmentation often remains after the skin lesions have subsided and often recurs for months or years.

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